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diff --git a/old/63313-0.txt b/old/63313-0.txt deleted file mode 100644 index 0c655b3..0000000 --- a/old/63313-0.txt +++ /dev/null @@ -1,19016 +0,0 @@ -The Project Gutenberg EBook of Neurosyphilis, by -Harry Caesar Solomon and Elmer Ernest Southard - -This eBook is for the use of anyone anywhere in the United States and most -other parts of the world at no cost and with almost no restrictions -whatsoever. You may copy it, give it away or re-use it under the terms of -the Project Gutenberg License included with this eBook or online at -www.gutenberg.org. If you are not located in the United States, you'll have -to check the laws of the country where you are located before using this ebook. - -Title: Neurosyphilis - Modern Systematic Diagnosis and Treatment Presented In One - Hundred And Thirty-Seven Case Histories - -Author: Harry Caesar Solomon - Elmer Ernest Southard - -Release Date: September 27, 2020 [EBook #63313] - -Language: English - -Character set encoding: UTF-8 - -*** START OF THIS PROJECT GUTENBERG EBOOK NEUROSYPHILIS *** - - - - -Produced by Richard Tonsing, Bryan Ness, and the Online -Distributed Proofreading Team at https://www.pgdp.net (This -file was produced from images generously made available -by The Internet Archive/Canadian Libraries) - - - - - - - - - - THE - CASE HISTORY SERIES - - - CASE HISTORIES IN MEDICINE - BY - RICHARD C. CABOT, M.D. - Third edition, revised and enlarged - - - CASE HISTORIES IN PEDIATRICS - BY - JOHN LOVETT MORSE, M.D. - Second edition, revised and enlarged - - - ONE HUNDRED SURGICAL PROBLEMS - BY - JAMES G. MUMFORD, M.D. - Second Printing - - - CASE HISTORIES IN NEUROLOGY - BY - E. W. TAYLOR, M.D. - Second Printing - - - CASE HISTORIES IN OBSTETRICS - BY - ROBERT L. DENORMANDIE, M.D. - Second Edition - - - CASE HISTORIES IN DISEASES OF WOMEN - BY - CHARLES M. GREEN, M.D. - - NEUROSYPHILIS - MODERN SYSTEMATIC DIAGNOSIS AND TREATMENT - Presented in one hundred and thirty-seven Case Histories - BY - E. E. SOUTHARD, M.D., SC.D. - AND - H. C. SOLOMON, M.D. - - Being Monograph Number Two of the Psychopathic Hospital, Boston, - Massachusetts. (Monograph Number One was A Point Scale for Measuring - Mental Ability by Robert M. Yerkes, James W. Bridges and Rose S. - Hardwick. Published by Warwick and York. Baltimore 1915.) - -[Illustration: METCHNIKOFF WASSERMANN EHRLICH SCHAUDINN NOGUCHI] - - - - - NEUROSYPHILIS - MODERN SYSTEMATIC DIAGNOSIS AND TREATMENT - PRESENTED IN ONE HUNDRED AND THIRTY-SEVEN CASE HISTORIES - - - BY - - E. E. SOUTHARD, M.D., Sc.D., - - Bullard Professor of Neuropathology, Harvard Medical School; - Pathologist, Massachusetts Commission on Mental Diseases; Director, - Psychopathic Department, Boston State Hospital; Vice-President, American - Medico-Psychological Association - - AND - - H. C. SOLOMON, M.D., - - Instructor in Neuropathology and in Psychiatry, Harvard Medical School; - Special Investigator in Brain Syphilis, Massachusetts Commission on - Mental Diseases; Acting Chief-of-Staff, Psychopathic Department, Boston - State Hospital - - - WITH AN INTRODUCTION BY - - JAMES JACKSON PUTNAM, M.D., - - Professor Emeritus of Diseases of the Nervous System, Harvard Medical - School - - - BY VOTE OF THE TRUSTEES OF THE BOSTON STATE HOSPITAL - - MONOGRAPH NUMBER TWO - - OF THE - - PSYCHOPATHIC HOSPITAL BOSTON, MASSACHUSETTS - - - BOSTON - - W. M. LEONARD, PUBLISHER - - 1917 - - - - - _Copyright, 1917. - By W. M. Leonard_ - - - - - =In= - - MASSACHUSETTS - - A STATE THAT - - BOTH TOLERATES AND FOSTERS - - RESEARCH - ------------------------------------------------------------------------- - - - - - PREFACE - - -This book is written primarily for the general practitioner and -secondarily for the syphilographer, the neurologist, and the -psychiatrist. Our material is drawn chiefly from a psychopathic -hospital, that modern type of institution in which the mental problems -of general medical practice come to a diagnostic head weeks, months, or -years before the asylum is thought of. - -It is this peculiar nature of psychopathic hospital material—a -concentrated essence of the most difficult daily problems of general -practice—that brings together such an apparent _mélange_ of cases as are -here described, ranging from mild single-symptom diseases like -extraocular palsy up to genuine magazines of symptoms as in general -paresis; from feeblemindedness, apparently simple, up to apparently -simple dotage, both feeblemindedness and dotage really syphilitic; from -the mind-clear tabetic to the maniacal or deluded subject who looks -physically perfectly fit; from the early secondaries to the late -tertiaries or so-called quaternaries; from peracute to the most chronic -of known conditions; from the most delicate character changes to the -profoundest ruin of the psyche. - -Although the bulk of our case-material is drawn from general practice -through the thinnest of intermediary membranes, the psychopathic -hospital, yet we have tried to depict the whole story by presenting -enough autopsied cases from district state hospitals to show exactly -what treatment has to face. Nor have we hesitated to insert cases in -which treatment has failed. - -In addition to (_a_) the Psychopathic Hospital, Boston, group of -incipient, doubtful, obscure, or complicated cases (the early clinical -group) and (_b_) the Danvers State Hospital, Hathorne, group of -longer-standing, committed, fatal cases (the finished or autopsied -group) we present (_c_) a miscellaneous group of cases, including many -from private neurological or psychiatric practice. No doubt those -familiar with Boston medicine will see traces of the teaching of our -former chiefs, notably Professors James Jackson Putnam and Edward Wyllys -Taylor. We are obliged to them for some well-observed cases. - -We have dedicated our work to the Commonwealth, but perhaps we should -more specifically ascribe to the Massachusetts Commission on Mental -Diseases (formerly the State Board of Insanity) the spirit that -permitted our special study of neurosyphilis treatment. To these -authorities, who have countenanced and encouraged a somewhat costly -piece of special work since 1914, we offer our thanks, hoping that other -states will be one by one stimulated to the state-endowment of research. -States doing full duty by research can be counted on one hand. - -To our Psychopathic Hospital colleagues and the internes, and especially -to Drs. Myrtelle M. Canavan and Douglas A. Thom of the Commission’s -Pathological Service, we also offer our best thanks. - -The Danvers traditions are tangible here: cases of Drs. A. M. Barrett, -H. A. Cotton, H. W. Mitchell, H. M. Swift, and others are presented. We -have been especially aided by the more recent work of Dr. Lawson G. -Lowrey. - -Nor should we have been able to present our samples of brain correlation -without drawing on the collection arranged and analyzed by Dr. Annie E. -Taft, Custodian, Harvard Department of Neuropathology. The photographs, -part of a collection of brain photographs now numbering over 10,000 -representing 700 brains of all sorts, were made by Mr. Herbert W. -Taylor. - -The Wassermann testing work has been done by Dr. W. A. Hinton of the -State Board of Health. Dr. Hinton himself wrote out the text description -of the Wassermann method. The method of his laboratory is held to the -standards of control set by previous chiefs, viz. by Professor F. P. -Gay, who brought immunological methods direct from the laboratory of -Bordet (whose method the Wassermann method essentially is), Prof. W. P. -Lucas, and the late Dr. Emma W. D. Mooers, who had assisted Plaut in his -first work with the Wassermann method in Kraepelin’s Munich Clinic. - -The material combed by us to secure this illustrative series amounts to -over 2000 cases of syphilis of the nervous system, including over 100 -autopsies in all types of case. We have presented these with very -varying fulness, chiefly to illustrate the contentions at the heads of -the case-descriptions. - -In using the book, we suggest early reference to the Summary and Key, -where for convenience are placed numerous cross-references permitting -extended illustration of almost every proposition from several cases. - -We have not made a large feature of the Medicolegal and Social section. -This kind of thing well deserves a volume by itself, with all the legal -and social-service implications drawn out in their amazing richness and -detail. The social service slogan, “A paretic’s child is a syphilitic’s -child” has already accomplished a great deal of good in our local world. -Some day we may not be compelled to _drive_ the paretic’s spouse and -offspring to the Wassermann serum test! The general practitioner must -help here. - -A note on the Treatment section. This is manifestly not the last word or -even, we hope, our own last word, since the systematic work of the -Massachusetts Commission must be kept up for some years to get a -reliable verdict. Some of the results give rise to greater optimism than -has prevailed in asylum circles, especially re general paresis. We are -confident that _no one can now successfully make a differential -diagnosis between the paretic and the diffuse non-paretic forms of -neurosyphilis in many phases of either disease_, even with all -laboratory refinements. If this be so, it is _improper not to give the -full benefits of modern treatment to all cases in which the diagnosis -remains doubtful_ between the paretic and the diffuse non-paretic forms -of neurosyphilis. We ourselves advocate modern treatment, not only in -the diffuse, but also in early paretic forms of neurosyphilis. - -It would have been out of place in a book in this Case History Series to -have dealt extensively with the history of our topic. We have -compensated inadequately for this lack by a few remarks at the head of -the Summary and Key. We are, like all others in the field, under the -inevitable obligation to Nonne of Hamburg, whose great work has gone -into three editions, the second of which has appeared in English -translation (Nonne’s Syphilis of the Nervous System, C. R. Ball, -translator). Mott’s work, embodied in a large volume of the Power-Murphy -System of Syphilis, has also been attentively consulted, as well as the -various systematic works on neurology and psychiatry. The topic of -Neurosyphilis is getting wide and appropriate attention in this country -through special journals, both those dealing with nervous and mental -diseases, and those dealing with syphilis. Syphilis is in a sense the -making of psychiatry and will go far to pushing psychiatry into general -practice. - -At the last moment we have been led to deviate from our plan of -presenting only local cases familiar and accessible to us. In a section -on Neurosyphilis and the War, we present excerpts and digests of -English, French, and German cases of neurosyphilis that have appeared in -association with the war. Our own country has not suffered greatly as -yet either from the lighting up of neurosyphilis under martial stress or -from the immediate or remote effects of syphilis obtained in the unholy -congress of Mars and Venus. Space forbids a large collection of these -martial cases, but, as will be seen, a fair sample of problems is -presented. - -Speaking for the moment as the senior author of this book, I wish to say -that, were it not for the energy, industry, and ingenuity of the junior -author, Dr. H. C. Solomon, the book would not have been written. Nor, in -all probability, would the systematic work of the Commonwealth on -neurosyphilis and its treatment ever have been begun. I can also accord -the highest praise to Mrs. Maida Herman Solomon for her social-service -work in this new field. - -Perhaps, in closing, we owe an apology to John Milton for our borrowings -from the two Paradises. Had he known much about syphilis, Milton might -have written still stronger mottoes for us. - - E. E. SOUTHARD - - 74 FENWOOD ROAD - _Boston, Massachusetts_ - - - - - TABLE OF CONTENTS - - - PAGE - - SECTION I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM - (NEUROSYPHILIS). CASES 1 TO 8 17 - - CASE - - 1. Paradigm: protean symptoms, nervous and mental. Autopsy, with - meningeal, parenchymatous, and vascular lesions. 17 - - 2. Tabes dorsalis (tabetic neurosyphilis). Autopsy 31 - - 3. General paresis (paretic neurosyphilis). Autopsy 37 - - 4. Cerebral thrombosis (vascular neurosyphilis). Autopsy 42 - - 5. Juvenile paresis (juvenile paretic neurosyphilis). Autopsy 45 - - 6. Extraocular palsy (focal meningeal neurosyphilis). Autopsy 50 - - 7. Gumma of brain (gummatous neurosyphilis). Autopsy 53 - - 8. _Meningitis hypertrophica cervicalis_ (gummatous - neurosyphilis). Autopsy 56 - - - SECTION II. THE SYSTEMATIC DIAGNOSIS OF THE FORMS OF NEUROSYPHILIS - CASES 9 TO 38 63 - - CASE - - 9. Neurasthenia _versus_ neurosyphilis 63 - - 10. Paretic neurosyphilis _versus_ manic-depressive psychosis 68 - - 11. Neurosyphilis _versus_ manic-depressive psychosis 71 - - 12. Dementia praecox _versus_ neurosyphilis. Autopsy 74 - - 13. Neurosyphilis: negative Wassermann reaction (W. R.) of serum 77 - - 14. Diffuse neurosyphilis: six tests apt to run mild 80 - - 15. Paretic neurosyphilis: six tests strong 85 - - 16. Taboparesis (tabetic neurosyphilis): tests like those of - paresis 92 - - 17. Paretic _versus_ diffuse neurosyphilis: confusion _re_ tests 97 - - 18. Vascular neurosyphilis: positive serum, negative fluid W. R. 101 - - 19. Seizures in diffuse neurosyphilis 103 - - 20. Seizures in paretic neurosyphilis 106 - - 21. Aphasia in paretic neurosyphilis 111 - - 22. Aphasia in paretic neurosyphilis 115 - - 23. Remission in paretic neurosyphilis 117 - - 24. Remission in diffuse neurosyphilis 122 - - 25. _Paresis sine paresi_ 126 - - 26. Paretic neurosyphilis. Autopsy 131 - - 27. Gummatous neurosyphilis. Operation 137 - - 28. Extraocular palsy (cranial neurosyphilis) 140 - - 29. Tabes dorsalis (tabetic neurosyphilis): six tests apt to run - mild 141 - - 30. Tabetic neurosyphilis, clinically atypical 143 - - 31. Cervical tabes 146 - - 32. Erb’s syphilitic spastic paraplegia 147 - - 33. Syphilitic muscular atrophy 149 - - 34. Neurosyphilis of the secondary period 151 - - 35. Juvenile paretic neurosyphilis: optic atrophy 154 - - 36. Juvenile paretic neurosyphilis 157 - - 37. Simple feeblemindedness, syphilitic 159 - - 38. Juvenile tabes 161 - - SECTION III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS - (INCLUDING NON-SYPHILITIC CASES). CASES 39–82 165 - - CASE - - 39. Paretic _versus_ diffuse neurosyphilis. Autopsy 165 - - 40. Paretic _versus_ vascular neurosyphilis, cerebellar. Autopsy 169 - - 41. Paretic _versus_ vascular neurosyphilis, cerebellar. Autopsy 172 - - 42. Tabetic combined with vascular neurosyphilis. Autopsy. 175 - - 43. Tabetic neurosyphilis: mental symptoms, non-paretic. Autopsy 177 - - 44. Cerebral gliosis. Autopsy 180 - - 45. Neurasthenia _versus_ neurosyphilis 183 - - 46. Hysteria. Neurosyphilis of the secondary period 185 - - 47. Manic-depressive psychosis _versus_ paretic neurosyphilis 187 - - 48. Cerebral tumor 190 - - 49. Early post-infective paretic neurosyphilis 192 - - 50. Atypical paretic neurosyphilis, hemitremor. Autopsy 197 - - 51. Paretic neurosyphilis. Autopsy 199 - - 52. Manic-depressive psychosis _versus_ paretic neurosyphilis 202 - - 53. Syphilitic(?) exophthalmic goitre. Autopsy 205 - - 54. Argyll-Robertson pupils 209 - - 55. Argyll-Robertson pupils: pineal tumor. Autopsy 212 - - 56. Neurosyphilis(?) with negative spinal fluid 216 - - 57. Disseminated syphilitic encephalitis, seven months - post-infective. Autopsy 218 - - 58. “Pseudoparesis” 222 - - 59. Syphilitic paranoia? 225 - - 60. Paretic neurosyphilis _versus_ alcoholic pseudoparesis 227 - - 61. Alcoholic pseudoparesis _versus_ paretic neurosyphilis 231 - - 62. Alcoholic neuritis and paretic neurosyphilis 234 - - 63. Chronic alcoholism _versus_ paretic neurosyphilis 236 - - 64. Neurosyphilis, diabetic pseudoparesis, or brain tumor 238 - - 65. Neurosyphilis and diabetes 240 - - 66. Neurosyphilis: hemianopsia 242 - - 67. Paretic neurosyphilis _versus_ syphilis and cerebral malaria 245 - - 68. Paretic neurosyphilis: gold sol test “syphilitic.” Autopsy 247 - - 69. Lues maligna 250 - - 70. Neurosyphilis _versus_ multiple sclerosis 253 - - 71. Atypical neurosyphilis 256 - - 72. Huntington’s chorea _versus_ neurosyphilis 258 - - 73. Senile arteriosclerotic psychosis _versus_ neurosyphilis 262 - - 74. Hysterical fugue _versus_ neurosyphilis 264 - - 75. Tabetic neurosyphilis _versus_ pernicious anemia 267 - - 76. Congenital neurosyphilis 270 - - 77. Congenital _versus_ paretic neurosyphilis 272 - - 78. Juvenile paretic neurosyphilis 275 - - 79. Epilepsy _versus_ juvenile neurosyphilis 277 - - 80. Addison’s disease and juvenile paretic neurosyphilis. Autopsy 279 - - 81. Neurosyphilis of the secondary period 283 - - 82. Taboparetic neurosyphilis and typhoid meningitis. Autopsy 284 - - - SECTION IV. NEUROSYPHILIS, MEDICOLEGAL AND SOCIAL. CASES 83–98 289 - - CASE - - 83. A public character, neurosyphilitic. Autopsy 289 - - 84. Debts, neurosyphilitic 295 - - 85. Suicidal attempt by a neurosyphilitic 296 - - 86. Neurosyphilis and juvenile delinquency 298 - - 87. Neurosyphilis in a defective delinquent 300 - - 88. _Paresis sine paresi_ in a forger 303 - - 89. Trauma: juvenile paretic neurosyphilis 306 - - 90. Trauma: paretic neurosyphilis 308 - - 91. False claim for trauma: neurosyphilis 309 - - 92. Traumatic exacerbation? in neurosyphilis 310 - - 93. Trauma: cranial gumma at the site of injury 311 - - 94. Occupation-neurosis _versus_ syphilitic neuritis 312 - - 95. Character change: neurosyphilis 314 - - 96. A neurosyphilitic family 316 - - 97. A neurosyphilitic’s normal-looking family 318 - - 98. The neurosyphilitic’s marriage 319 - - - SECTION V. THE TREATMENT OF NEUROSYPHILIS. CASES 99–123. - - (CASES 99–103 SHOW THE VARIETY OF STRUCTURAL LESIONS THAT - TREATMENT HAS TO FACE) 323 - - CASE - - 99. An incurable spastic paresis in paretic neurosyphilis. - Autopsy 323 - - 100. A theoretically curable case. Autopsy 328 - - 101. A highly meningitic case, theoretically amenable to - treatment. Autopsy 332 - - 102. A highly atrophic case, theoretically not amenable to - treatment. Autopsy 335 - - 103. Paretic neurosyphilis with markedly focal lesions. Autopsy 338 - - (CASES 104 TO 123 ARE EXAMPLES OF TREATMENT INCLUDING - SUCCESSES AND FAILURES.) - - 104. Diffuse neurosyphilis: treatment successful after nine months 342 - - 105. Atypical neurosyphilis: treatment successful 346 - - 106. Argyll-Robertson pupil not necessarily of bad prognosis: - treated case an insurance risk 350 - - 107. Spinal fluid cleared: symptoms persistent 355 - - 108. Arteriosclerosis does not contraindicate treatment 359 - - 109. Symptoms of intracranial pressure relieved by treatment 362 - - 110. Therapeutic improvement in tabetic neurosyphilis 366 - - 111. W. R. rendered negative in tabetic neurosyphilis 367 - - 112. Example of successful treatment of paretic neurosyphilis 370 - - 113. Another example 372 - - 114. Clinical recovery but tests persistently positive in treated - paretic neurosyphilis 375 - - 115. Improvement delayed in treated paretic neurosyphilis 377 - - 116. Non-neural syphilis in treated paretic neurosyphilis 380 - - 117. Partial recovery in treated paretic neurosyphilis 382 - - 118. Laboratory signs improved: clinical situation stationary: - treated paretic neurosyphilis 384 - - 119. Another example 386 - - 120. Failure of treatment 388 - - 121. Treatment, at first mild, later intensive 390 - - 122. Intensive treatment 392 - - 123. Syphilitic feeblemindedness improved by treatment 395 - - - SECTION VI. NEUROSYPHILIS AND THE WAR. - - CASES A TO N FROM BRITISH, FRENCH, AND GERMAN WRITERS - (1914–1916) 399 - - CASE - - A. Tabes “shell-shocked” into paresis? (Donath) 401 - - B. Latent syphilis “shell-shocked” into tabes? (Duco and Blum) 403 - - C. Aggravation of neurosyphilis by service? (Weygandt) 404 - - D. Aggravation of neurosyphilis _by_ service? (Todd) 406 - - E. Aggravation of neurosyphilis _on_ service? (Todd) 409 - - F. Duration of neurosyphilitic process important. (Farrar) 411 - - G. Latent syphilis lighted up to paresis by war stress without - shell-shock. (Marie) 412 - - H. Paresis lighted up by “gassing”? (de Massary) 414 - - I. Epilepsy in a neuropath lighted up by syphilis acquired at - war. (Bonhoeffer) 415 - - J. Syphilitic—after Dixmude epileptic. (Bonhoeffer) 417 - - K. Syphilitic root-sciatica in a fireworks man. (Dejerine, Long) 418 - - L. Paresis lighted up in civilian by domestic stress of the war. - (Percy Smith) 420 - - M. Shell-shock pseudoparesis. (Pitres and Marchand) 421 - - N. Shell-shock pseudotabes. (Pitres and Marchand) 424 - - - SECTION VII. SUMMARY AND KEY 427 - - APPENDICES: - - A. The six tests 471 - - B. Common methods of treatment 486 - - - - - INTRODUCTION - - -It is a privilege to be allowed to write a word of introduction to a -textbook which so richly fulfils its function as does this volume on the -manifold disorders classified under Neurosyphilis, a subject of which -the importance for the welfare of society is found to loom the larger -the more deeply its mysteries are probed. - -The case histories with which its pages are so amply stocked are -carefully analyzed in accordance with a broadly chosen plan, and the -generalizations that precede and follow them are obviously based on a -wide and varied personal experience such as alone could render a -familiarity with the literature of the subjects treated adequate to its -best usefulness. Both writers were indeed well adapted for this task. -Dr. Southard, as everyone is aware, has long been a highly -conscientious, ardent and productive worker in the department of -pathological anatomy, and of late years a careful student of clinical -diagnosis and methods, both at the Danvers State Hospital and still -more, at the Psychopathic Hospital which he worked so hard to found; -while Dr. Solomon’s researches, in the special field of neurosyphilis, -have been of the highest order. - -Undoubted as are the merits of the case-system of instruction that has -been so much in vogue in recent years, and excellent as is the modern -supplementation of this method by the use of published records, the -danger is still real that the student will have presented to him a -picture of nature in disease that is too diagrammatic, too concise, with -the result that while the task of memory is lightened through simplified -formulation, the training of the doubting and inquiring instincts is -often given too little stimulus and scope. In this book this danger is -deliberately met through the casting of emphasis rather on the -pluralistic aspects of the processes at stake than (primarily) on their -unitary aspects. - -The student who utilizes this volume cannot but emerge from his study a -more thoughtful person than he was at the period of his entry. He will -have seen that clinical rules of thumb cannot be followed to advantage, -and that, on the contrary, surprises are to be expected and prepared -for. Let the recognition of this fact, if it seems to increase the -difficulties in the way of diagnosis, not lead to pessimism in that -respect, or to hopelessness in therapeutics. On the contrary the -writers’ bias is towards the worth-whileness of clinical efforts and an -increased respect for accuracy and thoroughness in the utilization of -modern methods of research. The chance is indeed held open that even the -gaunt spectre of “General Paresis” may prove to be less terrible than it -seems, and for this hope good grounds are given. - -It is in this way made clear, on the strength of anatomical evidence of -much interest, that even if in the treatment of a given patient, the -time arrives when a fatal or unfavorable result seems manifestly -foreshadowed, it may be still worth while to renew the treatment with -fresh zeal, for the sake of combatting some symptom or exacerbation, for -which a locally fresh process furnishes the cause. - -Another noteworthy principle here emphasized and illustrated is that the -relationship between “functional” (hysterical, neurasthenic, migrainoid) -symptoms and the signs (or symptoms) of organic processes is clinically -important and worthy of much further study. This is a matter which, in a -general sense, has interested me for many years. Above and over the -“organic” hovers always the “functional,” as representing the first -indication of the marvelous tendency to repair, or substitution, for -which the resources of nature are so vast. Yet this functional tendency -also has its laws, of which, in their turn, the organic processes -display the action in quasi diagrammatic form. Hysteria, neurasthenia, -migraine, etc., do not arise _de novo_ in each case, but conform to -typical, though not rigid, formulas, susceptible of description. I have -recently had the opportunity to study in detail an analogous series of -transitions between the movements (and emotions) indicative of -apparently purposeless myoclonic movements (on an epileptoid basis) and -the movements of surprise, engrossment, purposeful effort, the -excitement and joy by which the former were excited and into which they -shaded over. - -Taken altogether, this book represents work and thought in which, for -amount and kind, the neurologists of Boston may take just pride. - - JAMES J. PUTNAM. - - ST. HUBERT’S, KEENE VALLEY, NEW YORK. - _August, 1917._ - - - - - Me miserable! which way shall I fly - Infinite wrath and infinite despair? - Which way I fly is Hell; myself am Hell; - And, in the lowest deep, a lower deep - Still threatening to devour me opens wide, - To which the Hell I suffer seems a Heaven. - - Paradise Lost, Book IV, lines 73–78. - - - - - I. THE NATURE AND FORMS OF SYPHILIS OF THE NERVOUS SYSTEM - (NEUROSYPHILIS) - - - PARADIGM to show possible abundance and variety of symptoms and - lesions in DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”). - Autopsy. - - -=Case 1.= Mrs. Alice Morton[1] was in the hands of at least five -well-known specialists in different branches of medicine and surgery -during the nineteen years of her disease. It appears that she acquired -syphilis upon marriage at the age of 23 to a man who later became -tabetic and acknowledged syphilitic infection previous to marriage. Mrs. -Morton remained without children and there were no miscarriages. - -At the age of 27, she developed iritis, paresis of the left eye muscles, -and ulceration of the throat, with destruction of the uvula. The -syphilitic nature of her disease was at once recognized and the -classical treatment was given, although, through numerous shifts in -consultants, this treatment was never pushed to the limit. At 28 Mrs. M. -began to suffer from severe headaches resembling migraine and -accompanied by attacks of paræsthesia; at 35, came severe pains in the -back and difficulty in walking. - -At 36, the migraine attacks began to be accompanied by blurring of -vision and dizziness. The difficulty in walking became extreme, -affecting particularly the right foot. The legs became spastic, there -were pains and hyperæsthesia of the chest, and severe cramps of the -legs. Antisyphilitic treatment at this time yielded marked improvement. - -During her thirty-sixth year, Mrs. M. sustained curious transient losses -of vision and of hearing. She was also irritable, and at this time -developed her first pronounced mental symptoms, namely, delusions -concerning her relatives. There were also a few seizures of an -epileptiform nature. - -At 38 there was a spell of total deafness, followed by improvement. The -eye muscles were also subject to a variable involvement with intervening -spells of improvement. The _knee-jerks were lost, but after a time -returned_ in less pronounced form. Shortly, an absolute paralysis and -extensive decubitus developed, and death occurred at 39. - -The autopsy is briefly summarized below, but it is important in the -understanding of Mrs. M.’s case (particularly some of the sensory -symptoms and the transiency of certain symptoms) to consider the -pre-infective history. Although there seems to be no doubt that the -patient acquired syphilis at about 23 years of age from a syphilitic -husband, who himself later became tabetic, yet it is of note that the -patient was the only child of parents, both of whom also suffered from -mental disease. Mrs. M.’s father died of what was called softening of -the brain (one should avoid terming _all_ old cases of _so-called_ -“softening of the brain” syphilitic, since the older diagnosticians did -not always distinguish between non-syphilitic arteriosclerotic effects -and syphilitic disease). Mrs. M.’s mother also died insane (confusion -and emotional depression). It is clear, then, that we do not need to -suppose that every symptom shown by Mrs. M. is directly due to -destructive or irritative lesions immediately due to the spirocheta -pallida. The case is, in fact, an excellent lesson as to the association -of structural and functional effects in neuropathological cases. - -Mrs. M. as a child had shown talent, but was somewhat nervous and -eccentric. At one time, she had an attack of hysterical dysphasia; at -another time, an attack of hysterical dyspnea; during another period, an -apparent obsession (kicking the mopboard at regular intervals). -Moreover, she had for years suffered from migraines of a severe and -unusual type. Both the hysterical tendency and the migrainous tendency -became mingled with the results of the neurosyphilis in later stages of -the disease in such wise that it was hard to tell exactly where the -structural phenomena left off and the functional phenomena began. - -For example, at the age of 32, nine years after infection and four years -after the earliest nerve symptoms traceable to syphilis, and at about -the time of the onset of spinal cord symptoms, an attack was described -as follows: - - The patient had a very severe attack of migraine (?) yesterday, - preceded and accompanied by paraphasia, so severe that for three - hours she was unable to make herself understood, and indeed felt “as - if her ideas were getting away from her.” This attack was ushered in - by a numbness of the forefinger and thumb of the right hand, which - lasted for about three hours, though the earlier attacks had lasted - for only about ten minutes. During this period the hand felt as if - it had been frozen and the loss of muscular power was so great that - she was unable to hold objects in the hand. In some of the attacks - this paræsthesia has affected the entire left half of the body, and - occasionally the right half. Sometimes the seizures come on with - great suddenness, so that once, when she was attacked while in the - middle of the street, she had considerable difficulty in reaching - the sidewalk. After the worst part of the attack is over a certain - amount of paraphasia may persist for some days, together with - awkwardness in the use of the right hand and numbness. She has had a - great deal of nausea and vomiting, without reference to the taking - of food.[2] - -Bearing in mind the mingling of structural with functional symptoms in -this case, let us consider the autopsy findings. - - - =ANATOMICAL= - - =FORMS OF NEUROSYPHILIS= - - AUTONOMIC (SYMPATHETIC) NEUROSYPHILIS? - - PERIPHERAL NEUROSYPHILIS - - CENTRAL NEUROSYPHILIS - MENINGEAL - VASCULAR - PARENCHYMATOUS - MENINGOVASCULAR - VASCULOPARENCHYMATOUS - DIFFUSE ( = MENINGOVASCULOPARENCHYMATOUS) - - GUMMA - - CHART 1 - - - =CLINICAL FORMS OF NEUROSYPHILIS= - - =HEAD AND FEARNSIDES, 1914= - - SYPHILIS MENINGOVASCULARIS - CEREBRAL FORMS - HEMIPLEGIA - AFFECTION OF THE CRANIAL NERVES - MUSCULAR ATROPHY - LATERAL AND COMBINED DEGENERATIONS - EPILEPSY - - SYPHILIS CENTRALIS - DEMENTIA PARALYTICA - TABES DORSALIS - MUSCULAR ATROPHY - OPTIC ATROPHY - GASTRIC CRISES - EPILEPTIC MANIFESTATIONS - - CHART 2 - - -=Peripheral neurosyphilis=: The lesions of the cranial nerves were -characteristically asymmetrical. Whereas the left third nerve looked -entirely normal, the =right third nerve= had its diameter reduced -two-thirds. On the other hand, the fourth nerves were equal and -apparently normal. The sensory portion of the left fifth nerve was -normal; the right fifth nerve was normal. The =right sixth nerve= agreed -with the right third nerve in being atrophic, and was in fact reduced to -a mere thread without contained nerve fibres at a point 2 mm. from its -superficial origin. Although the right third nerve was atrophic, it was -the =left seventh and eighth nerves= which had become atrophic; the -process had spared the right seventh and eighth nerves. The remainder of -the cranial nerves were grossly normal, except that the =optic nerves= -had an outer zone of a translucent nature. So far, no spirochetes have -been demonstrated in any portion of the nervous system of this case, but -such asymmetrical and focal cranial nerve lesions are perhaps due to -local spirochetal infection, punctuating (as it were) the diffuse -process. - -How much of the transient blindness, deafness, and ocular paralysis can -be explained on the anatomical findings in these nerves? Possibly a -portion of the phenomena can be so explained. Thus, the mechanical -conditions of pressure inside and outside these nerves, both in their -peripheral course and in their passage through the membranes, can be -readily understood to differ during the acute and subacute inflammation, -during the process of repair in the pial tissues, and during the process -of overgrowth of neuroglia tissue about the superficial origins of the -nerves. Of course, the majority of lesions of these nerves were entirely -extinct at the time of the autopsy, and their history could be surmised -only from the appearances in the _left eighth nerve_. Here occurred a -sharply marked focal area of gliosis with apparently total destruction -of nerve fibres and related with a _lymphocytosis_ of the investing -membrane (one of the few areas of lymphocytosis found anywhere in this -case). - -If it were not for the pre-infective history, the hysterical dysphasia -and dypsnea, the youthful obsessions, the migrainous tendency, and the -psychopathic inheritance, we might be tempted to try to explain the -transient blindness, the deafness, and ocular palsies on the basis of -mechanical and toxic variations in the conditions of the peripheral -cranial nerves. The existence of a trace of lymphocytosis in the left -eighth nerve leads to the hypothesis that treatment might still be -effective in this particular region (see below in discussion of spinal -symptoms). - -=Spinal neurosyphilis:= Not only the spinal cord but also the posterior -and anterior nerve roots exhibited severe lesions. These lesions were -both meningeal and parenchymatous. The meningeal process differed in its -intensity in different parts of the spinal cord, being severest in the -thoracic region. At one point in this region, the dura mater was so -firmly attached to the pia mater that the line of demarcation between -the two membranes was hard to make out. In fact, it seems clear that -there could have been no free intercommunication between the spinal -fluid above these adhesions of dura to pia mater and the spinal fluid -below the adhesions. Accordingly, it seems that _lumbar puncture_, had -it been practised in this case, _would have failed to show features -representative of the whole cerebrospinal fluid system_. Moreover, since -at no point in this region of adhesions or in the pia mater of the -spinal cord below this point, were found any lymphocytes, it seems clear -that the ordinary lumbar puncture would have failed to reveal a -pleocytosis. Whether this fluid would have yielded a positive globulin -and excess albumin test, it is now impossible to say; but it appears -that the process in the lower part of the spinal cord was to all intents -and purposes extinct. - -However, there was one region of more severe inflammatory involvement. -The _spinal cord in the cervical region showed a lymphocyte -infiltration_ of its vessels amounting to a mild myelitis (meaning, -thereby, an inflammatory process of the spinal cord remote from the pia -mater). Moreover, in this region, there was, besides the perivascular -infiltration of the substance, also an infiltration of the overlying -membranes themselves, especially in and near the posterior root zones. - -The lessons of this finding are several: The inflammatory process in -this case does not appear to have been entirely extinct! Can we not -suppose that treatment might still have benefited this local -inflammation (perivascular infiltration of the cervical spinal cord -substance and overlying lymphocytic meningitis)? Can we not also picture -the gradual ascent of the inflammatory lesions from lower segments to -higher segments and possibly conceive of the gradual elevation of the -zone of hyperæsthesia manifested in this case as following the gradual -displacement upward of the lymphocytic process? Are there spirochetes in -this tissue? So far none have been discovered, possibly through -inaccuracies of available technique. To the neuropathologist, however, -the lesion looks like a local reaction to organisms. - -In addition to the spinal meningitis, chronic and acute, as above -described, there were extensive parenchymatous spinal lesions. - -In the first place, the meningitis had affected practically all the -posterior roots so that the explanation of the posterior column -sclerosis of this case is clear. The meningitis had apparently been so -marked, also, that all the fibres anywhere near the periphery of the -spinal cord had been likewise destroyed. The posterior columns and the -posterior root zones were markedly sclerotic; or as we say (having -reference to the overgrowth of neuroglia tissue) gliotic. But there was -as much sclerosis (gliosis) of the lateral columns (particularly in the -posterior two-thirds) as there was in the posterior columns and root -zones. In fact, the entire posterior half or two-thirds of the spinal -cord markedly outstripped the anterior portions of the cord in the -severity of the gliosis (sclerosis) shown. - -But although we can explain the posterior column sclerosis, the -sclerosis of the posterior root zones and the marginal sclerosis -(_Randsklerose_) round the entire periphery of the cord, on the basis of -long-standing effects of old meningitis, we cannot thus explain another -finding, namely, the destruction of the fibres in the lateral columns. -This, in fact, is explained through lesions (mentioned below) that -affected the encephalon. The net result of all these lesions of the -spinal cord was to leave only the gray matter and a small amount of -surrounding fibres (belonging to short tracts uniting nearby segments) -intact. Briefly stated, =every long tract in the spinal cord appeared -upon examination to be extensively degenerated=. The genesis of this -parenchymatous loss was, however, double, being in part due to a local -meningeal process (sometimes known as “perimeningitis”) and in part due -to a cutting off of the pyramidal tract fibres on both sides by lesions -higher up in the nervous system. - -[Illustration: - - CASE I. SPINAL CORD (THREE LEVELS) SHOWING: - - A. Marginal sclerosis—effect of old meningitis now extinct. - B. Posterior column sclerosis—effect of meningitis about posterior - roots also now extinct. - C. Bilateral pyramidal tract sclerosis—effect of cerebral thrombotic - lesions. - - Note distortion of tissues in B and C, partly artificial (tissues in - places diffluent). -] - - - =ANATOMICAL FORMULAE= - - =MENINGOVASCULOPARENCHYMATOUS INVOLVEMENT= - - M, V, P, or Combinations Applied to the Classification of Head and - Fearnsides - - I. SYPHILIS MENINGOVASCULARIS - CEREBRAL FORMS M or V or MV[3] - HEMIPLEGIA V - AFFECTION OF THE CRANIAL NERVES M - MUSCULAR ATROPHY M - LATERAL AND COMBINED DEGENERATIONS M - EPILEPSY M or V - - II. SYPHILIS CENTRALIS - DEMENTIA PARALYTICA MVP or VP - TABES DORSALIS MP - MUSCULAR ATROPHY P - OPTIC ATROPHY P - GASTRIC CRISES (M? or) P? - - EPILEPTIC MANIFESTATIONS P? - - CHART 3 - - -Can we offer any explanation of the =partial return of knee-jerks= after -their temporary total loss at a certain period of the disease? We may -assume that the knee-jerks were functionally lost about a year before -the death of the patient through the partial or even almost complete -destruction of the entering posterior root fibres at that level of the -spinal cord which is directly related with the knee-jerk. The later -partial return of the knee-jerks apparently requires us to suppose the -maintenance of some fibres and collaterals by which a functional -connection can be effected between the fibres of the posterior roots and -the anterior horn cells which innervate the quadriceps femoris. Let us -now suppose that _pari passu_ with the actual return of the knee-jerks, -the destructive processes that are affecting both pyramidal tracts high -up in the nervous system are now advancing. It is clear that, whatever -inhibitory influence these pyramidal tracts have been exerting up to -this time upon the knee-jerk reflex arc, that influence is now to be -decidedly reduced in amount and possibly absolutely lost. Upon the loss -of such inhibitory influences exerted from above, the few persisting -connections of the posterior roots and anterior horn cells are now -permitted to resume their functions. - -=Encephalic neurosyphilis=: The lesions mentioned above as causing -destruction of the pyramidal tracts of the spinal cord were -symmetrically destructive and atrophic lesions of the gray matter of -both corpora striata with atrophy of the anterior segments of the -internal capsules. There was a degenerative process of the corpus -callosum especially affecting the forceps minor of the tapetum. The -ventricles were largely dilated, indicating a considerable destruction -and atrophy of the white matter in general. - -After the above discussion of the possible effects of pyramidal tract -lesion in this case, it is unnecessary further to discuss the paraplegia -produced by the cystic lesions of the corpora striata. The theorist -might inquire how these cystic lesions are produced: whether by vascular -blocking or by toxic effects of the accumulations of spirochetes. -Evidence is lacking which would completely sustain either hypothesis. -Still, we do know that lesions almost identical in appearance may be -produced by the necrosis consequent to the plugging of nutritive vessels -in an organ like the brain supplied with end arteries. Therefore, it is -probable that most pathologists would believe these lesions of the -corpora striata to be produced by vascular plugging of the nature of -thrombosis. - -It is worth while to note that there was a suggestion of foci of -encephalitis made out upon the gross examination. The cortex in general -showed strikingly few lesions. However, the convolutions did show in -places numerous ill-defined areas of hyperemia and slight swelling. -These areas were of irregular distribution and only a few mm. or cm. in -diameter. No gross vascular lesions were demonstrable in connection with -these focal areas. Microscopically, however, venous plugs of -polymorphonuclear leucocytes were found, and the local hyperemias were -found to be largely due to venous congestion. However, very few -polymorphonuclear leucocytes were found outside the blood vessels. - -The white matter of numerous convolutions showed microscopically certain -pale spots suggestive of an early atrophic process. Very possibly these -represent a general tendency in the cerebrum to the same process of -parenchymatous loss which had proceeded to such a marked degree in the -spinal cord. - -There was a single large so-called cyst of softening in the cerebellum -(1.5 mm. across by 0.5–7.5 cm. in depth). - -How far can we explain the symptoms of this case on the basis of these -encephalic lesions? We can offer no correlation with the cerebellar -lesion; and possibly this lack of correlation is to be expected on -account of its failure to affect the vermis. As to the cystic lesions of -the corpora striata, their effect in producing paraplegia at the close -of life is obvious, and their possible relation to the partial return of -knee-jerks has been discussed. Literally amazing was the comparative -integrity of the cortical gray matter of this case when the spinal cord -and the interior structures of the encephalon had been subjected to such -severe and numerous lesions. The only mental symptoms noted in the case -were sundry delusions directed against the patient’s relatives and a -certain optimism which led the patient to cling as if with an obsession -to the belief that in the end she would get well. - - - =VARIOUS FORMS OF NEUROSYPHILIS COLLECTED FROM SEVERAL SOURCES= - - =MENINGEAL NEUROSYPHILIS (M)= - - GUMMA OF DURA MATER M - GUMMATOUS MENINGITIS (PIAL) M - SYPHILITIC MENINGITIS (PIAL) M - SYPHILITIC CRANIAL NERVE PALSIES (PRIMARILY PIAL) M - SYPHILITIC BULBAR PALSY M - SYPHILITIC ROOT NEURITIS M - SYPHILITIC TRANSVERSE MYELITIS M - SYPHILITIC NEURITIS (SOME CASES BY EXTENSION) M - SYPHILITIC EPILEPSY (SOME CASES) M - SYPHILITIC MUSCULAR ATROPHY (SOME CASES) M - - =VASCULAR NEUROSYPHILIS (V)= - - SYPHILITIC ARTERIOSCLEROSIS V - SYPHILITIC CEREBRAL THROMBOSIS V - SYPHILITIC APOPLEXY V - ANEURYSM V - SYPHILITIC EPILEPSY V - - =PARENCHYMATOUS NEUROSYPHILIS (P)= - - GUMMA P - CEREBROSPINAL SCLEROSIS P - SYPHILITIC PARANOIA P? - SYPHILITIC CHOREA P - SYPHILITIC EPILEPSY P - TABETIC PSYCHOSIS P? - SYPHILITIC MUSCULAR ATROPHY P - SYPHILITIC NEURITIS P - - CHART 4A - - - =MENINGOVASCULAR NEUROSYPHILIS (MV)= - - CEREBRAL SYPHILIS MV - CEREBROSPINAL SYPHILIS MV - SYPHILITIC EPILEPSY MV - - =MENINGOPARENCHYMATOUS NEUROSYPHILIS (MP)= - - CEREBRAL SYPHILIS MP - CEREBROSPINAL SYPHILIS MP - TABES DORSALIS MP - ERB’S SYPHILITIC SPASTIC SPINAL PALSY MP - - =VASCULOPARENCHYMATOUS NEUROSYPHILIS (VP)= - - CEREBRAL SYPHILIS VP - CEREBROSPINAL SYPHILIS VP - PARETIC NEUROSYPHILIS (GENERAL PARESIS) VP - LISSAUER’S GENERAL PARESIS VP - - =MENINGOVASCULOPARENCHYMATOUS NEUROSYPHILIS (MVP)= - - CEREBRAL SYPHILIS MVP - CEREBROSPINAL SYPHILIS MVP - PARETIC NEUROSYPHILIS MVP - TABOPARESIS MVP - - =DOUBTFUL (TOXIC?, IRRITATIVE?) NEUROSYPHILIS (?)= - - “PARESIS SINE PARESI” - SYPHILITIC NEURASTHENIA - TABETIC PSYCHOSIS - SYPHILITIC PARANOIA - SYPHILITIC POLYURIA, POLYDIPSIA - SYPHILITIC NEURALGIA - - CHART 4B - - -=Summary:= We have here dealt at length with a long-standing DIFFUSE -NEUROSYPHILIS affecting to some extent the entire =meninges= and -producing a destruction of posterior column fibres and numerous other -fibres of the spinal cord (=tabetiform= portion of the neurosyphilis -=picture=). We have also found central lesions of the corpora striata -affecting the destruction of both pyramidal tracts (=paraplegic= portion -of the neurosyphilis =picture=). We have found evidences of acute -inflammation (=lymphocytosis=) in the cervical region of the spinal cord -and in the left eighth nerve (=progressive inflammatory= neurosyphilis -=picture=). In short, we have presented a case of =diffuse= -(meningovasculoparenchymatous) =neurosyphilis= characterized by an -ascending character in a course of at least 16 years; we have indicated -a number of possible clinical correlations, not only with the major -portion of the clinical course (symptoms of myelitis and pyramidal tract -destruction), but we have also mentioned, merely for their suggestive -value, a number of finer correlations between histological findings and -certain clinical features (notably transient losses of vision and -hearing, and a partial return of the lost knee-jerks). Bearing in mind -the clinical and anatomical findings of this case, we shall be able to -discuss the cases that follow in a briefer and more condensed fashion. - - - =TABETIC NEUROSYPHILIS (“tabes dorsalis,” “locomotor ataxia”) - complicated by vascular neurosyphilis (hemiplegia). Autopsy.= - - -=Case 2.= Francis Garfield had been a successful lumberman and had -enjoyed good health until his forty-fifth year. Suddenly one day, while -walking on the street, Garfield lost the use of his legs and for a time -was quite unable to walk. However, he recovered locomotion and after a -time there was nothing wrong with his leg movements except a slight -ataxia. - -At the age of 52 Garfield had to give up work. It appears that he had -been becoming cranky, sometimes, for example, shouting, whistling and -slamming doors, apparently to annoy the family. His intellectual -capacity seemed to be maintained, although his memory was slightly -impaired. - -At 67 years there was an ill-defined seizure, followed a few days later -by another seizure with aphasia (wrong words used and lack of -understanding of things said). - -For years Garfield had been totally deaf in the right ear (following -explosion of a gun?). Now, however, the left ear also showed a sensory -impairment. Slight slurring of speech had been noticed first in the -sixty-sixth year. - -=Physically= there was a slightly enlarged heart with accentuated second -aortic sound and irregular rhythm. =Neurologically=, inability to stand -or walk; marked ataxia in his leg movements; upper extremities quite -well controlled; the pupils were small and unequal, the left being -larger than the right; although the reactions were difficult to test, -the pupils seemed to react slightly to direct light stimuli; the -knee-jerks were absent; tests for sensibility so far as could be -determined did not show any abnormalities; there was much complaint of -sharp pains in the legs. - -There is no doubt that we are here dealing with a case of TABES DORSALIS -plus certain complications due to VASCULAR LESIONS. The case went on to -death from rupture of =aortic aneurysm= (also doubtless a syphilitic -complication). The death occurred at 71, four years after admission to -Danvers Hospital. - - - =MAIN FORMS OF NEUROSYPHILIS= - - =(CLASSIFICATION OF THIS BOOK)= - - DIFFUSE NEUROSYPHILIS - (non-vascular forms of “cerebral,” “spinal” and “cerebrospinal - syphilis”) - - VASCULAR NEUROSYPHILIS - (“cerebral arteriosclerosis,” “cerebral thrombosis”) - - PARETIC NEUROSYPHILIS - (“general paresis”) - - TABETIC NEUROSYPHILIS - (“tabes dorsalis”) - - GUMMATOUS NEUROSYPHILIS - (“gumma of membranes, of brain”) - - JUVENILE NEUROSYPHILIS - (paretic, tabetic, diffuse) - - CHART 5 - - - =POSSIBLE INVOLVEMENT= - - =BRAIN AND CORD SYPHILIS= - - [M]embranes, [V]essels, [P]arenchyma - - [MVP] EARLY, LATENT?, SYMBIOSIS?, ATTENUATION?.... - MVP CEREBRAL, CEREBROSPINAL SYPHILIS, PARESIS MVP - [M]VP PARESIS; SYPHILITIC ARTERIOSCLEROSIS VP - M[V]P ?SYPHILOTOXIN FROM MENINGITIS MP - MV[P] SYPHILITIC MENINGITIS; CEREBRAL OR CEREBROSPINAL SYPHILIS MV - [MV]P SYPHILOTOXIC ATROPHY OR SCLEROSIS P - M[VP] SYPHILITIC MENINGITIS M - [M]V[P] SYPHILITIC ARTERIOSCLEROSIS V - - M, V or P in brackets [] means not involved. - - CHART 6 - - - =NEUROSYPHILIS= - - =SIX TESTS= - - BLOOD WASSERMANN - SPINAL FLUID WASSERMANN - SPINAL FLUID CYTOLOGY - SPINAL FLUID GLOBULIN - SPINAL FLUID ALBUMIN - SPINAL FLUID GOLD SOL - - CHART 7 - - -This case has been especially worked up and published by Dr. A. M. -Barrett on account of the fact that the vascular lesions of the brain -had produced a condition of pure word-deafness. Reference is made to the -Journal of Nervous and Mental Disease, Vol. 37, 1910, for a complete -description of the brain findings and an analysis of the word-deafness, -a summary of which is as follows: - - “Reaction to Words and Sounds.—Total deafness to words spoken, but - gives attention to sounds; no ability to recognize meaning of sounds - heard; no ability to repeat words heard. Spontaneous - Speech.—Retained ability to speak spontaneously, with rare - paraphasic utterances; occasional inability to speak readily the - word desired, but later always giving the correct reaction; - calculation fair; spelling good except for occasional paraphasia; - spelling good for words pronounced. Reaction to Things Seen.—Objects - correctly recognized and named except for an occasional paraphasic - reply; mistakes in pronunciation not recognized; correct color - recognition. Reaction to Things Felt.—Good for familiar objects; an - occasional paraphasic reply. Reaction to Words Seen.—Reads printing - and writing understandingly; unimpaired reading except for an - occasional paraphasic reply; meaning of familiar signs recognized; - slight difficulty in readily understanding meaning of arithmetical - signs. Writing.—Spontaneous writing and drawing ability retained; - ataxia (tabetic) in writing movements; no ability to write from - dictation. Internal language.—No evidence of impairment.” - -The brain post mortem showed severe atheromatous degeneration of the -arteries at the base of the brain. Both middle cerebral arteries showed -scattered atheromatous patches. The pia mater was transparent and -delicate, except in the regions of both Sylvian fissures. There were -residuals of old softening in both temporal lobes. In the fresh brain -the regions of the right and left first temporal convolutions were -sunken inward, and the pia intimately adherent to the softened areas. -The limits and more exact localizing of these softenings were worked out -from serial sections. - -Barrett found in his serial sections that, although the transverse -temporal convolutions of the left hemispheres were intact, these -convolutions were undermined throughout their entire extent by -degenerations in the fibres of the center of the first temporal -convolution. Barrett, accordingly, regarded his case as essentially a -case of subcortical tissue destruction. He agrees with various authors -that the pure word-deafness of his case is the result of an isolation of -the receiving station in the transverse convolutions of the left -hemisphere. The tissue destruction produced by the vascular lesion had -cut off the transverse convolutions from the internal geniculate body. - -We are here, however, not considering the origin and relations of pure -word-deafness but present the case as one of =tabes dorsalis= of 20 -years standing, terminated by two characteristic syphilitic -complications, first, an extensive destruction of brain tissue through -=cerebral thrombosis= and secondly, =fatal aortic aneurysm=. - -=Summary=: We have here dealt briefly with a long-standing case of -NEUROSYPHILIS of the TABETIC type: A characteristic but not necessary -complication of the case is the LATE CEREBRAL VASCULAR INVOLVEMENT. The -=posterior column sclerosis= is virtually the only spinal change. Spinal -meningeal changes are absent (although it is to be assumed that chronic -inflammatory changes in the posterior roots were at one time present in -some quantity and although the spinal fluid characteristically shows -lymphocytosis in tabetic neurosyphilis). - -Whether the spirochetes produce special toxic components able to cause -tabes or whether special kinds of spirochete are the tabes-making kinds -is hard to say. Special qualities of individual tissue may be involved. - -The =cerebral lesions= of a =cystic= nature are of vascular origin, like -the differently localized encephalic lesions of Case 1 (Alice Morton). -Vascular syphilis is not a special property of the vessels of the -nervous system. In fact this very case died of =aortic aneurysm=. - - - =PARETIC NEUROSYPHILIS (“general paresis,” “dementia paralytica,” - “softening of the brain”). Autopsy.= - - -=Case 3.= James Dixon, 44, was first seen at the Danvers Hospital, -reciting verses in a dramatic and noisy way. He remained good-natured -and jolly; nor was there any change in his euphoria until he had become -physically weaker and more generally demented. In fact, Dixon appeared -to become more and more expansive as he became physically weaker. He was -in the habit of describing himself as “O. K., No. 1, Superfine.” - -=Physically= the patient was gray and bald on vertex, had a dusky -complexion, was very thin (6 ft. in height, weight 155 lbs.); the mucous -membranes were pallid; the teeth rather poorly preserved; the heart was -somewhat enlarged; the pulse irregular in rhythm, of poor volume and -tension. - -=Neurologically=, the patient showed a characteristic Romberg sign and -ataxia in walking a straight line. The tremulous tongue was protruded to -the left, and there was a coarse tremor of the extended fingers. The -knee-jerks were absent, and the Achilles jerks could not be obtained; -the plantar reactions were slight; the arm reflexes were present. The -pupils were stiff to light. There was a marked vocal tremor. The -sensations could not be tested on account of the patient’s mental state. - -It appears that Dixon had left school at about 16, at about 22 had gone -into the provision business, and later had become a hotel clerk. He had -married at 28; there had been two miscarriages, at three months and six -weeks respectively; one child was stillborn; four children were living. - -The patient was not very alcoholic. The patient’s wife thought the -symptoms had been coming on since his forty-first year when irritability -set in, but he was not discharged from work until about a year since. He -was taken back again after his wife’s pleas, and remained at work about -three months; but for ten months before admission to the hospital, Dixon -had done practically nothing, had shown a marked memory failure and -speech defect, at the same time claiming to be a person capable of doing -and accomplishing everything. He had become careless of his personal -appearance, collected a drawer-full of stumps of cigars, carried lumps -of coal in his pocket, laughed causelessly, and spat on the carpet. - -We here deal with a case of unknown duration from the initial infection, -but with symptoms lasting about three years and three months. Aside from -the cause of death (empyema of left pleural cavity associated with acute -hemorrhagic splenitis, acute ileitis, and bronchial lymphnoditis), the -body showed a number of other lesions outside the nervous system. There -was the usual sclerosis of the aorta, though perhaps less marked than -usual. There was a curious acute arteritis with fusiform dilatation of -the arteria profunda femoris, with an edema of the thigh muscles and -blebs of the overlying skin. There were also multiple chronic caseating -lesions of the liver, without evidence of fibrosis. The explanation of -these liver lesions is not yet clear. There was a cloudy swelling of the -kidney. - -The calvarium was dense and the dura mater thick and adherent. There was -a chronic leptomeningitis, which, however, was rather unusual in being -most marked in the posterior cisterna and along the sulci of the -cerebellar hemispheres. There was a general cerebral sclerosis, with a -question of atrophy of the superior temporal gyri (suggesting the -so-called Lissauer’s paresis). There was a marked cerebellar sclerosis -with a consequent sclerosis (grossly palpable) of the commissural fibres -of the pons. There was a generalized slight spinal sclerosis. As a fair -sample of the variety of head findings in paretic neurosyphilis, the -details of the =head examination= are presented. - -[Illustration: - - A. Normal postcentral cortex. (Compare B.) -] - -[Illustration: - - B. Nerve cell losses. Perivascular deposits of mononuclear cells, - amongst which are numerous plasma cells. Note decrease in number of - nerve cells. Note irregular disposition of nerve cells. From paretic - neurosyphilis. -] - - Crown bald, with a slight fuzzy growth of short hairs. Scalp - slightly adherent to calvarium; latter of usual thickness but denser - than normal. Dura adherent to calvarium in region of vertex; dura - not remarkable. Sinuses normal. Arachnoid villi moderately - developed. Pia mater a trifle thickened and rather evenly throughout - the cerebral portion. Linear sulcal markings are remarkable for - their absence. The wall of the cerebellomedullary cisterna is thick - and opaque. The most prominent pial thickenings are over the - cerebellum. These are linear or may show feathery out-growths and - are seated over the sulci, particularly in the neighborhood of the - fissure and about the great cerebellar notch. They correspond fairly - well with the focal variation in consistence of underlying tissues - noted below. - - =Brain= weight, 1265 grams. Consistence somewhat increased - throughout and somewhat evenly increased. The prefrontal region - shows the maximal increase of consistence but the remainder of the - frontal region and corresponding occipital region are much firmer - than normal. The two superior temporal gyri appear to be firmer than - adjacent gyri and are possibly slightly diminished in superficial - diameter. The hippocampal gyri are fairly firm. The substance on - section is a trifle more moist than normal. The gray and white - matter cut quite evenly. Diminution in depth of gray matter, if - existent, could not be demonstrated. The ventricles show a moderate - sanding throughout, best marked in the fourth ventricle. The basal - ganglia are not remarkable except for the development of numerous - dilated perivascular spaces about the lenticulostriate vessels. The - =pons= is atrophic, but more so on the right side. The pons, like - the prefrontal cortex, shows on section a distinct increase of - consistence immediately beneath the pia mater. The white bands of - the pons on section are distinctly firmer than the intervening - substance. The olives are of equal consistence. Weight of - cerebellum, pons, and medulla, 155 grams. The =cerebellum= shows an - obvious atrophic and gliotic process of a symmetrical character. The - superior surface, including both vermis and hemispheres, shows a - consistence above normal and general reduction of the depth measured - from the white matter. The reduction in depth gives rise to a - visible depression as compared with tissue posterior to the - postclival sulci. The lobus cacuminis, though slightly raised from - the surrounding lobes, is equally firm, if not firmer. The superior - and inferior surfaces show practically an equal increase of - consistence. The dentate nuclei are not especially increased in - consistence. The flocculi are reduced in size about one-third. - - There was slight universal increase in consistence of =spinal cord=, - best marked in lumbar region. - - =Microscopic findings= are here presented merely in sufficient - detail to establish the diagnosis. The left superior frontal gyrus - shows extensive and somewhat irregular cellular and fibrillar - gliosis of the plexiform layer, together with an increase of - thickened vessels having lymphocytes and plasma cells in their - sheaths. - - The perivascular infiltrations are most extensive in the lower - layers of the cortex. The lamination is in places thoroughly - obscured, except that representatives of the layer of large external - pyramids are almost always demonstrable. - - The layer of medium-sized pyramids has undergone more numerical loss - of elements than have the other layers. - - Gliosis of white matter. - - Specimens from the cerebellum show a destructive process of great - severity, but a little irregular in extent, affecting chiefly the - Purkinje cell belt. The Purkinje cells are often absent throughout - one side of a given lamina, and there has ensued a dense - accumulation of neuroglia cells along a former Purkinje cell belt, - together with a considerable gliosis of the molecular layer. - Considerable gliosis of the white matter, both diffuse and - perivascular in distribution. - - Perivascular plasma cell infiltrations as in cerebrum, but largely - meningeal or in the white matter. - - Sections from the corpora striata demonstrate a mild and early - granular ependymitis, considerable subependymal gliosis of cellular - type, considerable perivascular gliosis in the white portions of the - tissue, and a moderate infiltration of perivascular sheaths with - pigmented cells, lymphocytes, and plasma cells. There is little - evidence of alteration in the nerve cells. Some are unevenly - pigmented. - -=Summary=: We here present a case with numerous and widespread -neurosyphilitic lesions. However, the gross cerebral vascular -complications of Case 1 (Alice Morton) and of Case 2 (Francis Garfield) -are notably absent in James Dixon. Rather atypical (there seems to be -_always something atypical in cases of neurosyphilis!_) are the liver -lesions and arteritis of the leg, atypical, that is to say, for PARETIC -NEUROSYPHILIS. Highly typical of paretic neurosyphilis and almost -constant therein is the aortic sclerosis. - -[Illustration: - - Apparent new formation of small blood vessel. Photographed by Dr. A. - M. Barrett. -] - -[Illustration: - - Rod cells (Stäbchenzellen) in paretic neurosyphilis. Photographed by - Dr. A. M. Barrett. -] - -[Illustration: - - Granular ependymitis—microscopic appearance of a marked example of - “sanding” of ventricle. -] - -Characteristic and constant in paretic neurosyphilis is the -=Plasmocytosis and Lymphocytosis, Perivascular= in distribution about -small cortical vessels. There is also a characteristic (though -characteristically less prominent) =Plasmocytosis and Lymphocytosis, -Meningeal= in distribution. The pleocytosis of the spinal fluid, almost -constant though variable in amount in life, is an indicator of the -meningeal picture and less directly of the parenchymatous picture. - -=Granular Ependymitis= (“sanding” of ventricle floors) is characteristic -and may be regarded as part of the parenchymatous picture. This -ependymitis is an indicator how chemical changes could be readily -produced at least in the ventricular fluids, since the limiting -membranes of the nerve tissue are here subject to multiple breaks. The -“sanding” is a neuroglia reaction to these multiple small breaks -(Weigert’s explanation). - -Parenchymatous losses have led to =Atrophy and Sclerosis=, of very -varying extent in different parts of the encephalon. The atrophy is -characteristic in paretic neurosyphilis, but by no means constant. -Numerous cases have come to autopsy without clearly defined gross -atrophy. Sclerosis is also characteristic and even more frequent than -atrophy, doubtless because sclerosis represents an earlier phase of a -process eventuating in gross atrophy. - -A =Tabetiform Picture= characterizes the spinal cord, but in this case -the tabetic clinical picture did _not_ precede the paretic clinical -picture. We are consequently to regard the tabetic spinal process as -incidental and on all fours with the =Cerebellar and Pontine Atrophy=. - - - =VASCULAR NEUROSYPHILIS (“syphilitic cerebral thrombosis”). - Autopsy.= - - -=Case 4.= James Pierce was an almshouse transfer to the Danvers Hospital -in his fiftieth year. He died three years later. The accompanying brain -pictures demonstrate so extensive a lesion of the left hemisphere that -it is of great interest to determine if possible the genesis and course -of his disease. It appears that syphilis had been acquired somewhere -about the age of 38 or 40, so that the total duration of the process was -between 13 and 15 years. In Pierce’s forty-third or forty-fourth year, -he had a shock while walking in the streets of his native city, -whereupon he was subsequently transferred to the Danvers Hospital, whose -data have been summed up as follows (we are obliged to Dr. Charles T. -Ryder for these data): - - =Neurological examination:= Neuromuscular condition: Barely able to - walk or stand without assistance; hemiplegia of right side; swings - foot out and drags toe out and around in attempting to walk. Right - hand held by side, flexed at right angle; fingers contracted and - thumb thrown across palm. Can lift arm from side; practically no - movements of forearms or fingers; atrophy of deltoid, arm, forearm, - and hand. Muscular movements of left upper extremities fairly well - performed; good strength. - - =Cranial nerves:= Refuses to respond to any tests to determine - hearing or vision, but evidently hears what is said to him, and in - his movements gives no evidence of deafness. Right corner of mouth - droops; tongue protrudes straight. - - =Reflexes:= Pupils dilated; margins irregular; left pupil larger; - they vary in size but it is impossible to determine whether the - variation is due to light or accommodation reflex. Reflexes of right - side extremely exaggerated throughout; there is little ankle clonus; - Babinski is not obtained, patient holding his toes in flexed - position in resisting attempts to elicit reflexes. - - =Sensations:= Reaction to pain stimuli on either side. Evidently - some anesthesia on right side, but pressure is apparently very - painful. There is considerable spasticity of limbs on right side on - passive motion. Too demented to make accurate tests. - - The above examination was made on May 6, 1904. On May 20th the - record states: - - There is almost complete sensory aphasia with word-deafness; some - paraphasic circumlocution. Many of his words are very well - enunciated but have no meaning. Is apparently unable to recognize - objects or their uses. - - Brother stated that he was always supposed not to be over bright. - Physician’s certificate states that he is epileptic, averaging two - attacks per week. On the 15th of May he had a general convulsion; - was unconscious for half an hour, and dull and drowsy for two hours - afterwards. On the 19th, he had a similar attack in the afternoon, - the convulsion lasting a minute, and he was stuporous for an hour. - - On November 8th he had a severe epileptic convulsion. His body was - curled up to the right. The convulsive seizure lasted for two - minutes and was followed by complete unconsciousness for an hour, - when the patient roused and appeared as usual in a few minutes. From - that time to December 15th he had five epileptic convulsions; he was - much more feeble, and unable to help himself as much as formerly. - - Nov. 7, 1905: Patient has had occasional convulsions since last - note, but none during the last three months. He is confined to bed, - has become very much demented, and shows very marked speech defect, - so that he is almost unintelligible. He understands only the - simplest directions. Legs are considerably contracted and knees are - flexed. Arm and hand on the right are paralyzed and show some - atrophic changes; partially flexed. Left elbow jerk is very lively. - On May 23, 1906 he was reported as having Achilles on right side - only, and Babinski on right side. He died January 5, 1907. - - =The autopsy findings= were as follows: - - =Head:= Calvarium of moderate thickness; diploë present; dura - slightly adherent over bregmatic region. Longitudinal sinus contains - cruor clot. Dura is somewhat thickened and slightly more opaque than - normal. Pacchionian granulations, small but fairly numerous. Pia - contains throughout a considerable excess of clear serous fluid. The - convolutions in general are of good breadth and proportion. There is - an atrophic area roughly circular in outline and about 2 cm. in - diameter in the posterior part of the right third frontal - convolution corresponding to Broca’s area on the opposite - hemisphere. The space thus formed is filled with edema held by the - pia. On the left side is a similar subpial collection which covers - the site of the posterior portions of all of the third frontal - convolutions, parts of the lower end of the precentral convolution, - and the whole of the first temporal convolution, which have - disappeared entirely. The basal vessels show slight changes. - - Cerebellum and basal ganglia are grossly normal. - - The spinal membranes are negative. The regions of the pyramidal - tracts in the cord are firm, project slightly from surface of - section, and are china white. - -=Summary:= Here is a picture made up almost purely of VASCULAR -NEUROSYPHILIS, with SECONDARY SPINAL (PYRAMIDAL TRACT) CHANGES. -Doubtless the genesis of this picture is allied to that of Case 1 (Alice -Morton) and to that of the terminal vascular complications in a tabetic, -Case 2 (Francis Garfield). - -The absence of meningeal and parenchymatous (i.e., outside the region of -necrosis produced by the vascular disease) lesions is characteristic of -an important group of neurosyphilitic diseases. It is clear that the -case, although one of _extensive_ lesions, is _not_ one of _diffuse_ -lesions in the sense of Case 1 (Alice Morton). - -The spinal fluid picture in life may nevertheless show (as other cases -amply demonstrate) a certain amount of lymphocytosis and possibly -plasmocytosis, together with a variety of other changes. Treatment might -be expected to keep down these associated changes, although obviously -the effects of the necrosis are final and definite. Franz in Washington -has succeeded in “reeducating” some of these hemiplegics, employing -lower mechanisms of the nervous system. - -[Illustration: - - Vascular neurosyphilis—effects of syphilitic thrombosis of Sylvian - artery 10 years before death. (Case 4.) -] - -[Illustration: - - =Case 4.= (See previous figure for brain lesion.) Three levels of the - spinal cord showing unilateral pyramidal tract sclerosis, 10 years - after cerebral thrombosis. -] - - - =JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”). Autopsy.= - - -=Case 5.= John Lawrence was an under-sized negro, who came under -hospital observation when he was 23 years of age. There was some -evidence that the patient’s father was a neurosyphilitic although -accurate data were out of the question. At all events, John had -Hutchinsonian teeth, a forward bowing of the tibiae, and -Argyll-Robertson pupils. These findings together with a history of -backwardness at school seem to stamp the diagnosis. It seems that there -had been a change for the worse from the age of 18, though the boy had -been able to sell newspapers and black shoes up to within a year of his -arrival at the hospital. During the last months of his life, he showed a -general incoördination, with false movements suggesting those of a -drunken person. There were numerous tremors, the glance was shifting, -and there was a tendency to nystagmus. Some of these phenomena (taking -into account that the Hutchinsonian teeth were not entirely typical and -there was even at times some doubt as to whether the pupils were -actually stiff) led to a question of the diagnosis multiple sclerosis. - -There was, however, little doubt that the case was one of juvenile -paresis. Among the symptoms found at various times in this case are the -following: disorientation for time, place and persons, confusion, with -coarsely irrelevant replies to questions, ill-defined and transitory -delusions of persecution, auditory, tactile, and visual hallucinations, -and defective memory. - -Early in life, the patient had had a habit of falling asleep in school -hours, and had experienced a number of falls at various times. During an -attack of measles he had had a number of spasms, each of which lasted -ten minutes or more. - -The =autopsy= showed death to be due to an early bronchial pneumonia. -The thymus was persistent, measuring 3 × 2 × .5 cm. The marrow of the -femur was red. - -There was a moderate degree of =sclerosis of the aorta= confined to a -few plaques in the arch (not a characteristic syphilitic scarring of the -aorta). The spleen was small and had a thickened capsule. - -The majority of the lesions, however, were in the =nervous system=, and -the following description is taken from the routine hospital records to -exemplify the findings in a fairly characteristic case of JUVENILE -PARESIS. - - =Head:= Scalp closely adherent to =calvarium=. Calvarium heavy - without diploë. =Dura= adherent to calvarium in bregmatic region. - Sinuses contain liquid blood. Arachnoidal villi in considerable - quantity. =Pia mater= contains considerable clear fluid and shows - diffuse haziness and focal thickenings. The diffuse haziness is - almost universal and is best marked over the superior surface of the - cerebellum. The focal thickenings are of general distribution over - the veins of the sulci on the superior surface of the brim and are - heaped up to form considerable linear mounds near the region of the - arachnoidal villi. The superior surface of the cerebellum is - traversed by similar linear mounds of fibrous tissue running at an - angle to the laminæ. There is no notable increase of fibrous tissue - at the base. - - =Brain:= Weight 965 grams. The sulcation is roughly symmetrical - except in the occipital poles where there is unusually rich and - complex but shallow sulcation. The cortical substance is everywhere - firmer than normal, but the sulci fail to flare notably. In a few - places there is a focal increase of consistence of still greater - degree with apparent local hypertrophy (or gliosis with increase of - substance). These foci are in the right second temporal gyrus (3 cm. - in diameter) and in the left first temporal gyrus (of same size but - somewhat less firm) and are of a whitish, waxen appearance, being - visible several feet away by reason of their color and apparent - encroachment upon the adjacent sulci. The foci are sharply limited - by the sulci laterally, but pale out gradually before and behind. - - The convolutions of the vertex show another type of lesion. The - tissue of the greater part of the vertex resembles that of the - flanks and base in being firmer than normal and of a grayish pink - color. Behind the fissure of Rolando on the right side and behind - the anterior limits of the ascending frontal region on the left side - the brain tissue of the vertex becomes suddenly still firmer and of - a yellowish gray color. This lesion disappears gradually into the - occipital microgyria behind and the gyri gradually lose their - yellowish tint. The lesion fades away gradually so that it fails to - involve the temporal convolutions. - - The cerebral tissue cuts firmly and smoothly. The tissue of the - frontal region is a little edematous. The white matter is of a - normal appearance. The ependyma of all the ventricles is somewhat - sanded. The fourth ventricle is most affected. - - The =cerebellum= is not edematous and is as firm as the normal - olivary bodies. The cerebellar hemispheres are symmetrical and of a - normal appearance, save that the laminæ are slightly narrower than - usual and very compactly set. The color, where not obscured by the - haziness of the pia mater, is of a grayish pink somewhat suggestive - of freshly tanned shoe leather. The substance cuts smoothly and - firmly. The dentate nuclei are unusually firm. The =pons= is small, - but of the usual color. Lower structures normal except the =cord= - which is small and shows curious deviations from the normal - markings. The posterior horns and gray commissure are at many levels - the only structures to preserve the normal gray appearance, so that - the H or butterfly appearance is replaced by a crescent. At these - levels, traces of gray matter often stand out in the loci of the - anterior horns. - -The important =anatomical diagnoses= in the nervous system are as -follows: - -Atrophy of cerebrum, 965 grams (there is of course a question whether we -are not dealing with a degree of cerebral hypoplasia). - -Focal scleroses of cerebrum, suggesting the tuberous scleroses of -Bourneville. - -Occipital microgyria. - -Cerebral and cerebellar gliosis. - -Chronic ependymitis. - -Gliosis of the gray matter of the spinal cord. - -Chronic diffuse and focal leptomeningitis. - -The =microscopic examination= confirmed the diagnosis of paresis. The -hypertrophic nodules were of special interest. They were found to be -overlain by a characteristic though thin exudate of lymphocytes and -plasma cells, together with pigmented cells. The nodules appeared to be -supplied with an unusual number of vessels of small calibre, about which -were a few lymphocytes. The large vessels and those with well developed -adventitiæ were surrounded by more numerous lymphocytes and by more -focal accumulations of pigmented cells. The cortex in the middle of a -nodule had almost lost its characteristic cortical layering. The cortex -was here reduced (specimen from temporal lobe) to about one-quarter of -its normal thickness, and was found to be composed largely of expanded -neuroglia cells and vascular tissue, with a few nerve elements, small, -shrunken, and dark-staining. The destructive process appeared to have -borne hardest on the layer of internal large pyramids and the fusiform -layer. There was, however, nowhere any evidence of focal necrosis such -as ought to characterize a true gumma. The sections stained by the -Marchi method failed to show evidence of fatty degeneration within the -focus, although there was a marked diffuse accumulation of fatty -granulations along the nerve fibres in the underlying white matter. A -special study of the cerebellar material was made by one of the -authors.[4] Occasional Purkinje cells showed the characteristic -binucleate condition, which has frequently been noted in recent -literature. - -The cerebellum of this case was perhaps the most markedly diseased of -all portions of the nervous system. As noted, the cerebellar tissue was -exceedingly firm. How far the notable incoördination of the case (he was -observed on staff rounds characteristically curled up in a heap, showing -quite an unusual degree of general incoördination) was due to the -cerebellar lesions, it is perhaps not possible to say. - -=Summary=: John Lawrence, JUVENILE PARETIC NEUROSYPHILIS, is a foil to -Case 3 (James Dixon), paretic neurosyphilis due to acquired syphilis. - -Both showed =Cerebral Atrophy=, but Lawrence the more markedly because -of hypoplasia incidental to the congenital origin of his condition. - -Whereas Dixon gave little or no sign of =stigmata=, Lawrence (besides -being under-sized, having suspicious teeth, and showing at autopsy a -persistent thymus) showed a =Hydromyelia= and curious trefoil shape to -the spinal cord. Dixon on the other hand had liver lesions and arterial -lesions of the leg. - -The suggestion of =Tuberous Sclerosis= in Lawrence is not found in -Dixon; but we have not found it elsewhere. Bourneville did not describe -tuberous sclerosis as syphilitic. - -Binucleate Purkinje cells emphasize the congenital source of the lesions -in Lawrence. - -=Plasmocytosis and Lymphocytosis=, =Perivascular=, and (less marked) -=Meningeal=, are found in both the congenital and the acquired cases, as -also parenchymatous changes, both =nerve cell losses= and =gliosis=. -Both also show granular =ependymitis=. - -It is clear that, over and above the factors of destruction evident in -both Lawrence and Dixon, the congenital case, Lawrence exhibits also the -effects of arrest (in brief not merely atrophy but also hypoplasia). -Early treatment is, therefore, theoretically indicated in the juvenile -group, which means early diagnosis. Early diagnosis and treatment are -still more to be recommended because these juvenile cases progress often -very slowly at first. - - - =FOCAL BASILAR MENINGEAL NEUROSYPHILIS (“syphilitic extraocular - palsy,” plus other symptoms). Autopsy.= - - -=Case 6.= Flora Black, a housewife of 43 years, had been tired out for a -year but had been apparently in fair health. She awoke one day with -double vision due to a left internal strabismus. The visual difficulty -gradually passed away so that five months after the sudden seizure she -was apparently quite well again. There was one exception: about three or -four months after the attack of diplopia, Mrs. Black had begun to feel a -kind of weakness in various parts of the face and there were also fairly -definite paresthesiæ. In the sixth month after the initial attack, the -patient began to be unable to chew and was fain to support the lower jaw -with a bandage to aid in mastication. Deglutition was, however, quite -unaffected and there was never any regurgitation of food. There were -pains in the face, the forehead and the back of the neck. - -Upon =physical examination= at entrance to a general hospital, no -changes in the body at large were discoverable. There was a slight edema -of the ankles, otherwise no sign of bodily disease. - -Conditions in the =head= were as follows: The facial lines were (notes -by courtesy of Dr. E. W. Taylor) smoothed out; both upper and lower -eyelids and the corners of the mouth drooped slightly and more markedly -on the left side. There was slight photophobia and considerable -lachrymation. The patient was unable to pucker forehead, nose or mouth. -The unsupported lower jaw fell and the patient was unable to open the -mouth widely. The movements of the tongue were normally performed. -Speech was mumbling. Sensations of touch, heat and cold were preserved -all over the face except that the left cheek below the level of the -mouth yielded a less accurate registration of tactile sensations. A hot -test tube did not feel as hot in the lower left cheek as elsewhere. -Quinine and sugar could not be tasted over the left half of the tongue -in front. Smell and hearing were also diminished on the left side. It -appeared that there was a complete paralysis of the 5th and 7th nerves -and a partial paralysis of the 8th, 11th and 12th, as well as a defect -in smell. - -The patient died suddenly, three weeks after admission, running a slight -temperature during her stay. The autopsy showed (rather surprisingly) a -double ovarian carcinoma with metastases into the retroperitoneal -glands. Both kidneys were found to be riddled with nodules of carcinoma. -The pelvic veins were thrombosed and there was a complete occlusion of -the pulmonary artery. There was a riding embolus in the foramen ovale -and there was coronary embolism. - -The striking nature of these complications and the interest of the case -neurologically would warrant its publication in complete detail. We here -present the case with utmost brevity as an example of a SYPHILITIC -CRANIAL NEURITIS by extension from the meninges. - -The =brain= was in general without change but there was a considerable -exudate over the entire =pontine region= which had involved several -cranial nerves. The 5th nerves, especially the left, showed gross -effects of the inflammatory lesion. There seems to be little or no doubt -that this neuritis was of syphilitic origin despite the complication of -the case with carcinoma of the ovary and despite the fact that the case -was observed and came to autopsy before the modern methods of systematic -diagnosis could be applied. It is the best case available to us for the -demonstration of a focal cranial nerve lesion of the type characteristic -of neurosyphilis. We may well suppose that similar conditions would have -been found at various stages in the development of Case 1 (Alice -Morton). The pontine region of Case 1 was entirely free from lymphocytic -exudate at the time of the autopsy. Possibly the clearing up of the -pontine pia mater in Case 1 was a therapeutic effect of the thorough -treatment therein used. Whether a case like Mrs. Black’s could be cured -(aside from the ovarian carcinoma and its complications) by the -institution of vigorous systematic treatment is a matter of doubt. -Still, in a general way, these cases of focal syphilitic neuritis are -among the most favorable cases for treatment. - -=Summary=: We present the case of Flora Black to emphasize how slight in -extent and theoretically curable neurosyphilis may be. We fear that Case -1 (Alice Morton) may present too unrelieved and pessimistic a picture. -The extensive vascular lesions and complications of Alice Morton, of -Case 2 (Francis Garfield), of Case 4 (James Pierce) arrest attention by -the incurability of their residual effects (if we omit modern attempts -at reeducation of lower arcs). On the other hand the unrelenting -progress to destruction of important parenchymatous structures, as shown -in the paretic James Dixon (Case 3) and his juvenile replica John -Lawrence (Case 5), as well as in Alice Morton (Case 1) and the tabetic -Francis Garfield (Case 2), lead to a certain justifiable pessimism. For -it is only the meningeal and fine vascular infiltrations of these cases -that we can theoretically hope to combat, probably by destroying the -spirochetes in these meningeal and perivascular loci. We seem -theoretically less able to stop the progress of the often highly -systemic and symmetrical, parenchymatous lesions of the tabetic and -paretic group. - -The condition in Flora Black is clearly much more hopeful, both being -more focal and being almost purely meningeal and therefore accessible to -therapy. - -The two cases which conclude our general survey of neurosyphilis are -also focal cases, one of gumma (Lecompte) and one of focal dural lesion -(Wyman). - -[Illustration: - - 1. Pons, normal except for focal infiltration of left fifth nerve. -] - -[Illustration: - - 2. Higher power view of infiltrated left fifth nerve. -] - -[Illustration: - - 3. Detail of infiltrated left fifth nerve, showing: 1, diffuse - infiltration with mononuclear cells; 2, perivascular infiltration; - 3, strands of relatively unaffected nerve fibers. -] - -Microscopic appearances in Case 6. Extraocular palsy (focal meningeal -syphilis, especially of left fifth nerve). Illustrates exquisite -focality of the syphilitic process sometimes found, as well as its -unilaterality (giving rise to asymmetrical symptoms and signs). Process -in itself probably curable. - - - =GUMMATOUS NEUROSYPHILIS (“gumma of brain”). Autopsy.= - - -=Case 7.= Mrs. Lecompte was a woman of middle age, who, according to the -history given by her son, had been entirely well until her final -illness, which began approximately two years before admission to Danvers -Hospital. The beginning of her trouble seemed to be chiefly headaches, -which would last continuously for several days, or more than a week at a -time. These headaches lasted throughout the course of the disease. In -the morning, on arising, she would feel very dizzy, but this would pass -away during the day. She had had a number of spells of unconsciousness, -lasting about fifteen minutes. In these attacks she would breathe -heavily, there was frothing at the mouth, twitching of the hands, and -the eyes would roll about. Her memory failed gradually, her disposition -changed and she became very irritable. Vomiting occurred almost every -day, and at times was of a projectile character. She became -hallucinated; the hallucinations were chiefly of a visual nature. - -About four months before admission to the hospital, after one of her -seizures, the entire right side was found to be completely paralyzed, -and she complained that it was numb. At this time, she had difficulty -with her speech. In a few days, however, she was able to talk correctly -again, and in a week she was back at work, although the right side was -weak and awkward. She continued to grow worse, and then began to have -spells lasting several days, so that it became necessary to have her -placed in a hospital. - -On admission to the hospital, aside from obesity, the general viscera -showed no points of special interest, and there was no evidence of any -new growth outside of the nervous system. She was unsteady on her feet, -standing with them wide apart. The gait was quite ataxic; the whole -right side was weaker than the left and used more awkwardly. There was a -paralysis of the right side of the face; the right angle of the mouth -drooped; the right eyelid could not be closed but remained continuously -open; nor could the right side of the forehead be wrinkled. Vision and -hearing were not affected. She miscalled tastes and smells; whether this -was due to aphasic difficulties or to cranial nerve involvement could -not be divined. There seemed to be some difficulty in deglutition. The -knee-jerks were markedly exaggerated; slight clonus was obtained but was -not always present. Both pupils reacted well to light and distance and -consensually. Sensation could not be readily tested. There was marked -ataxia, especially with the eyes closed. The speech was thick and -mumbling. The patient was unable to write or copy. Mentally the patient -was quite dull; at times, stuporous; when aroused, was found to be -entirely disoriented. Memory almost entirely absent. In general she -showed herself to be very much confused. - -She remained practically in this condition, even gaining in weight, for -the following two years, when suddenly one morning, she had an epileptic -seizure, vomited, coughed a great deal, with bleeding from the mouth and -ears, and died in a few hours. - -The symptoms in this case pointed to brain tumor. The only inconsistent -thing was the long-continued life,—four years,—after the symptoms were -observed. As she lived before the W. R. and spinal fluid tests were -known, no light was gained in these ways. The post mortem examination -showed the patient had a GUMMA OF THE BRAIN. - -The =summary of the anatomical diagnoses= at autopsy was: - -Decubitus. - -Lymphadenitis of the mesenteric nodes. - -Chronic fibrous peritonitis. - -Chronic fibrous myocarditis. - -Pulmonary hypostasis. - -Thrombosis of vein in right adrenal, with hemorrhage. - -Syphilitic leptomeningitis. - -Gumma of left hemisphere. - -Focal softenings in the pons. - -The =anatomical description of the head= (Dr. A. M. Barrett) is as -follows: - - The sutures in the =calvarium= are well outlined; diploë large in - amount. The =dura= is diffusely but lightly adherent to the - calvarium; it is very dense, especially over the left hemisphere. - The meningeal arteries are thickened but not atheromatous. The - sinuses contain a small amount of fluid blood and post mortem clot. - The inner surface shows nothing abnormal. There is a great - flattening of the convolutions of the left hemisphere, which is not - the case on the right side. Over the convexity, the =pia= is thin - and not abnormal except for some slight adhesions between the - frontal lobes and the two lips of the Sylvian fissures. The pia at - the base over the cisterna, pons, and medulla is thick, cloudy, and - of a grayish gelatinous appearance. It is so thick that it is easily - removable in a large piece. - - The surface of the left hemisphere is dry, and the whole brain is - flabby and bulges as if from internal pressure. A section through - the hemispheres at the region of the optic chiasm shows a hard, firm - area in the left hemisphere deep down in the white substance. It is - about 2½ cm. in diameter, with a wavy border. The central part is of - a silver-gray gelatinous-like appearance, with red spots and whitish - streaks radiating from the centre. In the pons on the right side, in - a plane passing through the posterior corpora quadrigemina, are two - pinhead size softenings among the pyramidal fibres. The ependyma of - the fourth ventricle is granular. - - =Microscopic examination of the tumor=: The area evidently contains - several central necrotic foci surrounded by zones of infiltration - and proliferation, with bordering areas of nervous tissue showing - secondary reactions. The necrotic area stains poorly. From the edge - there are projections of reddish homogeneous bands, some intermixed - with well-differentiated fibrillæ, probably glia fibrils. The - bordering zone is densely infiltrated with lymphoid, plasma, and a - few epithelial cells. The nerve tissue outside of this zone is - spongy and infiltrated with lymphoid and plasma cells. There are a - few scattered, shrunken nerve cells. In this zone and in the zone of - infiltration near the necrotic area, there are scattered cells - resembling giant cells. There are many obliterated vessels in the - area, and other vessels show many infiltrating lymphoid and plasma - cells in the walls. The examination of the specimen stained by the - methods for bacilli of tuberculosis are negative. The growth is a - classical gumma. - - - =GUMMATOUS NEUROSYPHILIS (gumma of spinal meninges, “meningitis - hypertrophica cervicalis of Charcot?”). Autopsy.= - - -=Case 8.= John Wyman was first seen in his thirty-sixth year by Dr. -James J. Putnam. He denied syphilitic infection and stated that the -first symptoms had come four months before. He had begun to notice a -numbness of the fingers, at first of the right hand and shortly -thereafter of the left hand. After a few weeks there had been difficulty -in walking, and a few weeks later headaches, especially on the right -side, developed. Two weeks before he was first seen medically, he had -begun to have a feeling of tightness or constriction in his arms. - -It appears that micturition had been impaired early, that is to say, a -few weeks after the initial sensory disorder had begun. A catheter was -used for a time and improvement followed. Shortly before consultation -retention of urine developed again, this time associated with rectal -incontinence. The feet began to feel heavy and dead. Then the legs began -to be increasingly weak so that the patient was almost bedridden. Vision -appeared to be normal except that reading was followed by fatigue. The -speech was also slow but the slowness could be attributed to fatigue. - -Notes of Dr. Putnam’s =physical examination= are as follows: The patient -lay in bed on the left side, without motion, and almost incapable of -motion. The tongue was protruded, and there was no paralysis of facial -muscles, or of the eye muscles (the right pupil had been reported to be -slightly larger than the left). There seemed to be a disinclination to -move the head, but with some effort it could be moved, and without pain. -The arms and hands were held rigidly in median positions; many movements -were possible, but all were imperfect and of slight amplitude. The -fingers were flexed to a moderate degree, and could not easily be -straightened, and there was, in fact, a general rigidity of most of the -muscles of the body below the neck, and even, in some degree, of the -neck. The immobility was so great that the general impression made was -almost that of a patient with fracture of the spine in the cervical -region. Even the breath, and especially the inspiration, was imperfect. -The legs were more freely movable than the arms, but still the motions -were very stiff and awkward, and of slight amplitude; with effort the -whole leg could be lifted from the bed, and flexed or extended with -moderate force. The right leg was rather stronger than the left, but the -left hand and arm were stronger than the right. The sensibility was -almost absent over the hands and lower part of the arms, and was -impaired over the entire head and neck, except the forehead, the middle -part of the face, and the nose. It is interesting to compare the -conditions of the sensibility here present with those seen in cervical -syringomyelia. The sensibility of the upper part of the forehead was -less good than of the lower part, and there was slight impairment even -over portions of the lower jaw. The sensibility of the left (stronger) -arm was rather more impaired than that of the right arm, while on the -contrary the sensibility of the left leg was better than that of the -right leg, though the difference between them was not great. These -statements apply to sensory tests by contact, heat, cold, and pricking. -Knee-jerks were highly exaggerated, and likewise the wrist-jerks. All -forced attempts at movements were attended by a high degree of muscular -tremor, especially when the patient was fatigued or under emotional -strain. The fingers especially were the seat of coarse tremor. - -The remainder of this clinical description (courteously supplied us by -Dr. Putnam) may be quoted. A second examination which included also a -few facts not given in the first examination was made on the following -March 28, 1905. This report says “the ends of the fingers became numb -about June 1, 1904. Work was given up on July 3, and at that time the -patient was walking very badly. No treatment was used and no -satisfactory diagnosis made. In the course of July he improved somewhat, -and during August he was able to ride out a little (these spontaneous -improvements are of interest for the diagnosis). He went away from home -for a short time, but from the time of his return, about the last of -September, he grew worse rapidly, and fell into the condition above -described, in which he was wholly unable to help himself, even to -turning in bed. At times he had a great deal of pain in the neck and -forehead. Antisyphilitic treatment was recommended, and for a time -potassium iodid and other iodid preparations were given, but at first in -relatively small doses (grs. 75 daily). Under this treatment the -excretion of urine rose to four quarts daily as a maximum though -sometimes the quantity was not so great.” - -Under this treatment the patient began soon to improve, and continued -doing somewhat better till about five months later. He became able to -walk downstairs and out of doors, and regained considerable use of his -hands. The quantity of urine passed became greatly increased by the use -of the iodid. - -About the middle of March he became worse again. A careful examination -of the sensibility showed that in general the condition was much the -same as that previously reported. The iodid treatment, with perhaps some -mercurial, was resumed; the potassium iodid was given in doses which -were increased up to 850 grains daily, although this maximum dose was -taken only for about one week. This large quantity gradually impaired -the sense of taste for the time being, and blurred his vision, but -otherwise did him no harm. Under this he improved, so that he became -able to run more or less, and went about freely, and attended to his -business, though still retaining some stiffness in his movements. - -This improvement continued until about two years later, when he again -had a relapse, and was seen medically once more. His condition at this -time was still a pretty good one, but the movements were stiff and -awkward. The bin-iodid of mercury was advised, which was taken in doses -of 9⁄25 grain daily. It will be remembered that this was long before the -days of salvarsan treatment. - -This was toward the end of June, 1907. Contrary to expectation, there -was no material gain from this treatment, and the patient died early in -October, without being seen again. - -The =autopsy= was limited to the =nervous system= and the findings were -as follows (Dr. A. R. Robertson): - - =Head=: Hair abundant, fair, of fine texture and rather curly. Scalp - of medium thickness and strips readily from calvarium. The latter - appears normal and upon removal is of about the normal thickness. It - lifts readily from the dura mater, except for the numerous - attachments of Pacchionian granulations. - - =Meninges=: The dura is smooth, moderately injected and shows no - areas of thickening; it lifts readily from the pia-arachnoid. The - pia-arachnoid shows discrete and in many places diffuse areas of - opacity. There is a moderate amount of subpial clear fluid and the - vessels are moderately injected. Over the anterior surface of the - medulla and lower portion of the pons and largely confined to the - right side there is a very marked thickening of the pia-arachnoid to - which the dura is densely adherent. This thickening extends down - anteriorly and laterally on the right side over the upper part of - the cervical cord. The thickened meninges over the upper part of the - medulla completely surround the right vertebral artery, shortly - before it joins its fellow of the opposite side to form the basilar. - Dissection of the arteries shows them to be patent and thin walled. - Over the =cerebrum= and cerebellum the pia-arachnoid strips readily - leaving a smooth surface. Section of the cerebral cortex, basal - nuclei, pons and cerebellum show no gross lesions. The ventricles - are moderately distended with fluid. The ependyma contains numerous - small cysts. Section of the =pons= shows no lesions of the nervous - tissue, but very marked thickening of the surrounding meninges as - noted above. - - =Cord=: Throughout the cervical and dorsal region the dura is quite - tensely distended with an abundance of clear, light, straw-colored - fluid. Upon snipping the dura this fluid escapes with a small spurt, - as if under considerable pressure. The cord within, for the most - part, lies free, but over the upper three or four centimeters of the - cervical portion it is densely adherent to the dura anteriorly and - laterally on the right side. Cross sections were made through the - upper three or four centimeters of the cord, and over this area the - cord is constricted by very marked thickening of all the meninges. - The meninges here average from one to three millimeters in - thickness. On the right side and somewhat anteriorly opposite the - junction of the atlas and axis there is a single nodular, firm mass - which on section shows a yellowish, firm center surrounded by very - dense, pearl-gray tissue. The demarcation between the homogeneous - yellowish centre and its surrounding gray tissue is very sharp. This - nodule measures about 0.75 to 1 cm. in diameter. The adjacent cord - is deeply indented by it. Below this nodule there is a translucent, - grayish appearance of both posterior sensory columns which extends - downwards and diminishes in intensity until it finally disappears in - the upper dorsal region. This same appearance is well marked on the - right outer margin of the upper cervical cord corresponding to the - crossed pyramidal tract, and extends downwards diminishing in - intensity until it disappears about the mid-dorsal region. The left - pyramidal tract appears to be similarly but very slightly involved; - section of the lower dorsal cord entirely negative. - =Microscopically=, characteristic GUMMA. - -It is a question whether this case is one of the group described in 1871 -by Charcot under the name of _pachymeningitis cervicalis hypertrophica_. -Charcot did not regard his new disease as syphilitic, and it is very -probable that syphilis is not responsible for all cases. Charcot, -however, noted that his new disease was not incurable: he noted that the -resulting paraplegia, although it might be very marked and accompanied -by flexion of the leg on the thigh and although the paraplegia might -have lasted a very long time, might end in recovery. Charcot thought -that surgical intervention was necessary. He described three periods in -the disease, the first or neuralgic (pseudo-neuralgic) was characterized -by sharp pains in the neck and by the sensation of constriction in the -upper part of the thorax. The second phase of the disease was, according -to Charcot, the paralytic phase, in which a cervical paraplegia -accompanied by muscular atrophy developed. Sometimes cases were found to -remain in this paralytic phase and even to end spontaneously in cure. If -the muscular atrophy was degenerative, then the atrophy was never -replaced; but, according to Charcot, some cases of atrophy were simple -and accordingly curable. If, however, the spinal cord itself became -involved in the meningeal inflammation, then phenomena of transverse -myelitis set in with a spastic paraplegia and involvement of the bladder -and rectum. Muscular atrophy never developed in the legs, at least in -typical cases. - -Among the causes of this condition the following have been mentioned: -cold, overexertion, alcoholism, tuberculosis and syphilis. Syphilis -undoubtedly plays the major part. Even before the days of the W. R., -observers, among whom may be mentioned Dejerine-Tinel and Pförringer, -discovered syphilis in nearly all sufferers from _pachymeningitis -cervicalis hypertrophica_. - -It should be differentiated from caries of the spine and cord and -meningeal tumors. The spinal fluid examination makes this somewhat easy. - -Antisyphilitic remedies are indicated, and should be tried even when the -etiology is obscure, if only as a therapeutic test. - - - But what have been thy answers? What but dark, - Ambiguous, and with double sense deluding, - Which they who asked have seldom understood, - And, not well understood, as well not known? - - Paradise Regained, Book I, lines 434–437 - - - - - II. THE SYSTEMATIC DIAGNOSIS OF THE MAIN FORMS OF NEUROSYPHILIS - - - =PARETIC NEUROSYPHILIS (“general paresis”) sometimes persistently - receives the diagnosis NEURASTHENIA simply through omission to apply - approved diagnostic methods.= - - -=Case 9.= Greeley Harrison, a man of 46, certainly looked like a -neurasthenic. He wanted aid for nervous indigestion of years’ standing, -headache, insomnia, nervousness, failing memory, and deafness. He -volunteered, in fact, that he had neurasthenia, and that he had been -treated for this by hypophosphites. - -During the practically negative =physical examination=, Harrison -complained of headache and throbbing in the head, and during examination -of the abdomen felt much nauseated and proceeded to vomit rather -persistently. There were hemorrhoids. - -=Neurological examination= showed that the left pupil was smaller than -the right, was irregular, failed to react consensually, and reacted very -slowly to direct light. For the rest, however, the neurological -examination was negative. On account of the nausea and vomiting, special -examination of the gastric contents was made, but nothing abnormal was -found. - -=Mentally=, it was rather striking that the patient’s memory was quite -inaccurate both for remote and for recent events. His school knowledge -was very meagre. As for delusions, the only approximation thereto was -the patient’s continually dwelling upon his bodily symptoms. -Emotionally, he varied between depression and a sanguine attitude. - -Although there was no symptom directly suggesting syphilis in the -Harrison case, the slightly abnormal pupillary reactions and the amnesia -warranted the suspicion of syphilis. The blood and spinal fluid both -proved positive to the W. R.; the gold sol reaction was of the “paretic” -type; there were 18 cells per cmm.; there was considerable globulin, and -an excess of albumin. On the whole, therefore, we felt entitled to make -the diagnosis GENERAL PARESIS. Why should not a careful observer have -considered syphilis seriously? Yet in our experience such cases are -frequently diagnosticated neurasthenia, thus entailing dangerous delay -in treatment (in this case, five years’ delay). - -Going over the history of the case with still greater detail, we learned -that for a number of years past, there had been symptoms of a -neurological nature. For instance, five years before, at the age of 41, -the patient had been apparently overcome when working near a stove, and -went upstairs talking incoherently, but recovered shortly. Thereafter, -such spells occurred almost every month; later, more frequently; still -later, the attacks were associated with unconsciousness and amnesia. -Occasionally preceding the attack there would be twitching of the mouth, -jerking of the arms, and incoherent talk. Throughout these last five -years, in point of fact, the patient had been unable to do regular work, -had been given to much complaining, and had been far less efficient than -formerly. In short, it would seem that, with the improved technique now -in the possession of medical science for the diagnosis of general -paresis, cases like that of Harrison will be diagnosticated earlier and -earlier. - - 1. How typical is the insidious onset of symptoms in the case of - Harrison? The onset of symptoms in neurosyphilis is ordinarily - considered to be sudden, and this statement is generally true - despite the fact that after the diagnosis is established a number - of mild prodromal symptoms can be remembered by the relatives. - However, some cases, of which Harrison is an example, have an - exceedingly insidious onset without sudden access of striking - symptoms. Joffroy and Mignot remark that with the improvement of - clinical methods, the course of paretic neurosyphilis must now be - stated to take some six or seven years for completion. In point of - fact, there were early episodic symptoms (seizures almost monthly) - which should not have escaped medical attention. They did escape - medical attention, however, and Harrison was wont to say “Why - wasn’t I told that my disease was syphilis five years ago?” - - 2. Is there such a disease as syphilitic neurasthenia? According to - Kraepelin, syphilitic neurasthenia has been described as occurring - shortly after infection and in the first stages of syphilis. There - are milder and severer forms; the milder forms show discomfort, - difficulty in thinking, irritability, insomnia, cephalic pressure, - indefinite variable, uncomfortable sensations, and pains. The - severer cases acquire anxiety, more pronounced emotional disorder, - dizziness, disorder of consciousness, difficulty in finding the - right word, transient palsies, pronounced sensory disorders, - nausea, and increase of temperature. Kraepelin is in doubt whether - there is any definite clinical picture of this sort, and whether - there is any causal relation between the syphilitic infection and - such symptoms as those described. If the effect of knowledge - concerning infection is a merely psychic effect, then it is - improper to term the neurasthenia in question a syphilitic - neurasthenia. For the relation of hysteria to the acquisition of - syphilis, see below the case of Alice Caperson (46). In point of - fact, modern work has shown even in the primary and secondary - stages of general syphilis more or less pronounced neurosyphilitic - phenomena in the shape of the so-called meningitic irritation of - French authors. (Besides the case of Caperson (46), see the case - of Fitzgerald and the discussions under these cases.) - - 3. What is the relation of the early symptoms of this case to the - so-called preparesis of Dana? The case might well have been an - example of Dana’s preparesis. For a discussion of this, see Case - of William Twist (13). - - 4. What is the classical differential diagnosis between paretic - neurosyphilis and neurasthenia? The testing of the blood by the W. - R. is unconditionally necessary. If the W. R. is negative, the - diagnosis of paretic neurosyphilis is extremely improbable. (It - must be borne in mind that a number of cases of paretic - neurosyphilis have been shown to have a negative W. R. in the - serum, and receive a proper diagnosis only after spinal fluid - examination.) Next to the serum W. R. stand the pupillary and - aphasic symptoms. In the presence of Argyll-Robertson pupil or - even a slight speech defect, the diagnosis of neurasthenia must - certainly be made with caution if at all. Kraepelin remarks: The - sudden occurrence of neurasthenic disorders in a male of middle - age without any evident cause therefor is always suspicious. Yet - it must be emphasized that a complaint of occasional dizziness, - slight speech defect, tremor of tongue, and a moderate increase of - tendon reflexes do not possess any marked diagnostic significance. - Clear insight and understanding of the nature of the disease - phenomena, a persistent search for recovery, reasonableness in - conversation, progressive improvement under appropriate treatment, - speak for neurasthenia. - - Joffroy and Mignot differentiate what they call preparetic - neurasthenia from other neurasthenic states, not only on the basis - of its etiology but on the basis of its symptoms. They also call - attention to the fact that neurasthenia, being a pure neurosis, - develops either on a manifestly hereditary basis or upon some - physical injury, weakening disease, or moral shock. The pure - neurotic suffers a great deal more than the patient who is - destined to become a victim of paresis. The character change in - neurasthenia does not amount to that entire transformation of - personality (even to the performance of criminal acts) that we - find in paretic neurosyphilis; at the most, the neurasthenic shows - minor emotional disturbances and a certain pathological egoism. - The psychotherapeutic test also rather readily dissipates many of - the neurotic, hypochondriacal fears and feelings. Although both - pure neurasthenia and the paretic pseudoneurasthenia are - characterized by sexual weakness, the sexual anæsthesia of the - preparetic is practically always preceded by a stage of sexual - over-excitement. These finer clinical indications, however, fade - into insignificance beside the data that can and should be - obtained from laboratory tests. - - 5. How exceptional is such a case as that of Harrison? We have in - our experience seen many patients with a similar course and - configuration of symptoms, although the majority of these cases in - a community advanced enough to provide easy access to a Wassermann - laboratory are now diagnosticated far earlier than was the case of - Harrison. - - 6. What attitude shall we take toward so-called syphilophobia? It - seems to us that resort to a serum W. R. is indicated, both from - the standpoint of the community and still more importantly from - the standpoint of the patient. We are even inclined to suggest for - a case of persistent syphilophobia, when the serum W. R. has - proved negative, a lumbar puncture. Syphilophobia must be - considered, not as a syphilitic psychosis, but as a phobia to be - classified among the psychoneuroses. It becomes a difficult - question to decide at times whether a patient who has had - syphilis, has had a considerable course of treatment and shows the - symptoms of a syphilophobiac should be further treated for - syphilis or merely for his phobia. We have seen recently such a - patient who gave a certain history of syphilis and who was greatly - disturbed lest he should be developing paresis. This fear bothered - him greatly. Examination showed irregular pupils, but no other - signs of syphilis. The W. R. in blood and spinal fluid was - negative as were the other spinal fluid tests. It was considered - wise to treat him only for his phobia and under this treatment he - was given some relief. - - - =PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like - MANIC-DEPRESSIVE PSYCHOSIS.= - - -=Case 10.= The mental picture in Lyman Agnew, an architect, 58 years of -age, was wholly characteristic of manic-depressive psychosis. In the -first place, there had been (at 55) a previous attack of depression, -lasting a few months, from which Agnew had completely recovered. He had -remained entirely well up to four months before consultation. -(Manic-depressive psychosis is, at least in a majority of cases, -hereditary. There had been mental disorder in one maternal cousin, and -mental impairment in the patient’s mother some time before her death -from cerebral hemorrhage. There was no other report of mental disease in -the family.) - -It appears that in the interval between attacks, Agnew had been working -very hard and had been fairly successful in paying off a mortgage on his -house. A marked elation, somewhat natural, followed this success and -continued to an abnormal degree. Agnew labored under considerable -excitement, was over-fussy, and at times showed a flight of ideas. His -mania or hypomania gradually diminished and depression set in, in which -depression he arrived for consultation. He had marked ideas of -self-accusation, was emotionally unstable, wept much, and showed a -characteristic retardation of activities and unrest. - -=Physically=, there was no neurological disorder. The patient appeared -rather under-nourished. The heart borders lay 2 cm. to the right and at -11½ cm. to the left of the mid-sternal line. The aortic second sound was -very loud. There was a moderate radial arteriosclerosis. Systolic blood -pressure was 210, diastolic 155. - -The high blood pressure suggested nephritis, possibly of -arteriosclerotic origin, but urine examination and blood-nitrogen tests -yielded no evidence of kidney disease. Moreover, it is our experience -that a manic-depressive psychosis in persons past middle life is not -infrequently complicated by high blood pressure. In point of fact, some -authors insist upon a relation between manic-depressive psychosis and -the arteriosclerosis which rather frequently sets in in this disease. - -Routine examination of the blood serum, however, yielded a positive W. -R. Following the approved rule of making an examination of the spinal -fluid in all mental cases having a positive serum W. R., we proceeded to -lumbar puncture. The fluid was clear and contained 35 cells per cmm., -the albumin was in excess, and there was a positive globulin reaction. -The gold sol reaction was of the “paretic” type; the W. R. was strongly -positive. - -On this basis, it seems worth while to consider the diagnosis of GENERAL -PARESIS or that of some form of non-paretic neurosyphilis. The former is -the diagnosis which we prefer. - - 1. What is the classical differential diagnosis between - manic-depressive psychosis and neurosyphilis? The laboratory tests - have naturally supplanted the older purely clinical methods of - differential diagnosis. The difficulties lodge, in the first - instance, in depressive states. It would appear to be impossible - on purely clinical grounds in certain cases to tell the depression - of neurosyphilis from the depression of manic-depressive - psychosis, since the slightly greater interest in the outer world - taken by manic-depressive patients and their greater - responsiveness to diagnostic threats (suggestion that patient is - to be pinched or cut) are of no special value in the individual - case. Identical considerations hold for the maniacal phases of - manic-depressive psychosis, for these maniacal phases may even - develop delusions (Kraepelin) of precisely the same nature as the - characteristic expansive delusions of the excited paretic. - - 2. If the clinical symptoms are insufficient in differential - diagnosis, are not the pupillary signs and the speech defect of - greater value? They are of value if present, but as in the case of - Agnew, the victim of neurosyphilis may show no pupillary or speech - disorder. Instances are familiar, also, in which the pupillary and - speech signs are absent in very advanced cases of non-paretic or - even of paretic neurosyphilis. - - 3. Would not a circular course or recurrence of attacks be decisive - for manic-depressive psychosis? Paretic neurosyphilis sometimes - exhibits the same circular or recurrent course. We conclude that - neither the clinical symptoms, the classical pupillary and speech - signs, nor the ups and downs of a particular disease, are at all - decisive as between manic-depressive psychosis and paretic - neurosyphilis. Resort must be had to laboratory tests. - - 4. What is the significance of the high blood pressure in paretic - neurosyphilis? Work from our laboratory (Southard and Canavan) has - shown plasma cells in the kidneys in 17 out of 30 paretics (56%), - and in 16 of these 17 paretics with renal plasmocytosis, the - plasma cells were found in the periglomerular region. What the - relation of these findings may be to heightened blood pressure is - as yet unknown. The severe syphilitic involvement of the aorta so - characteristic in paretic neurosyphilis, as in other forms, may - possibly have a bearing on blood pressure. - - - =A POSITIVE SERUM WASSERMANN REACTION associated with mental - symptoms (even with grandiosity) does NOT prove the EXISTENCE OF - PARETIC NEUROSYPHILIS (“general paresis”).= - - -=Case 11.= Juliette Lachine came to a general hospital with pain in the -right upper quadrant of the abdomen, wherein was found an enlarged -liver. This liver was regarded as syphilitic on the ground that the -patient had a positive serum W. R. and that her two elder children were -clearly suffering from congenital syphilis. The liver mass was promptly -reduced by antisyphilitic treatment of the classical sort. When, -however, the patient was given an injection of salvarsan, she shortly -began to develop marked mental symptoms, whereupon she was removed to -the Psychopathic Hospital. - -The =mental picture= at the Psychopathic Hospital was as follows: Lack -of orientation for time, marked distractibility of attention, with a -certain jumping from one subject to another, delusions of a religious -nature, claims of wonderful powers possessed by the patient, moods -variable, though as a rule of a euphoric and elated nature, with -laughing and singing. The activity seemed to be of a mental rather than -a peripheral nature. The patient did not regard herself as mentally -abnormal. The liver was still 4 cm. below the costal margin in the -nipple line. We found the W. R. to be positive in the serum but negative -in the spinal fluid. In fact, the spinal fluid was entirely negative. - -So far as we are aware the picture presented by this case is one of -MANIC-DEPRESSIVE PSYCHOSIS. We regard the disease as merely complicating -the syphilis, although it is entirely possible that some visceral -condition incidental to the syphilis might be proved (in a higher stage -of psychiatric science) to have produced the mania. - -In any event, the patient quite recovered from her mental symptoms in a -month. She was then able to tell us of a previous attack of depression -some 12 years previously, namely, at the age of 26. It appears that she -had at that time been committed to a hospital for the insane. - - 1. In this case, in which the diagnosis of manic-depressive - psychosis and not paretic neurosyphilis was made, are we sure that - the symptoms that we term manic-depressive psychosis were not - actually produced by syphilotoxins? In other words, in the absence - of spinal fluid signs of inflammation or chemical change, might it - not be possible for generalized syphilis outside the nervous - system to produce manic-depressive symptoms? There is so far in - the literature no experimental or other evidence of syphilotoxins. - The existence of products and substances permitting the W. R. and - the gold sol reaction is not of course evidence of syphilotoxins. - Although there is no evidence of soluble syphilotoxins, it is - thought that in the so-called Järisch-Herxheimer reaction (the - intensification of clinical symptoms after salvarsan injection) - effects may be due to the liberation of products from the killed - bodies of spirochetes. Such endotoxins are not here in question. - - 2. Is visceral syphilis, such as gumma of the liver, able to produce - characteristic syphilitic reactions in the spinal fluid? We have - had an autopsied case in which there was a “paretic” gold sol - reaction of the fluid (though without other signs). The autopsy - showed gummata of the liver. However, the finer anatomy of the - nervous system showed a mild but definite meningo-encephalitic - process, which was doubtless responsible for the gold sol - reaction. - - 3. What is the value of grandiose ideas? Ballet distinguishes two - groups of grandiose ideas: (_a_) ideas of self-satisfaction, - including ideas concerning extraordinary capacity, strength, - power, and wealth on the part of the patient; and (_b_) ideas of - ambition; the latter being of a more exact, constant, uniform and - systematizing nature. The more vague and less systematized ideas - of self-satisfaction rest in a phase of contentedness and - optimism; the more definite ideas of pride and ambition are - responsible for striking transformations of personality. General - paresis shows, according to Ballet, these ideas of - self-satisfaction in their most developed form. A certain - variability, absurdity, incoherence, and contradictoriness - characterize these ideas and the patient has little or no insight - into their nature. When such ideas occur at the outset of the - disease, they naturally may be of medicolegal interest. Cotard - explains these ideas of megalomania on the part of paretics on the - ground that they are essentially motor or will disorders and rest - upon a sort of hyperbulia, exhibiting itself in exuberant - activity. Régis has thought that the delusional generosity and - liberality of the paretic, and his willingness to lend his wealth - and talents to social progress, is helpful for diagnosis when - contrasted with the more personal egoism of the victim of - manic-depressive psychosis. The self-satisfaction of the - manic-depressive patient often does not reach a delusional stage, - but remains a mere feeling of pathological well-being or euphoria. - The maniacal patient may compare himself with some great man but - he does not identify himself with him. It must be remembered that - these ideas of self-satisfaction occur also in alcoholism, but - according to Ballet they occur only in the dementing phase of - chronic alcoholism, and have no special diagnostic value. They may - be a clinical stumbling-block for a time in the cases of alcoholic - pseudoparesis. As for the ideas of ambition in which the patients - believe themselves to be princes, emperors, divine messengers, and - the like, these are less characteristic of paretic neurosyphilis - than of delusional psychoses of a non-syphilitic nature. At all - events, such ideas if definite, of long-standing, and systematized - by the patient to form a thorough-going portion of his life, are - not characteristic of neurosyphilis. The victim of paretic - neurosyphilis can as a rule be persuaded out of his delusions, at - least for the time being. These distinctions, it must be added, - are hardly of value in the early cases of any of the psychoses in - question, and cannot be made as a rule in either private or - psychopathic hospital practice. Typical examples of grandiosity, - although not so frequent as might be thought from textbooks, are - always on display in institutions for the chronic insane. - - - =PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like - DEMENTIA PRAECOX. Autopsy.= - - -=Case 12.= Henry Phillips remains a striking case in the memory of those -who knew him and his medical findings. Phillips came to the hospital -voluntarily at 42 years of age from the bank where he worked as a clerk; -he came at the suggestion of his employer. It seems that he had been -annoying his associates because he had fallen into a habit of -continually scratching himself. Phillips was entirely sure that he was -the victim of what he called the “Scotch itch,” and explained off-hand -that this itch had been put upon him by the Free Masons as a matter of -revenge because he would not join their order. He said once, for -example: “At times I feel like raising Hell; then I get a psychic -intimation; and then I get to using a foot-rule on my back and to -slapping my face.” He explained this psychic intimation as coming from -the order of Scottish Rites. Another example of talk is as follows: “My -father is a fighting man; that is part of it. They mean to throw me -down. I am through now trying for membership in the Free Masons. They -have good cause, they must fight. They do not want me for some personal -matters. I can go just so far in agreeing and seconding their advances, -but in the end it fails. I have no strength nor endurance.” - -Aside from these delusions, there was little abnormality to be found, -though his recollection for minor events of the immediate present was -inaccurate. He was rather abnormally impulsive, gesticulating a good -deal while talking, and was of the appearance that the laity call -“nervous.” It appears that he had always been peculiar, subject to -violent fits of temper, in which fits he might throw things at other -members of the family. He always had pronounced likes and dislikes which -he never concealed. He had never had friends, had always been secretive; -and he was often termed a great student. For some five years he had been -studying Japanese from time to time, associating himself with a -Japanese. - -It never does to jump at the diagnosis dementia praecox. However, the -picture seemed characteristic enough for the paranoid form of this -disease. Physically, Phillips had no particular abnormality; the -knee-jerks were a little lively, and the pupils reacted a little -sluggishly. However, the routine W. R. of the serum proved to be -positive. Examination of the spinal fluid was resorted to,—as in all -cases with a positive serum W. R.—and it also proved to be positive and -strongly so; the globulin and albumin were increased, and there was a -pleocytosis. A diagnosis of neurosyphilis was hardly avoidable. Phillips -later admitted a chancre, which he claimed was located on the mucous -membrane of the cheek and acquired by using the same utensils as his -Japanese friend, which friend, he stated, had active syphilis. - -Antisyphilitic treatment of considerable intensiveness was begun, with -intravenous injections of salvarsan and intraspinous injections of -salvarsanized serum, but the patient grew steadily worse. His mental -symptoms became more marked, although not especially characteristic of -general paresis. =Neurologically,= he did develop signs more suggestive -of general paresis, and 18 months later died. - -The =autopsy= showed features of GENERAL PARESIS. It is not necessary to -enter into the question of the details of histological correlation at -this time. - - 1. What conclusion can be drawn from lively knee-jerks? Lively - knee-jerks are of very little significance. Not only certain - neurosyphilitics but also a variety of neurotic persons, victims - of dementia praecox and hysteria, are very prone to have active - tendon reflexes. Of course, extreme degrees of exaggeration are of - importance, and especially an association of the hyperreflexia - with the Babinski reaction, the Gordon, or Oppenheim reflexes, - ankle clonus, and the like. - - 2. Is there any special or differentiating factor in an extragenital - chancre as against a genital chancre? Probably this question - should be answered in the negative. Some have claimed that - chancres draining by lymphatic channels of the head are more - likely to lead to cerebral syphilis. This idea cannot be said to - be established. - - 3. Is there any significance in the story, if true, that Phillips - acquired his syphilis from a Mongolian? It seems to be fairly well - established that syphilis of the nervous system is extremely rare - in China and Japan, whereas bone syphilis is very frequent there. - It has been held that this has to do (_a_) with strains of - spirochetes, (_b_) with the state of civilization, or (_c_) with - the degree of “syphilization.” Apparently when a race is first - infected with syphilis the lesions are chiefly of the cutaneous - and osseous systems; only in later generations the vascular and - nervous systems suffer. However, involvement of the nervous - systems of Mongolians resident in this country is no rarity, a - point possibly in favor of the theory of special strains affecting - the nervous system as prevalent in western countries. Little or - nothing is known as to the effect of transmission from one race to - another, as from Mongolian to Caucasian in Phillips’ story. - - - =NEUROSYPHILIS is NOT to be entirely ruled out by a negative serum - Wassermann Reaction; for the fluid Wassermann Reaction may be - positive.= - - -=Case 13.= William Twist is a case of note in the matter of the -so-called preparetic period (the idea of Charles L. Dana which was -scoffed at when first proposed by him in 1910). The patient, a very -successful traveling salesman, 35 years of age, was admitted to the -Psychopathic Hospital showing a typical picture of general paresis. - -Thus, =mentally=, the patient showed elation, grandiosity (millions of -dollars to give away), intellectual weakness, disorder of memory, lack -of judgment, rambling talk, speech defect, omission of letters in -writing and spelling. - -=Neurologically=, there was tremor of the lips, slight irregularity of -the pupils, which however reacted well, and lively knee-jerks. - -Mr. Twist had sought advice at our out-patient department in his -thirty-third year. The records show that at that time he was somewhat -depressed, and his speech was even then, according to his own statement, -stammering. However, we found the W. R. at that time to be negative in -the blood serum. It appeared that his mother had died of consumption; -his father was said to have committed suicide. A brother had once -recovered from an attack of depression, presumably an attack of -manic-depressive psychosis. Accordingly, we thought at the time that the -case was probably one of manic-depressive psychosis. Moreover, our -routine serum W. R. failed to indicate any syphilitic process. As for -the so-called stammering of speech, this appeared to be a matter of the -patient’s own recollection rather than of our observation. In any event, -the patient had gone into the country and appears to have entirely -recovered; falling, again, however, into mental difficulties after a -short period, and finally arriving at the hospital in the -above-mentioned classical condition. - -The W. R. in the blood serum proved again negative. The test was -repeated a number of times; also, after salvarsan had been given. The -salvarsan did not act provocatively, and the blood serum has remained -consistently negative. - -In cases of syphilis the W. R. is at times negative. Swift claims that -in such cases an injection of salvarsan will often produce a positive W. -R. if the blood is tested on several days following the injection. - -The spinal fluid, however, did show a positive W. R. as well as a gold -sol reaction of a “paretic” type. There were at the first examination -194 cells per cmm., there was a moderate excess of albumin, and a -positive globulin test. In short, there was no question of any other -diagnosis than GENERAL PARESIS. - - 1. How can the negative W. R. of the blood serum be explained? It is - difficult or impossible to explain this. Figures differ as to the - percentage of cases of general paresis with negative blood serum; - perhaps 3 to 5% of these cases yield a negative serum W. R. - - It is important to note the long preparetic period: at least a year - and a half. Could our diagnostic methods be sharpened a trifle, - such cases as these could be obtained early in this preparetic - period and it might then be safe to promise good therapeutic - results. - - 2. What is the nature of the preparesis of Dana? When Dana’s brief - paper on preparesis was written, there was of course hardly any - idea that cases of paretic neurosyphilis could be cured or would - recover, except possibly vanishingly few _curiosa_ about which - there would always rage a diagnostic question. Accordingly, Dana, - having found certain cases that seemed to him to have early signs - of paresis but had apparently been cured by treatment, proposed to - call them cases of preparesis. His idea was that he would thereby - not offend those who held that general paresis was theoretically a - fatal disease. With modern work and the display of more and more - atypical cases of neurosyphilis, and the observation of relatively - numerous cures or remissions under treatment, the designation of - preparesis for a separate entity, or even for a sub-form of - neurosyphilis, becomes superfluous. - - 3. What is the percentage of cases of paretic neurosyphilis that - show a negative serum W. R.? Among the best figures are those of - Müller, who found that of 386 examples of paretic neurosyphilis, - 379 showed all reactions positive, or 98.5%. - - 4. What is the meaning and value of the so-called provocative - salvarsan injection? In practice, there may be a series of - negative W. R.’s in the blood serum before a positive reaction is - finally obtained, owing to technical difficulties or biological - peculiarities. Where intensive work is being done upon the - neurosyphilis problem, it is beyond question desirable to make the - W. R. test upon at least three separate samples of blood drawn at - intervals, for the second or third test may prove positive. This - situation makes the interpretation of the so-called provocative - salvarsan injection exceedingly doubtful; that is, the reaction - might have been positive on repetition without the injection of - salvarsan. The present case, as above stated, failed to yield a - serum W. R. even after repeated tests and the “provocative.” - - 5. What is the significance of the irregular pupils in this group? - Paretic neurosyphilis shows inequality of the pupils in a high per - cent of cases. Irregularity of outline of the pupils is commonly - thought to be an important sign and to suggest neurosyphilis. It - is true that many cases of pupillary irregularity are syphilitic, - but the sign is of little or no differential value since - congenital malformations and relics of old injuries and adhesions - may produce effects identical with those of neurosyphilis. - - - =DIFFUSE (that is, meningovasculoparenchymatous[5]) NEUROSYPHILIS is - typically associated with six positive tests (serum Wassermann - reaction, fluid Wassermann reaction, spinal fluid gold sol reaction, - pleocytosis, positive globulin, excessive albumin); but one or more, - and frequently several, of these tests are likely to run mild as - compared with the tests in PARETIC NEUROSYPHILIS (“general - paresis”). The clinical course of the diffuse (and especially the - meningovascular) cases is likely to be protracted, with a good - prognosis as to life (barring fatal vascular insults).= - - -=Case 14.= We shall present the case of John Jackson, a surveyor, 31 -years of age, suffering from a left hemiplegia, with this in mind: To -exhibit difficulties in diagnosis in the presence of an embarrassment of -symptomatic riches. - -The patient arrived at the hospital, in the first place, because he had -been threatening a woman who lived next door to him. He believed that -this neighbor had been talking about him and circulating reports against -him. Excited by these ideas, he had threatened to cut her throat. - -Now the occurrence of hemiplegia in adult life before the approach of -senium is always suspicious of syphilis, and this suspicion we naturally -entertained from the beginning. However, there was upon the scalp a -crooked linear furrow about six inches long, running from the vertex to -the right parietal eminence. Another furrow about an inch long was -present upon the forehead. These furrows appeared to be of a bony nature -and were not tender. There was evidence of an old decompression -operation on the right side of the head; there were also large scars on -both sides of the neck, evidently the result of old operations; and -there were numerous palpable glands—the largest about the size of a lima -bean—all firm and not tender. - -[Illustration: - - Station in syphilitic hemiplegia. Syphilitic pigmentation of skin. -] - -It seems that at the age of eight, according to the patient’s mother, -Jackson had received a head injury and had remained unconscious for -three weeks. Upon recovery, he had to relearn both to walk and to talk; -however, he was able to begin school where he left off. He became more -nervous and irritable after the accident than previously. Nothing -further had developed until, at about 25 years of age, a tubercle was -discovered in his eye (the right pupil was smaller than the left, -reacting more slowly; right iris bound down by adhesions, with white -opacity of anterior chamber). For two years, 25 to 27, the patient was -under medical treatment for tuberculosis, and at the conclusion of this -period numerous glands were removed from the neck and diagnosticated -tuberculous. However, the neck did not heal and he carried bandages upon -it for two years. - -At 28, the patient’s mother described the occurrence of a slight shock, -with head retraction, for a minute or two, and inability to speak. -Thereafter there had been five or six similar attacks, less severe, and -without loss of speech. The attacks were never accompanied by convulsive -movements. Then occurred a paralytic stroke, leaving the patient with a -left hemiplegia, which had somewhat improved. Mentally, the patient had -gone down hill, becoming less alert and more apathetic, and to some -extent amnestic. One had to consider, accordingly, the somewhat doubtful -possibility of post-traumatic and post-operative conditions, and the -question of tuberculosis (possibly errors in diagnosis; the lungs showed -no evidence of tuberculosis). - -=Physically,= the signs of a left hemiplegia were appropriate. -Spasticity on the left side was found; there were Babinski, Gordon, -Oppenheim reflexes and ankle clonus on the left side (all absent on the -right). Speech defect was present. =Mentally,= aside from the delusions -noted at the beginning of our analysis, a striking feature was the -patient’s childishness. While reciting delusions, the patient was -overactive and evinced a somewhat childish interest. Arithmetically, -Jackson had preserved a fair ability but his apathy and lack of interest -interfered with tests, and possibly also with the exercise of memory. As -above noted, we were compelled to maintain the suspicion of syphilis -throughout despite the attractive hypotheses of traumatic and -post-decompressive effects and cerebral tuberculosis. A history of the -acquisition of syphilis an unknown number of years before admission -entered to strengthen the suspicion of the syphilitic nature of the -mental symptoms. - - - TYPICAL LABORATORY FINDINGS IN NEUROSYPHILIS (NONNE, 1915) - ─────────────┬─────────┬─────────┬─────────┬───────────────┬─────────── - DIAGNOSIS │ W. R., │ W. R. │ SPINAL │ PHASE I, │PLEOCYTOSIS - │ BLOOD │0.22 CC. │ FLUID, │ GLOBULIN │ - │ SERUM │ BLOOD │ 1.0 CC. │ │ - │ │ SERUM │ │ │ - ─────────────┼─────────┼─────────┼─────────┼───────────────┼─────────── - PARESIS OR │POSITIVE │POSITIVE,│POSITIVE,│POSITIVE, │POSITIVE, - TABOPARESIS│ IN │ 85–90% │ 100% │ 95–100% │ ABOUT 95% - │ ALMOST │ │ │ │ - │ 100% │ │ │ │ - │ │ │ │ │ - TABES (not │POSITIVE,│POSITIVE,│POSITIVE,│POSITIVE, │POSITIVE, - combined │ 60–70% │ 20% │ 100% │ 90–95% │ 90% - with │ │ │ │ │ - paresis) │ │ │ │ │ - │ │ │ │ │ - CEREBROSPINAL│POSITIVE,│POSITIVE,│POSITIVE │POSITIVE almost│POSITIVE - SYPHILIS │ 70–80% │ 20–30% │ ALMOST │ always; │ ALMOST - │ │ │ ALWAYS │ NEGATIVE only│ ALWAYS - │ │ │ │ EXCEPTIONALLY│ - ─────────────┴─────────┴─────────┴─────────┴───────────────┴─────────── - CHART 8 - - -[Illustration: - - Syphilitic thrombosis. Contours of brain preserved. -] - -The W. R. proved positive in blood and spinal fluid. The gold sol -reaction was of the syphilitic type; 37 cells were found per cmm.; there -was a slight amount of globulin and a slight excess of albumin. - -We made a diagnosis of CEREBROSPINAL SYPHILIS rather than general -paresis on account of, first, the slow course of the disease; second, -the vascular type of the cerebral insult, hardly typical of paresis; and -third, the mild spinal fluid reaction. Treatment will hardly cure the -hemiplegia, at least so far as restoration of cerebral tissues lost in -the insult is concerned. We were perhaps entitled to consider that, as -in the cases of Petrofski (17), O’Neil (19), Robinson (45), the -meningitic process could be arrested. Unfortunately, our treatment of 20 -injections of salvarsan over a period of 10 weeks, followed by a number -of months of bi-weekly injections of mercury salicylate, proved -incapable of making any change in the mental and physical picture or in -the laboratory findings. - - 1. Can we explain the apparently poor reaction to treatment of the - cerebrospinal syphilis in the case of Jackson by supposing a more - deep-seated involvement than the meningovascular involvement - indicated by the hemiplegia and the signs in the fluid? Autopsied - cases in our experience show focal parenchymatous involvements - that have not caused obvious clinical symptoms at any time during - the course of the disease. These symptomatically silent lesions - may have been present. - - 2. What is the comparative prognostic value of seizures in paretic - neurosyphilis and in such a meningovascular case as that of - Jackson? Paretic seizures are often and indeed characteristically - recovered from. Moreover, autopsies in paretic neurosyphilis - characteristically show no gross focal destructive lesions to - correspond with the seizures. The paretic seizures are apparently - more irritative than paralytic. However, the seizures of the - meningovascular group of neurosyphilis are also, though less - commonly, recovered from, so that the differential diagnosis on - the basis of the outcome of seizures is not safe. Rarely paretic - neurosyphilis itself also develops seizures from which no recovery - is made. - - 3. What is the relation of neuropathic heredity to neurosyphilis? - The family history of John Jackson is undoubtedly poor, since his - father died of diabetes and a paternal uncle was insane; and on - the mother’s side, the grandmother died of tuberculosis and an - aunt died insane. This general question was more interesting in - the days before the syphilitic nature of general paresis and of - allied diseases was known. However, we may still hold perhaps that - not only syphilis but also various intoxications, especially - alcoholism, do flourish upon a neuropathic soil. This question, - like that of Krafft-Ebing’s celebrated claim of the relation - between syphilization and civilization, needs revision in the - light of more extensive applications of the W. R. in larger and - larger groups of persons under various community conditions. - - - =The SIX TESTS (serum Wassermann reaction, fluid Wassermann - reaction, pleocytosis, gold sol reaction, globulin, excess albumin) - are likely to run STRONGER in PARETIC NEUROSYPHILIS (“general - paresis”) than in DIFFUSE (especially meningovascular) - NEUROSYPHILIS; in particular, the gold sol reaction is likely to - prove “paretic” rather than “syphilitic.” The clinical course of - paretic neurosyphilis (“general paresis”) is likely to terminate in - death within a few years.= - - -=Case 15.= Pietro Martiro was a well developed and nourished man, 30 -years of age, who had been doing erratic things and acting peculiarly -for a few weeks before entering the hospital. In the hospital, Martiro -proved to be very excitable and given to violence. He had marked -delusions of grandeur, saying he was worth many millions of dollars, was -the greatest singer in the world, the greatest athlete in the world, and -the like. - -=Physically=, there was no disorder except overactivity of some -reflexes. The diagnosis of GENERAL PARESIS offered no difficulties, and -it was confirmed by the laboratory tests (positive serum and fluid W. -R., “paretic” gold sol reaction, 42 cells per cmm., an excess of -albumin, and a positive globulin test). - -=Treatment=: The perfect physique of this case and the extremely brief -clinical duration (a few weeks) would naturally suggest a probably -favorable outcome. However, cases with marked delusions of grandeur have -very frequently proved to be cases with extensive brain tissue loss as -shown in certain studies with Danvers material. - -In any event, the treatment in this case proved unavailing. Enormous -doses of salvarsan, twice a week, aided by mercury and potassium iodid, -were given. Although other cases had been helped by such intensive -treatment, Martiro went steadily downhill, nor was there the slightest -diminution in the intensity of any of the spinal fluid reactions. After -50 injections of salvarsan over a period of 30 weeks without -improvement, treatment was discontinued. A few months later, the patient -died. - - - =PARETIC NEUROSYPHILIS (GENERAL PARESIS)= - - =PHYSICAL SYMPTOMS= - - EARLY HEADACHE - VISUAL DISORDER - HYPALGESIA - ADIADOCHOKINESIS - ATAXIA - NASOLABIAL FLATTENING - VOCAL CHANGE - SPEECH DISORDER - WRITING DISORDER - LOSS OF MANUAL DEXTERITY - PUPILLARY CHANGES - REFLEX CHANGES - SEIZURES - LATE: PARALYSIS, CONTRACTURE - - CHART 9 - - - =PARETIC NEUROSYPHILIS (GENERAL PARESIS)= - - =MENTAL SYMPTOMS= - - INTAKE IMPAIRED - CONSCIOUSNESS CLOUDED - FATIGUABILITY INCREASED - HALLUCINOSIS RARE - AMNESIA—RECENT! CHRONOLOGY AND STORAGE IMPAIRED. FABULATION - OVER-SUGGESTIBILITY - JUDGMENT IMPAIRED - FANTASTIC DELUSIONS - INSIGHT INTO ILLNESS NIL - EARLY IRRITABILITY OR HEBETUDE - QUICK SHIFTING EMOTION - CHARACTER CHANGE - CONDUCT SLUMP - - CHART 10 - - - 1. What is the duration of paretic neurosyphilis (“general - paresis”)? If we omit the doubtful, early, and prodromal stages - and count the beginning of the disease with the occurrence of - definite symptoms, we find (Kraepelin) that almost half the - patients with pronounced paretic signs die within the first two - years of their disease. Kraepelin’s observations upon 244 cases - are as follows: - - Year: 1 2 3 4 5 6 7 8 9 10 14 - Cases: 51 63 52 41 22 4 5 2 2 1 1 - - The average duration of the disease in months has been calculated - as varying from 24 to 32 months. Juvenile paresis runs a slower - and more insidious course. The duration of paresis, according to - many observers, diminishes with the increasing age of the patient. - It is now held that a combination of tabes with paresis does not - prolong the duration of the paresis. As noted above in the - discussion of Case Harrison (9), our conceptions of the - characteristic duration of paretic neurosyphilis must alter with - the increase of our knowledge due to the early application of - laboratory tests. - - 2. What is the significance of the term _general paresis_? The case - of Martiro is, of course, a good instance to show that the term is - sometimes a misnomer. The characteristic generalized motor - incapacity denoted by the term _general paresis_ is shown in - patients in the institutions for the chronic insane in their last - few months of life. The term _paresis_ is perhaps to be preferred - to the term _paralysis_ because the paralysis is not complete but - partial; but perhaps the best reason is that the word _paresis_ is - a shorter word. When the mental side is to be emphasized, the term - _paralytic dementia_ is employed. In this book we have used the - term _paretic neurosyphilis_ to mean a more precise statement of - the etiology of general paresis (general paralysis, paralytic - dementia). The lay term, _softening of the brain_, like the terms - _metasyphilis_ and _parasyphilis_ is in the present phase of our - knowledge to be eschewed. - -[Illustration: - - Euphoria in paretic neurosyphilis (“general paresis”). The head, arms - and trunk were shaking with mirth; hence, the indistinct outlines of - the photograph. -] - - 3. If this fatal case be typical of general paresis (for more - favorable results, see Part V), what is the toll of deaths from - this disease in the community at large? A striking statement may - be quoted from Dr. Thomas W. Salmon’s “Analysis of General - Paralysis as a Public Health Problem:” - - “With the information in our possession at the present time, we - are able to state that not fewer than 1000 persons in whom general - paralysis is recognized die in New York State every year. Let us - compare this with the lives lost from some other important - preventable diseases. It means that _one in nine_ of the 6909 men - who died between the ages of 40 and 60 in New York last year died - from recognized general paralysis and that _one in thirty_ of the - 5299 women who died in the same age-period died from this disease. - - “The number of deaths from general paralysis in New York last year - about equalled the number of deaths from typhoid fever. The - following table gives the number of deaths due to the ten most - important specific infectious diseases. Of course, deaths in - measles, typhoid fever and scarlet fever will be found also under - the names of some of the complications of these diseases, but it - should be remembered that these primary diseases are not - invariably fatal as general paralysis is. Many of the patients - with measles who died from bronchopneumonia would have recovered - but for this complication, while the paretics with - bronchopneumonia would have died even if this complication had not - arisen. No attempt is being made to compare the _prevalence_ of - general paralysis with that of other diseases—we are trying only - to estimate its share in the _mortality_. - - “1. Tuberculosis (all forms) 16,133 - 2. Pneumonia 9,302 - 3. Bronchopneumonia 7,217 - 4. Diphtheria and croup 1,854 - 5. Influenza 1,381 - 6. Measles 1,071 - 7. Typhoid Fever 1,018 - _General paralysis (recognized)_ 1,000 - 8. Scarlet fever 837 - 9. Whooping cough 818 - 10. Syphilis 782” - - - =PARETIC NEUROSYPHILIS (GENERAL PARESIS)= - - =CHARACTERISTICS= - - AMNESIA - QUICK SHIFTING EMOTIONS - CHARACTER CHANGE - CONDUCT SLUMP - NERVOUS DISORDERS - SPEECH DISORDERS - PUPILLARY CHANGES - REFLEX CHANGES - SEIZURES - CEREBROSPINAL FLUID PICTURE - - CHART 11 - - - =SYPHILITIC PSYCHOSES= - - SYPHILITIC NEURASTHENIA - GUMMA - SYPHILITIC PSEUDOPARESIS - APOPLECTIC CEREBRAL SYPHILIS - SYPHILITIC EPILEPSY - SYPHILITIC PARANOIA - TABETIC PSYCHOSIS - HEREDITARY - PARESIS - - KRAEPELIN, 1910 - - CHART 12 - - - =TABOPARETIC NEUROSYPHILIS (“taboparesis”) is CLINICALLY a - combination of the symptoms of TABES DORSALIS and those of GENERAL - PARESIS. The COURSE of TABOPARESIS is likely to be from a - characteristic tabes dorsalis (often of years’ standing) to a - characteristic general paresis; the ultimate paretic picture is - likely to retain various characteristics of tabes. The LABORATORY - TESTS in the paretic phase are characteristic of ordinary - (non-tabetic) general paresis. The PROGNOSIS, after the paretic - phase has arrived, is apt to be that of general paresis.= - - -=Case 16.= Joseph Sullivan, a waiter, 50 years of age, sought assistance -at the Psychopathic Hospital voluntarily. His complaint of severe and -lancinating pains in the legs, difficulty with his gait, and a feeling -of constriction about the waist, was forthwith suggestive of tabes -dorsalis. He was a rather poorly nourished, white-haired man, with a -drooping of the left side of the face. The pupils reacted sluggishly to -light, the right somewhat better than the left. A marked Romberg -reaction could be demonstrated. Ataxia in walking was marked. There was -some incoördination of the hands, considerable tremor, and writing was -poorly performed. The ankle-jerks and knee-jerks were absent. On the -whole, the diagnosis of TABES DORSALIS was clear enough. - -The most appealing situation was =mental=. Sullivan was exceedingly -apprehensive about his condition on the ground that it was growing -progressively worse; if it was to get worse, Sullivan feared he would -commit suicide. From his own account, he had become irritable, -quick-tempered, and often unreasonable. As usual in these cases, the -question arose whether the depression was psychopathic or natural. - - - =TABETIC SYMPTOMS AND SIGNS IN ORDER OF THEIR FREQUENCY= - - =ANALYSIS OF 250 CASES= - - PER CENT - - 1. ROMBERG SIGN 96.4 - 2. ABSENT KNEE-JERKS 90.0 - 3. LANCINATING PAINS 88.4 - 4. STAGGERING GAIT 87.2 - 5. ARGYLL-ROBERTSON PUPIL 80.0 - 6. ATAXIA IN UPPER EXTREMITIES 68.2 - 7. SPHINCTER DISTURBANCES 67.6 - 8. SENSORY DISTURBANCES 58.2 - 9. VISUAL DISTURBANCES 43.6 - 10. PARESTHESIA AND NUMBNESS OF FEET AND LOWER EXTREMITIES 42.8 - 11. GIRDLE SENSE 31.2 - 12. PTOSIS OF EYE-LIDS 23.2 - 13. PARESTHESIA OR NUMBNESS IN HANDS OR UPPER EXTREMITIES 13.6 - 14. STRABISMUS 12.0 - 15. VISCERAL CRISES 12.0 - 16. LOSS OF SEXUAL DESIRE 11.5 - 17. CHARCOT JOINTS 9.2 - 18. VERTIGO 4.0 - 19. MAL PERFORANS 3.2 - 20. PAIN IN JOINTS 2.8 - 21. RECTAL TENESMUS 2.8 - 22. MENTAL DEGENERATION (other than paresis) 2.4 - 23. HEMIPLEGIA 2.4 - 24. VESICAL TENESMUS 2.0 - 25. DIFFICULTY IN ARTICULATION 2.0 - 26. DEAFNESS 1.2 - 27. ANOSMIA 0.8 - - BALDWIN LUCKE. - - CHART 13 - - -While in the hospital things shortly came to a crisis. In the midst of a -fit of depression, Sullivan attempted suicide by beating his head -against the wall. Whether this attempt could be regarded psychopathic, -however, remained in question. Sullivan had been drinking very heavily -although he had stopped about six weeks before admission, fearing that -the alcohol was causing a development of symptoms. The remedy was almost -worse than the disease because he then became more nervous, lost his -appetite, and had a marked insomnia. - -According to the patient’s own history, he had had several attacks of -gonorrhœa and a syphilitic infection at the age of 19; that is, some 31 -years before admission to the hospital. However, the first -_neurological_ symptoms of which the patient was aware came about 27 or -28 years after infection, namely, 3 or 4 years before admission, when -facial paralysis developed. At that time, he had suddenly felt a -peculiar sensation in the throat and became unable to swallow for a -time. His voice remained hoarse and low for some time, and his face -began to droop. The lancinating pains and the ataxia also dated back -several years. - - 1. How shall we evaluate the mental symptoms? The prognosis of tabes - dorsalis is relatively good so far as life is concerned, and it - might even be possible for Sullivan by training to remain capable - of being a waiter. The manual incoördination was not marked, and - possibly the manual tremor was in part due to alcohol. - Accordingly, the mental symptoms, such as emotional lability and - memory defect, were in the foreground of attention. In point of - fact, the laboratory examinations showed positive W. R. in the - serum and the spinal fluid, which latter also contained 60 cells - per cmm., positive globulin, and an excess of albumin. THE - DIAGNOSIS MADE WAS THAT OF TABOPARESIS, meaning thereby a tabes - associated with appropriate symptoms of a mental nature. - - 2. How shall the term _taboparesis_ be used? Some use the term, as - we feel erroneously, for instances of general paresis which happen - to show crural areflexia (absence of knee-jerks). We feel that the - best usage of the term is for instances in which well-defined - symptoms of tabes (as well as of paresis) are present, namely, - characteristic ataxia, lightning pains, and the like. If the term - is used more loosely, as above mentioned, then practically every - case of general paresis might perhaps be termed _taboparesis_, - since almost every case of paresis does show involvement of the - cord as well as of the cerebrum. Such involvement may lead to - hyperreflexia, hyporeflexia, or areflexia according to the - localization of the process. In true taboparesis, in which there - is a commingling of the features of tabes with those of paresis, - we should find the posterior roots of the spinal cord affected. - The spinal lesions of paresis itself are more apt to be - intraspinal; that is, confined to the nervous system within the - pial investment. - - 3. Bearing in mind that Sullivan was a waiter, what shall be said - about the infectivity of these cases? It is counted as a rule as - negative, since there are no open spirochete-bearing lesions. The - longer the period since infection the less, as a rule, is the - chance of contagion in syphilis; and as tabes and paresis occur - fairly late in the disease, the infectiousness at this stage is - practically negligible. - - 4. Of what differential value is the insight shown by Sullivan into - the nature of his symptoms? Kraepelin remarks that a genuine - insight into the nature of the disease does not as a rule occur in - paresis. At the beginning of the disease, there may sometimes be a - correct understanding of the nature of the disease and of its - probable outcome; but the presence or absence of insight into the - fact of mental disease is by no means a differential sign of - practical value. - - 5. What is to be said of the occurrence of depression and excited - states in paretic neurosyphilis? A variety of classifications of - sub-forms of paretic neurosyphilis have been propounded. - Kraepelin, for example, deals with four: the demented, depressive, - expansive, and agitated forms, but remarks that the division is - merely convenient for exposition. The institutional intake does - not accurately represent the distribution of cases. Under - psychopathic hospital conditions with the relatively easy resort - to such institutions, the number of quiet cases increases; under - the less advanced conditions in Heidelberg, Kraepelin took in 53% - demented paretics as against 56% at Munich (73% women) under the - easier conditions of admission. The admissions of demented - paretics varied from 37 to 56%. The variations depend much upon - the facility with which the cases can be brought to institutions. - Where admission is beset with various legal restrictions, the - quiet and demented cases are more apt to be treated for long - periods at home. The depressive type of paretic neurosyphilis - forms a much smaller group, according to Kraepelin, as only about - 12% of his Heidelberg admissions were of this type, and still - fewer of his Munich admissions. Other authors give percentages as - high as 16 and 19. The so-called expansive group is larger, - Kraepelin finding 30% of his Heidelberg cases to be of this group, - and 21 to 22% of his Munich cases. The rarest sub-form of paretic - neurosyphilis is the agitated form: 6% of Kraepelin’s Heidelberg - admissions; 14% among males and 5% among females in his Munich - admissions, where the diagnosis of agitated paresis was entered on - somewhat broader lines. French authors (Sérieux and Ducaste) have - enlarged the number of sub-forms of paretic neurosyphilis as - follows: Expansive 27%; sensory 24%; demented 24%; persecutory 3%; - depressive 2%; circular 7%; hypochondriacal 7%; and maniacal 6%. - - - =DIFFUSE (meningovasculoparenchymatous) NEUROSYPHILIS may look - precisely like PARETIC NEUROSYPHILIS (“general paresis”) at certain - periods of clinical and laboratory examination.= - - -=Case 17.= The police found Gregorian Petrofski crouching on his knees -on a Boston sidewalk, attempting to take pickets off a fence. Petrofski -knew little English; he said that he had slept in Poland the night -before. He did not appear to be alcoholic. - -When he was examined, through an interpreter, he told how he had been in -America two days, and in Boston two years; that he was at the present -time in Poland, and that his brother had brought him to the hospital and -left him there. - -The =physical examination= showed Petrofski to be well developed and -nourished. His pupils were somewhat dilated and reacted somewhat slowly -to light and accommodation. =Neurologically,= there was nothing else -abnormal found upon systematic examination although, through lack of -coöperation, sensory and coördination tests proved difficult if not -impossible. There was a large ulcer on the under surface of the glans -penis, with several small smooth scars on the upper surface. There was a -purulent discharge from the external meatus. There were exostoses of -both tibiae. - -The initial diagnosis had to consider uremia and diabetes, which could -be easily excluded on examination. Alcoholism was excluded through -absence of alcohol on the breath. There remained such diagnoses as -epilepsy, some post-traumatic condition, or meningitis, to say nothing -of the hypothesis of syphilis raised by the tibial exostoses and the -lesions of the penis. The hypothesis of trauma was given up, as well as -epilepsy and meningitis upon the data of the lumbar puncture. The spinal -fluid proved to be clear but with enormous amounts of globulin and -albumin, 80 cells per cmm., a “paretic” gold sol reaction, and a -positive spinal fluid W. R. (the serum W. R. was also positive). -Accordingly, it was clear that the case was one of neurosyphilis. - -Treatment was instituted with injections of mercury salicylate, a grain -and a half twice a week, and potassium iodid. After some weeks, -diarrhoea and salivation with marked symptoms of mercury poisoning set -in; the treatment was suspended, but later re-instituted. In a few weeks -Petrofski was apparently quite well, the spinal fluid tests had all -become negative, as had the serum W. R. - -Petrofski now began to pick up a good deal of English, and gave a -consistent narrative of his past life, although the period just prior to -and during his early stay in the hospital has remained blank. Without -further treatment Petrofski has remained well for over a year. - - 1. Does the “paretic” gold sol reaction mean general paresis? In - connection with this general question, a brief summary of the - significance of the gold sol reaction in this group may be made. - (1) Fluids from cases of general paresis in the vast majority of - cases will give a strong and fairly characteristic reaction, - especially if more than one sample is tested. (2) Very rarely - general paresis fluid will give a reaction weaker than the - characteristic one. (3) Fluids from cases of syphilitic - involvement of the central nervous system other than general - paresis often give a weaker reaction than the paretic, but in a - fairly high percentage of cases give the same reaction as the - paretics. (4) Non-syphilitic cases may give the same reaction as - the paretics; these cases are usually chronic inflammatory - conditions of the central nervous system. (5) When a syphilitic - fluid does not give the strong “paretic reaction” it is - presumptive evidence that the case is not general paresis, and - this test offers a very valuable differential diagnostic aid - between general paresis, tabes, and cerebrospinal syphilis. (6) - The term “syphilitic zone” is a misnomer, as non-syphilitic as - well as syphilitic cases give reactions in this zone, but no fluid - of a case with syphilitic central nervous system disease has given - a reaction out of this zone, so that the finding may be used - negatively; and any fluid giving a reaction outside of this zone - may be considered non-syphilitic. (7) Mild reactions may occur - without any evident significance, while a reaction of no greater - strength may mean marked inflammatory reaction. (8) Tuberculous - meningitis, brain tumor, and purulent meningitis fluids - characteristically, though not invariably, give reactions in - higher dilutions than syphilitic fluids. (9) The unsupplemented - gold sol test is insufficient evidence on which to make any - diagnosis, but used in conjunction with the W. R., chemical and - cytological examinations, it offers much information, aiding in - the differential diagnosis of general paresis, cerebrospinal - syphilis, tabes dorsalis, brain tumor, tuberculous meningitis, and - purulent meningitis. (10) We believe that no cerebrospinal fluid - examination is complete for clinical purposes without the gold sol - test. - - - =FREQUENT SYMPTOMS IN DIFFUSE AND VASCULAR NEUROSYPHILIS= - - =(“CEREBRAL” AND “CEREBROSPINAL SYPHILIS”)= - - PUPILLARY DISORDER - HEADACHE - VERTIGO - INSOMNIA - DROWSINESS - CHANGE IN DISPOSITION - Irritability Slow thinking - SEIZURES - PARALYSES - Permanent Transient - APHASIA - HEMIANOPSIA - SENSORY DISTURBANCES - GASTRIC CRISES - SPHINCTER DISTURBANCES - INTRACRANIAL PRESSURE SYMPTOMS - POLYURIA, POLYDIPSIA, GLYCOSURIA - MÉNIÈRE’S SYNDROME - NYSTAGMUS - - CHART 14 - - - See Appendix B for technical details. - - 2. What is the relation of the tibial exostosis to neurosyphilis? - The syphilographers have always stressed the tibial lesions in the - diagnosis of syphilis. Although not so much attention has been - paid to these and kindred osseous lesions in neurosyphilis, yet we - have frequently found such lesions and they afford an important - auxiliary means of diagnosis. - - - =A POSITIVE SERUM Wassermann reaction with a NEGATIVE FLUID - Wassermann Reaction may be found in NEUROSYPHILIS, particularly in - VASCULAR NEUROSYPHILIS: the remaining signs in the fluid, although - frequently positive, may even be negative.= - - -=Case 18.= Frederick Wescott was a promoter, an elderly looking man of -60 years. His health had been failing for 18 months. There had been -shortness of breath, dizziness, a tired feeling, inability to “get the -words he wanted,” and forgetfulness of names. About eight weeks before -examination, Wescott had had a convulsion, following which he had been -unable to express himself at all well. This convulsion was not -accompanied by loss of consciousness. Besides a marked motor aphasia, -there was agraphia. - -=Physically=, Wescott showed arteriosclerosis and a blood pressure of -135 systolic, but, except very lively knee-jerks, no other reflex -disorders or anomalies were discovered. In particular, the pupils -reacted fairly well. - -There was, perhaps, no special reason to implicate syphilis in the case, -yet Wescott gave a history of syphilis at 35 years. The W. R. of the -blood serum proved positive; that of the spinal fluid was negative, and -the albumin was but slightly increased; there was a very slight amount -of globulin, and there were 16 cells per cmm. in the fluid. The gold sol -reaction suggested syphilis. - -We felt entitled to make a diagnosis of SYPHILITIC CEREBRAL -ARTERIOSCLEROSIS, regarding the convulsion or seizure eight weeks before -as due to a vascular insult. The laboratory picture in the spinal fluid -in Wescott’s case seems to be rather characteristic of this group of -syphilitic arteriosclerotics. - - 1. What is the reason for the negative spinal fluid W. R.? The - theory would be that the syphilitic lesion is localized in the - vascular system and that the parenchyma is only secondarily, if at - all, involved. The W. R. producing bodies are accordingly not - found in the fluid. - - 2. How frequently are several of the spinal fluid tests negative, - while others are positive? Whereas, clinically speaking, the five - tests in the spinal fluid (W. R., globulin reaction, excess - albumin, pleocytosis, and gold sol reaction) are each indicative - of a pathological condition in the central nervous system, yet a - specially intensive study of the distribution of these tests has - shown that they are prone to occur independently. Consequently, we - must concede that they do not all represent the same inflammatory - products and chemical conditions. The W. R. producing bodies, the - gold sol reaction producing bodies, as well as the globulins and - albumins, have been proved to be separate. Special work has also - shown that _these tests disappear under treatment at different - rates_. There is, unfortunately, no doubt that the rate and - intensity, presence or absence, and the order of disappearance of - these tests in either treated or untreated cases, do not at all - parallel the clinical conditions of the patients. - - 3. What is the prognosis in vascular neurosyphilis, such as in the - case of Wescott? The prognosis is identical with that of cerebral - arteriosclerosis in general, that is to say, bad, but with - frequent periods of improvement. In the neurosyphilitic type of - arterial disease thromboid formation is frequent. Where the lesion - is chiefly perivascular infiltration, rather than disintegration - of the vessel wall, improvement may very well occur as a result of - treatment. Wescott showed slight improvement under treatment. He - has already lived two years since his first convulsion, and three - and a half years since the onset of symptoms. - - - =DIFFUSE NEUROSYPHILIS (so-called “cerebrospinal syphilis”) is often - marked by SEIZURES.= - - -=Case 19.= Agnes O’Neil, an unmarried woman of 28 years, was first -examined five weeks after the initial symptoms. It appears that she had -had certain seizures, with unconsciousness and twitching of the limbs -(otherwise not well described), followed by confusion of mind and -sometimes by a weakness of the left side and a difficulty in speaking. -Headache had been almost constant, as well as pains in the arms and -legs. - -=Physically=, both in general and =neurologically=, there were no signs -or symptoms; mentally, we could discover no symptoms. Syphilis was -denied, although possible exposure to syphilis was admitted. - -The =diagnosis= of some form of organic brain disease was clear with the -picture of convulsions followed by slight aphasia with headaches and -limb pains. With onset at 28, the most frequent cause for such -epileptiform seizures is certainly syphilis. Examination of the blood -and spinal fluid showed a positive W. R., in both. The albumin was also -somewhat increased. The clinical picture suggested a fairly generalized -meningitic involvement. - -The =prognosis= in such cases of generalized meningitic involvement is -in general good, and this principle was illustrated in the O’Neil case, -in which the symptoms soon disappeared under intensive antisyphilitic -treatment. In fact the spinal fluid W. R. became negative in the course -of four weeks. The blood serum W. R., however, has remained positive -despite eight months of active treatment. - - - =CONDITIONS IN WHICH CONVULSIONS OCCUR= - - NEUROSYPHILIS - HYSTERIA - EPILEPSY MAJOR (GRAND MAL) - EPILEPSY MINOR (PETIT MAL) - DEMENTIA PRAECOX - TOXIC CONDITIONS: - Asphyxia, Uremia, Alcohol, Absinthe, Lead, Mercury, etc. - ORGANIC BRAIN LESIONS - Apoplexy, Meningitis, Intracranial Growths - STOKES-ADAMS DISEASE - MALINGERING - DISSEMINATED SCLEROSIS - - CHART 15 - - - 1. Are certain cases of syphilitic epilepsy really cases of - Jacksonian epilepsy? As a matter of nomenclature, Jacksonian - cortical epilepsy is usually the result of a focal and - circumscribed irritative lesion in the cortex. Gumma, local - syphilitic meningitis, and syphilitic vascular lesions, as well as - scars consequent upon the latter, are among the causes of - Jacksonian epilepsy, along with such other focal lesions as - trauma, tumor abscess, tubercle, and the like. Even non-syphilitic - Jacksonian epilepsy has been observed from time to time in cases - of diffuse intracranial pressure. Jacksonian attacks also have - been found in so-called genuine epilepsy. Accordingly, we must not - conclude from the occurrence of Jacksonian convulsions, even - though in a proved syphilitic case, that the convulsions in - question are surely due to a focal lesion, for they may be due to - diffuse syphilitic lesions. - - 2. What is the significance of aphasia in Agnes O’Neil? Aphasia is - not a characteristic symptom in ordinary Jacksonian epilepsy, but - the aphasia is another sign of focal lesion and forms an added - argument against the diagnosis of genuine or idiopathic epilepsy. - See also discussion of aphasia in paretic neurosyphilis under Case - Levenson (22). - - 3. What is the behavior of the serum W. R. and the spinal fluid W. - R. under systematic treatment? Sometimes, as in this case, the - serum W. R. remains positive and the fluid W. R. becomes negative; - but in other equally well-defined cases, the reverse holds true, - and the serum W. R. reaction becomes negative whereas the spinal - fluid reaction remains positive. The obvious conclusion is that we - cannot always be sure even by faithful tests of either the serum - or the fluid alone, whether the treatment has succeeded in - abolishing the laboratory signs. - - 4. Can this case be regarded as one of cure? Not by the definition - adopted in this book or by the syphilographers who take into - account not only the nervous system but the body which contains - it. To be sure, the spinal fluid of Agnes O’Neil is now entirely - negative and she is clinically free from symptoms; yet from the - broad standpoint of syphilis therapy in general, this patient is - not cured, as is evidenced by the positive serum W. R. - - - =PARETIC NEUROSYPHILIS (“general paresis”) is often marked by - SEIZURES.= - - -=Case 20.= Lester Crane, a plumber, 37 years of age, came to the -hospital with a slow and defective speech. Moreover, there seemed to be -some mental disorder since his answers to questions were not always -relevant. It appeared that he was seeing bugs on the wall. - -=Physically=, Crane was a well developed and nourished man, with -overactive knee-jerks and a Babinski reaction on the left side. - -It developed that there was an impairment in hearing. The pupils reacted -well both to light and to distance. The patient was very restless and -smiled in a silly fashion. His memory was decidedly defective in all -spheres, and he was very slow in the intake of ideas. - -The plumber’s wife said that, at about the age of 23 or 24, he had a -spell of confusion lasting two or three days, with peculiar conduct, -unintelligible talk, and a good deal of weeping. The medical diagnosis -at that time took into account the fact that Crane was a plumber and was -“lead encephalopathy.” - -However, according to his wife, Crane had acquired chancre at about 26 -years, was treated mercurially for about three years and declared well. -He had remained well up to about 18 months before entrance, when, -without previous warning, the patient had a convulsion with the -continuous movements for about half an hour. He was semi-conscious for -about 18 hours and vomited continuously. There was amnesia for the whole -affair on regaining consciousness. In a week’s time, Crane was entirely -well. But six weeks later there was another convulsion. Upon removal to -a hospital, the diagnosis of general paresis was made, and the patient -was given the Swift-Ellis intraspinous treatment. This seemed to be very -successful, and the patient discontinued treatment after 14 weeks -(during which time there had been seven treatments) on the ground that -he was entirely well. - -However, after discontinuing treatment, there was another convulsion in -about a month, and further convulsions occurred once a month. For six -months, however, the patient took no treatment, but finally returned to -the hospital and was given mercury. This treatment appeared to suspend -convulsions again for three months, but at the expiration of six months, -the patient had three convulsions in one day, and several more during -the following days. After the last of these convulsions, there had been -numbness on the right side of the body and considerable headache. - -The diagnosis of PARETIC NEUROSYPHILIS (“general paresis”) is borne out -by the laboratory tests. The W. R. of the blood serum was, to be sure, -negative, but the W. R. of the spinal fluid was positive, and there was -a “paretic” type of gold reaction, together with other laboratory signs. - -The case well demonstrates that group of paretic cases in which -convulsions periodically occur, leaving the patient worse after each -convulsion. Treatment with salvarsan was instituted, and mercury and -iodid was given by mouth. During the period of eight months which have -now elapsed since the beginning of this treatment, there have been no -convulsions; there has been a great improvement in the memory, the -hearing has improved, the W. R. in the spinal fluid is much less -intense, the gold sol test has become negative, and the other tests are -all less intense. - -The patient, however, has not been entirely well, for in place of the -generalized convulsions, he has had minor seizures, beginning as a rule -with a tingling sensation in the right hand, extending up the arm, down -the trunk and leg, and through the right side of the face, with a bitter -sensation on the right half of the tongue. The patient maintains that -this sensation is absolutely confined to the right half of the body (in -this connection we may recall case Morton (1), in which there was also a -hemiplegia together with other apparently hysterical symptoms at several -times during the long course of a disease with abundant structural -correlations). During these minor seizures, the patient is unable to -talk, although he does not lose consciousness and is entirely aware of -everything going on about him. These attacks have of late been growing -somewhat less frequent. - - - =LOSS OF DEEP REFLEXES= - - NEUROSYPHILIS - NEURITIS - (alcohol, diabetes, diphtheria, lead, arsenic, tubercle, - cachexia, etc.) - Peripheral nerves sensory or motor - PERIPHERAL NERVE PALSIES - TEMPORARILY FROM COMPRESSION BY TOURNIQUET - FRIEDREICH’S ATAXIA - SUBACUTE COMBINED DEGENERATION OF POSTERIOR AND LATERAL COLUMNS - Posterior column disease - FOCAL LESION IN GRAY MATTER OF CORD - INFANTILE PARALYSIS (ACUTE ANTERIOR POLIOMYELITIS) - PROGRESSIVE MUSCULAR ATROPHY - (chronic anterior poliomyelitis) - Anterior cornua of cord - AMYOTROPHIC LATERAL SCLEROSIS - SYRINGOMYELIA - THROMBOSIS OF ANTERIOR SPINAL ARTERY - LANDRY’S PARALYSIS - Anterior cornua and peripheral motor nerves - MYOPATHIES - (pseudohypertrophic and atrophic types) - MuscLe itself - AMYOTONIA CONGENITA - FAMILY PERIODIC PARALYSIS - (during attacks) - INCREASED INTRACRANIAL PRESSURE - (especially hydrocephalus and tumors of posterior fossa) - PNEUMONIA - IMMEDIATELY AFTER ATTACK OF MAJOR EPILEPSY - (post-epileptic coma) - TOXIC COMA - (uremia, morphine, etc.) - DURING SPINAL ANESTHESIA - COMPLETE TRANSVERSE LESION OF CORD - - PURVES STUART - - CHART 16 - - - 1. What is the cause of the negative serum W. R.? It is claimed that - 3 to 5% of all cases of general paresis yield a negative blood - serum. In this particular case, there had been considerable - treatment, including some Swift-Ellis treatment, so that it may be - that this treatment had reduced a formerly positive blood serum W. - R. to a negative one. - - 2. What is the nature of the typical seizures of general paresis? - The most frequent seizures are epileptiform and bear a general - resemblance to cortical epilepsy; but more rarely these seizures - resemble the ordinary epileptic attack or consist of a violent - general shaking of the whole body. A variety of initial minor - disorders usher in the attacks: the temperature is often - increased. The attacks are over after one or at most after a few - hours. Kraepelin speaks of one that lasted 14 days. Sometimes a - _status paralyticus_ develops, suggestive of the _status - epilepticus_. Another rarer form of characteristic seizure is the - apoplectiform, which can hardly be told from an ordinary stroke, - and may be followed by the usual post-apoplectic phenomena. A good - many of the strokes leading to sudden death in middle life are - probably cases of neurosyphilis although often set down as early - arteriosclerosis of a non-syphilitic nature. Besides the - epileptiform and apoplectiform seizures, there are certain - seizures of a less definite and complete nature, ranging from - simple fainting spells, dizzy spells and petit mal attacks, to - various special forms of irritative muscular contractions and - temporary speech disorders. Sometimes these attacks occur with - complete preservation of consciousness. Transient paresthesias, - visual field defects, and especially attacks of vomiting, which, - according to Kraepelin, may precede paresis by years (of course in - this connection gastric crises of tabes must be thought of), may - be counted as sensory seizures. - - 3. What is the proportion of paretic cases developing seizures? - Figures vary from 30 to 90%. According to Kraepelin, seizures - occurred in 30 to 40% of his cases at Heidelberg; he was of the - impression that treatment in bed had reduced the number of - seizures. 65% of paretics admitted to Munich (under very free - conditions of admission) were determined to have shown seizures - before their admission to the hospital. Seizures are said to be - somewhat more frequent in men than in women. These paretic - seizures are not due to either hemorrhages or vascular plugging—at - least in the vast majority of cases—and must be ascribed to the - effects of microscopic injuries. - - 4. What is the effect of seizures upon the future course of paretic - neurosyphilis? The current idea as expressed, for example, by - Mercier, is that “immediately after each crisis the patient is - much worse than he was before it, and thereafter there is some - improvement, but he never improves up to the point at which he was - before the occurrence of the crisis.” That is, “The course of the - disease is one of sudden plunges, each deeper than the last, each - followed by a gradual recovery that is less complete than the - recovery from the previous plunge.” - - 5. During what period of the disease are seizures most common? Late - in the disease many cases have convulsions, even though there were - none for the first year or two. In other cases the convulsion is - the first indication of paresis. - - - =DIFFUSE (non-paretic) NEUROSYPHILIS (“cerebrospinal syphilis”) is - often marked by APHASIA.= - - -=Case 21.= Martha Bartlett, a woman of 40 years, was brought to the -Psychopathic Hospital aphasic, or at least unable to talk distinctly -enough to be understood, or even to give name and address. The police -had found her wandering aimlessly about the streets. Although she was -well-dressed, she was mud-bespattered and apparently had not changed her -garments for several days. It shortly developed that the patient, -although unable to express herself either in words or by writing, could -understand everything that was said to her and could indicate by the -monosyllables _yes_ or _no_ whether she agreed or disagreed with -statements made. It was thus determined that she was pretty well -oriented. She was able to understand both speech and printed words. -Although she approximated more than is at all common a pure type of -_motor aphasia_, it appeared that there was a slight involvement on the -sensory side, especially in the sphere of visual imagery. - -=Neurologically=, the patient showed moderate strabismus, slight -deviation of the tongue to the right, and considerable tremor on -protrusion of the tongue. The right side of the palate hung lower than -the left. The ankle and arm reflexes were possibly more active on the -left side, and the left grasp was somewhat better than the right. Both -knee-jerks were active, but again the reflex on the left side was more -active than the right. No other abnormalities of reflex were determined. -There was no Rombergism but the gait was somewhat ataxic. For the rest, -the physical examination was normal. The blood pressure was 120 -systolic, 85 diastolic. - - - =CONDITIONS IN WHICH SPEECH DEFECT IS FOUND= - - NEUROSYPHILIS - HYPOGLOSSAL PARALYSIS - FACIAL PALSY - PARALYSIS OF PALATE (POST-DIPTHERITIC) - BULBAR PALSY - PSEUDOBULBAR PALSY - MYOPATHY—FACIO-SCAPULO-HUMERAL TYPE OF LANDOUZY AND DEJERINE - MYASTHENIA GRAVIS - FRIEDREICH’S ATAXIA - LARYNGEAL TABES - ALCOHOLIC INTOXICATION - POST HEMIPLEGIC - LENTICULAR DISEASE - BILATERAL ATHETOSIS - MULTIPLE SCLEROSIS - DEAF MUTISM - PARALYSIS AGITANS - CHOREA - STAMMERING - TICS - HYSTERICAL APHONIA - - CHART 17 - - -The ready suspicion was that the case was one of apoplexy of slight -degree with post-apoplectic phenomena. Upon investigation, this -suspicion was confirmed since it appeared that Mrs. B. had been -apparently quite well until about six months before admission, when -without particular warning she began to act strangely and promptly fell -into a series of convulsions. These convulsions would begin with -twitchings of the face, and then spread throughout the body. There would -be a period of unconsciousness for two or three hours. It is not certain -how many of these convulsive seizures the patient had. At all events she -is reported to have recovered therefrom completely, remaining well for -three months; whereupon, suddenly, while visiting a friend, she suffered -a paralysis of the left side of the body. She remained dazed and had -hospital treatment for about a week. Ever since this left-sided -paralysis, the aphasic condition above described has persisted. - -Such a phenomenon has often been dismissed in the past as due to an -early arteriosclerosis, but most neurologists and internists of today -would look beyond the diagnosis of mere arteriosclerosis and consider -syphilis. The only suggestive feature in the case, aside from the -post-apoplectic reflex disorder and spastic phenomena, is the -irregularity and diminished light reaction of the pupils. Our suspicions -were confirmed by the positive serum W. R. The W. R. of the spinal fluid -proved, however, to be negative. There was a moderately strong gold sol -reaction of the syphilitic type. There was a slight excess of albumin, -and there was an exceedingly slight amount of globulin. There was but -one cell per cmm. - -On the whole, it would seem best to consider the case of Mrs. Bartlett -to be one of CEREBRAL ARTERIOSCLEROSIS OF SYPHILITIC ORIGIN, and a case -in which there is no evidence of meningitis or meningoencephalitis. - - 1. What is the explanation of the negative spinal fluid W. R.? It - may be that none of the W. R. producing bodies have gone over into - the spinal fluid. It has been shown by the work of Weston that the - W. R. producing body is not identical with the bodies responsible - for the other tests in cerebrospinal syphilis. Moreover, it has - been clearly shown that these several tests of the spinal fluid do - not run at all parallel with one another. Especially is it true - that the chemical tests do not correspond at all with the degree - or nature of the pleocytosis. On the whole, when involvement of - the nervous system is entirely vascular, it is not only - theoretically proper but also practically common, to find a spinal - fluid negative to several tests. - - 2. Omitting consideration of the syphilitic gold sol of this case, - what conclusion could be drawn from the albumin and globulin - findings? It would not be warrantable to assume syphilis since it - is a common finding after cerebral hemorrhage due to - non-syphilitic arteriosclerosis to find excess albumin and also - globulin in the spinal fluid. Occasionally, also, pleocytosis - occurs in cases of cerebral hemorrhage even when the hypothesis of - an active meningitis can be excluded. We may recall in this - connection the pleocytosis in so-called meningitis sympathica of - certain brain tumors. (See also the case of Milton Safsky (48), a - case of brain tumor in which there was an excess of albumin, a - large quantity of globulin, and a pleocytosis of 146 cells per - cmm.) - - 3. What can be expected from treatment in these cases of vascular - cerebral syphilis? The condition offers very little opportunity - for therapeutic results. However, antisyphilitic therapy is - indicated to prevent if possible further progress of the lesions. - Since the lesions are, however, vascular, and since it must remain - a question how far these vascular lesions are due directly to - spirochetal action, and since in any event it may be difficult to - reach the spirochetes thus active, perhaps it is best to place - most reliance on potassium iodid. In any event, potassium iodid - should be given. Salvarsan and mercury are also indicated. It is - common to warn against administration of large doses of salvarsan - in this type of case on the ground that further vascular ruptures - may be produced. (See Friedberg, 108.) - - 4. If we conclude that the aphasia of the Bartlett case is due to - vascular disease, can we conclude a relation between this vascular - disease and vascular tension? It is not safe to draw such a - conclusion. The Bartlett case itself showed low blood pressure. To - be sure, some cases of neurosyphilis show high blood pressure from - which one draws the _à la mode_ clinical conclusion to the effect - that the kidneys are probably involved in the arteriosclerosis; - but other cases do not show a high blood pressure but may in fact - show a low blood pressure. The vascular disease doubtless - responsible for the aphasia in the Bartlett case is probably not - at all an effect of blood pressure conditions, but is, on the - contrary, an effect of local syphilitic vascular lesions. - - - =PARETIC NEUROSYPHILIS (“general paresis”) is often marked by - APHASIA.= - - -=Case 22.= Meyer Levenson, a traveling salesman of 36 years, had for the -last two or three years been undergoing a change of disposition, quite -interfering with his work. He had begun to take unreasonable aversions -to people, had become irritable and emotionally depressed, and often -fell to weeping without cause. - -About nine months before hospital observation, it seems that a -trunk-cover had fallen on Levenson’s head, and there is some question as -to whether he did not have a convulsion at that time. However, a month -later he had a definite seizure, followed by speech disorder, a slight -paralysis, and a staggering gait. Four weeks later, however, he had -gotten over these post-convulsive difficulties and had gone back to -work. - -At his work, he became tired easily, his gait and speech did not seem -entirely normal, and there was a considerable memory disorder. After -five more months, another attack of a convulsive nature, with twitching -of hands and face and tongue-biting occurred, and the attending -unconsciousness remained for two days. Again improvement followed, -though without ability to return to work. Four (?) months later there -were several severe convulsions and Levenson would remain unconscious -for a day or two at a time. Restlessness, irritability, and irrational -talking followed. - -=Physically=, the patient was fairly well developed and nourished; blood -pressure 168 systolic, 68 diastolic; pupils reacted very sluggishly to -light. There was a marked motor aphasia, which the patient recognized as -a speech difficulty. On the whole, however, Levenson was very euphoric -and was entirely sure that he was improving and would surely get well. - -Shortly after entrance, Levenson had a severe convulsion, with -unconsciousness. The movements were mainly on the right side of the -body, and there was a post-convulsive weakness of the right side for -several days, followed by a slow recovery of strength. - -The course of the disease—convulsions followed by improvement—is very -characteristic of a paretic onset. The =laboratory findings= were in all -respects confirmatory. It was rather striking that a permanent _motor -aphasia_ followed the convulsions in this case, since the seizures of -paresis do not in the vast majority of cases leave permanent paralyses. -The course of the disease continued to show convulsions, which would in -each instance leave him at a lower terrace of capacity than had been -before shown. The patient died four years after the onset of symptoms of -a general asthenia. With the exception of the permanent motor aphasia, -this case might be regarded as a fairly typical one of general paresis. - - 1. What is the general nature of speech disorder in paretic - neurosyphilis? Speech disorder is, along with the pupillary - changes, one of the most important clinical symptoms in paretic - neurosyphilis. There are aphasic and articulatory disturbances. - The aphasia that accompanies paretic seizures is of a transient - nature as a rule. A case with such long-standing motor aphasia as - shown by Levenson is not common. Paraphasia, with incorrect naming - of objects, may last longer. The so-called “sticking” phenomenon - is often observed. - - Word-deafness is said to be rarer but is difficult to test on - account of the patient’s dementia. Agrammatism (incapacity to form - correct sentences) is sometimes observed. But the most - characteristic disorder is in the syllabic composition of words. - Syllables are left out (“medaltricity” for medical electricity), - or fused (“exity”), or doubled (“electricicity”). Besides the - central speech disorders of which the above are examples, there - are disorders in articulation, which at first occur as a - consequence of paretic seizures or in states of excitement, but - later become permanent. These are divided into paretic and ataxic - disturbances. - - 2. What is the structural basis of these forms of aphasia? It is - believed that they are due to microscopic changes, not to coarse - destructive lesions. - -[Illustration: - - BROOKLINE, MASS. -] - -[Illustration: - - BROOKLINE, MASS. - - Mss. of Levenson, case 22. Paretic neurosyphilis. Tremor, misspelling. - Metathesis of letters (Bk, not Br) omission of letters (Book). -] - -[Illustration] - -[Illustration: - - God save the Commonwealth of Massachusetts - - Mss. of Safsky, case 48, brain tumor. Tremor not marked. Misspelling, - omission of letters. Wrong letters (h in hweth). -] - -[Illustration: - - Mss. of Halleck, case 31, cervical tabes. No brain disorder. - Pen-holding and bearing on difficulties. Crowding of phrases result - of ataxia. -] - -[Illustration: - - Mss. of Collins, case 61, paretic neurosyphilis. One misspelling - (-chussetts); not psychopathic? Characteristic tremor. -] - - - =REMISSIONS of identical appearance occur in PARETIC and in DIFFUSE - (non-paretic meningovascular) NEUROSYPHILIS.= - - -=Case 23.= Thomas Donovan, a merchant 44 years of age, acquired syphilis -according to his own story at the age of 31, and he was at that time -treated at a well-known watering-place with mercurial injections. Later -he continued treatment under his family physician, and at 34 was -pronounced cured. However, four years later—that is seven years after -his initial infection and in his 38th year—he had his blood examined and -it proved positive. He was accordingly treated by salvarsan and his W. -R. became negative. The story did not end there, however, for at 43, -mental symptoms appeared of the nature of depression and a diagnosis of -paresis was made. He was released from the institution against advice at -that time, and without treatment, made a partial recovery. - -A sudden outburst of violence brought Mr. Donovan to the Psychopathic -Hospital; he was very surly, combative, and difficult to manage, -standing 6′ 2″, and weighing 210 pounds. He was oriented only fairly -well and his surliness was streaked with humor. He facetiously said that -the Psychopathic Hospital was the largest hospital in the country, and -that it was, in fact, a horse hospital; that he had come because he -liked the surroundings, not to make money; that he was the healthiest -man in the world, never having been sick; that the Psychopathic Hospital -was a club, for which you have to get somebody to propose your name. -There was amnesia and no knowledge of current events. He regarded the -food as poisoned, refused to eat, and was very irritable and untidy. - -=Physically=, there were few abnormalities, but the pupils failed to -react either to light or accommodation, and the knee-jerks and -ankle-jerks were absent. There was a slight Rombergism. There was a -marked speech defect to test phrases. Both serum and spinal fluid W. -R.’s were positive; the fluid showed 41 cells per cmm., there were large -amounts of globulin and albumin, and the gold sol reaction was of the -“paretic” type. - - - =ATAXIA OR INCOÖRDINATION= - - NEUROSYPHILIS - LESION OF PERIPHERAL SENSORY NERVES - DIVISION OF POSTERIOR ROOTS - TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COLUMNS - SUBACUTE COMBINED DEGENERATION - VESTIBULAR ATAXIA - FRIEDREICH’S ATAXIA - FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS - THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY - MARIE’S HEREDITARY CEREBELLAR ATAXIA - LESIONS OF CEREBELLUM, TUMORS, ETC. - WRITERS’ CRAMP - PREHEMIPLEGIA - MULTIPLE SCLEROSIS - PSEUDO-SCLEROSIS - HYSTERIA - - CHART 18 - - - =CONDITIONS IN WHICH VERTIGO IS FOUND= - - NEUROSYPHILIS - HEAD TRAUMA - CEREBRAL ANEMIA AND HYPEREMIA - MENOPAUSE - ARTERIOSCLEROSIS - RENAL DISEASE - CEREBRAL HEMORRHAGE AND THROMBOSIS - INTRACRANIAL TUMORS - MULTIPLE SCLEROSIS - EPILEPSY (AURA) - TOXIC CONDITIONS: - alcohol, tobacco, constipation - PSYCHONEUROSIS - OCULAR DISTURBANCES - EAR DISEASE - MÉNIÈRE’S DISEASE - MIGRAINE - - CHART 19 - - -Salvarsanized serum was injected intraventricularly through a trephine -opening in the right frontal region. Injections were made through the -corpus callosum into the third ventricle. There was progressive -symptomatic improvement after each of four injections. In fact, after -the fourth injection the patient was allowed to leave the hospital -despite the fact that there was only a slight improvement in the spinal -fluid findings. The speech defect had entirely disappeared. (Speech -defect, according to many authorities, including Kraepelin, is of very -grave diagnostic significance.) His memory returned. Mr. Donovan is now -able to handle figures rather extraordinarily well. He now has a good -insight into his delusions and tells stories about them with great -humor. - - 1. What is the definition of a remission in general paresis? - Remissions form a foil to seizures; just as seizures mark a sudden - advance in the severity of the disease or may even lead to death; - so remissions may cause a sudden cessation of both mental and - nervous phenomena in the disease. Whereas the seizures occur most - often, according to Kraepelin, in the demented types of paresis, - the remissions occur in all cases except in the terminal phase. - Kraepelin quotes Hoppe as observing pronounced remissions of long - duration in 17% of male and 15% of female paretics. Gaupp observed - marked improvement in less than 10%, and very marked improvement - indeed in only 1% of his cases. Kraepelin states that such - improvements are most frequent in agitated and especially in - expansive forms of paresis, and that they are rarer and less - complete in the depressive and demented forms. Sometimes the - improvement occurs over night, although the full extent of the - remission becomes complete only gradually, perhaps in the course - of months. The sensorium clears, the disorientation disappears, - the delusions retreat, and the former delusions are treated as - dreams and imaginations. There is often a good deal of persistent - uncertainty as to events during the height of the disease. The - nervous disorders are far more obstinate than the mental. Still, - both speech and writing may often greatly improve. - - Cotton in New Jersey found, among 127 cases of paresis - diagnosticated by modern methods during seven years, that - remissions occurred in but five, or about 4%, lasting from a half - to three years. - - 2. Does a remission ever amount to a cure? The classical case quoted - in this connection is one observed by Tuczek. This case developed - a picture of paresis in 1876, at the age of 36; and a remission, - or cessation, of symptoms, occurred in 1878; but in 1883, at 43 - years, the patient developed a tabes without any trace of mental - disorder, which tabes gradually advanced. By the middle of 1898, - when the patient was 58, certain symptoms of excitement and - confusion occurred, which led to death with dementia, 22 years - after the beginning of the disease. Nissl pronounced the cortex to - be undoubtedly the characteristic cortex of a paretic. This - observation seems to indicate that a clinical remission tantamount - to a clinical recovery may occur without the death of the - spirochetes engaged. This observation is to be held in mind in - connection with all therapeutic work with neurosyphilis. - - Nonne states that during his clinical experience of 19 years he - had followed 10 cases of paresis with apparent recovery; but of - these ten cases, four had to be thrown out by Nonne because the - apparent recoveries turned out to be only long and almost complete - remissions, finally issuing in characteristic dementia. Of the - remaining six cases, perhaps two should hardly be counted as - paretic and Nonne rather preferred to term them cases of - syphilitic dementia in the sense of a non-paretic cerebral - syphilis. At the end, therefore, of his review of observations, - Nonne found himself with four cases of true recovery from paresis. - - Spielmeyer holds that there is no theoretical reason why paresis - might not be cured, since all the different changes that have been - described in the disease can be halted, and many of them can be - repaired. In particular, he reminds us that the acute infiltrative - process, the neuroglia reaction, and the phagocytic action of the - large mononuclear cells are distinctly removable processes. (See - discussion below under Section V, for apparent cures and - remissions occasionally secured under treatment.) - - - =REMISSIONS of identical appearance occur in PARETIC (“general - paresis”) and in DIFFUSE (non-paretic) NEUROSYPHILIS.= - - -=Case 24.= Michael O’Donnell, a laborer of 48 years, came home, one day, -at 5:30, complaining of severe headache. His wife told him he should lie -down and, taking him by the arm, tried to help him to the bed. At this -moment, O’Donnell lost control of both left arm and left leg, and fell, -unable to move but with consciousness preserved. The wife noted that the -left side of his face was drawn up and that he drooled. He was at once -carried to a general hospital, remaining there for about three weeks, -talking at random in a delirious manner and tied in bed. Two -intraspinous injections of salvarsan were given, and O’Donnell showed -considerable improvement and went home. - -However, upon his return from the hospital, he became very wilful, would -not remain in bed, and on one occasion actually took the mattress from -the bed, carried it to another room, and then returned to his own room -and slept upon the springs. He became irritable and emotional, insisted -upon going to the hospital, did not go there but upon returning home -insisted that he had been there. That night, O’Donnell left the house -only partly dressed. - -It appears that O’Donnell had been excessively alcoholic, but that -before August 15, when he sustained the left-sided hemiplegia above -mentioned, there had been no symptoms except that in February he had -once been very dizzy. It appears that there had been another dizzy -spell, three nights before the paralysis, accompanied by a fall and -unconsciousness for about 15 minutes. - - - =TRANSIENT OR FLEETING PARALYSES= - - NEUROSYPHILIS - MYASTHENIA GRAVIS - MYOTONIA CONGENITA (THOMSEN’S DISEASE) - PARAMYOTONIA CONGENITA - MYOTONIA ATROPHICA - INTERMITTENT CLAUDICATION - OCCUPATION NEUROSES - FAMILY PERIODIC PARALYSES - ETANY - EPILEPSY MINOR - HYSTERIA - MULTIPLE SCLEROSIS - APOPLEXY - CEREBRAL THROMBOSIS - - CHART 20 - - -O’Donnell was brought to the Psychopathic Hospital some six weeks after -the paralysis, complaining merely of a slight headache and desirous of -treatment. There were no mental symptoms of any sort. =Physically=, -O’Donnell was in general not abnormal (there was a slight pre-systolic -murmur and a blood pressure of 190 mm. systolic). The pupils were -slightly irregular, the left larger than the right; both reacted -sluggishly. Both ears were moderately deaf; the tendon reflexes of the -left arm and leg were somewhat more lively than those on the right. The -systematic =neurological= examination otherwise revealed no -abnormalities. The urine was negative. The serum W. R. was positive but -the spinal fluid reaction was negative. There were but 2 cells per cmm., -and there was a very slight trace of albumin. - - 1. How shall we account for O’Donnell’s transient paralysis? We - might invoke brain tumor, alcoholic pseudoparesis, or some form of - neurosyphilis. The diagnosis of brain tumor seems quite untenable - in view of the absence of premonitory symptoms and in the absence - of intracranial pressure. As for alcoholic pseudoparesis it is - true that the patient was excessively alcoholic. - - However, against these two diagnoses and in favor of the diagnosis - of NEUROSYPHILIS, are the positive serum W. R. and the pupillary - reactions (although these are short of the true Argyll-Robertson - phenomenon). Dizziness with retention of consciousness and - associated with the paralyses mentioned suggests rather a - subcortical than a cortical lesion. We are inclined to regard this - lesion as probably THROMBOTIC, and to place it possibly in the - region of the internal capsule. We are inclined to regard the - phenomenon as purely vascular and as not in this case associated - with an encephalitis. We are, however, not entirely satisfied with - the diagnosis. - - 2. What shall be said as to treatment? A full-blown left-sided - hemiplegia may be produced even when the thrombotic lesion is - itself exceedingly small. It is common to explain this on the - basis that there is an area of collateral edema about the small - necrotic, thrombotic, or hemorrhagic area responsible for the - lesion. In short, numerous neurones are functionally rather than - structurally affected, or at all events capable of early - restitution of function. - - 3. What is the prognosis in such cases? It appears that now and - again patients run for several years without further trouble, both - with and without treatment. We are inclined, however, to advocate - treatment rather than absence of treatment for a variety of - reasons. In the first place, vascular lesions may at any time - become associated with meningitic lesions, and treatment by - salvarsan may perhaps be counted on to head off this process; - secondly, the treatment with iodids may possibly aid in the - resolution of a local thrombotic process. - - 4. What are the prodromal symptoms of cerebrospinal syphilis? - According to Nonne, headache, dizziness, sleeplessness, mental - symptoms of the irritability group, loss of capacity as to mental - work, whether severe or not, and loss of capacity for difficult - thinking; also impairment of memory. Nonne does not regard these - phenomena as characteristic of syphilitic vascular disease, and - calls attention to the fact that in every organic disease the same - subjective symptoms occur. The triad—headache, dizziness, and - impairment of memory—is for example now counted as a prodromal - symptom complex for arteriosclerotic apoplexy (Cramer). Of course, - apoplectic attacks occur without such preliminary symptoms: - particularly, according to Nonne, the nocturnal attacks. - - 5. Can the fleeting paralysis be of service in differentiating the - diffuse from the paretic form of neurosyphilis? Probably not. In - both forms transient paralyses occur as well as the permanent - ones. In general, however, the transient paralyses are more - frequent in paretic neurosyphilis, whereas the permanent ones - occur more often in diffuse neurosyphilis. - - - =There are cases of NEUROSYPHILIS in which the laboratory signs are - positive but in which there are no clinical signs or symptoms - (PARESIS SINE PARESI?).= - - -=Case 25.= Richard Lawlor[6] was admitted to the Psychopathic Hospital, -October 29, 1914, being sent there from a general hospital where he had -gone on account of a self-inflicted wound of the wrist, apparently made -in a period of depression with suicidal intent. Routine notes follow. - -=Family History.= Paternal grandparents both died of heart disease. -Maternal grandfather died at seventy-two of dropsy. Moderately -alcoholic. Maternal grandmother died of shock at fifty-six. Father died -at age of forty, after an illness of eight years, from heart disease. -Father all his life was subject to fainting spells and headaches. The -only paternal cousin died at thirteen months of brain fever. Mother, -aged forty-seven, is, to say the least, eccentric. Says “she has several -times been given up from tuberculosis.” Two maternal uncles died of -tuberculosis, one from rupture, one from heart disease. One uncle who -“doesn’t know anything after he has a teaspoonful of liquor.” Several -other uncles and aunts whose history is not obtained. Patient is -mother’s only child. Mother was twice married. There were several -miscarriages by both husbands; patient child by first marriage. - -=Past History.= Patient born thirty-two years ago, full term, normal -delivery and development. Measles, mumps, and chickenpox in childhood. -Subject to headaches since seven or eight years old. Kicked in the face -by horse at seventeen or eighteen, not considered serious. Hit by a -baseball three or four years ago, leaving him hard of hearing on left -side. Married ten years ago; no children because he says his wife needed -an operation. He denies venereal disease by name and symptoms. For past -ten years has had attacks of depression lasting but a short time, but -quite severe. Never caused him to quit work as a barber and he felt -better when working. His married life he says was fairly happy except -for his wife’s extravagances, and on this account he left her a little -over a year ago, and she has applied for a divorce, which he is willing -that she should have, but does not wish to give her alimony. He admits -moderate alcoholism. - -=Present Trouble.= Patient states that since he left his wife a year ago -he has felt sorry a number of times. He has wished he had her back. He -has felt lonely. He has had six or eight periods of depression in that -time similar to those he has had for many years, lasting two or three -days, and sometimes a week. These were always precipitated by some cause -for worry. In these attacks he feels nervous, sleeps poorly, has little -or no appetite, sweats during his work and everything looks black. -Several times in these attacks he has had suicidal ideas. Ten months ago -he considered taking corrosive sublimate. For a little over a week -before entrance to hospital he had been out of work and had been -“sporting.” The day before entrance he had a telephone message from his -lawyer which upset him somewhat and he walked the floor all night. He -had just been shaving when the idea of suicide came to him. He sat down -a minute when suddenly the thought “to hell with the world” came to him; -he took the razor and slashed his wrist. He does not remember drawing -the razor across his wrist. As soon as he saw the blood he felt sorry, -called his mother, and was taken to an emergency hospital and then sent -to the Psychopathic Hospital. - -=Physical Examination.= Patient is a well developed and nourished man -thirty-two years of age. Head is normal as to size and shape; there are -no scars or marks of injury. Hair and skin not remarkable in any way. -Ears negative to external examination. Teeth well kept; two missing, -several gold fillings. Tongue very slightly coated. Throat negative. -Tonsils easily visible without evidence of inflammation or exudation. -Neck, no thyroid enlargement, no abnormal pulsations, no adenopathy. -Chest, symmetrical, expansion good, resonant throughout. Breath sounds -transmitted normally. No râles or rubs heard. Heart, no enlargement or -cardiac dulness. Sounds of good quality, no murmurs heard. Rate regular. -Pulses equal, regular and synchronous, and of good volume and tension. -Systolic blood pressure 130, diastolic 65. Abdomen, flat, soft and -tympanitic throughout; no masses; no tenderness. Liver edge not felt, -below costal margin. Spleen not palpable. Extremities negative, except -for incised wound on left wrist. - -=Neuromuscular Examination.= Pupils are large, round, regular, equal and -react readily to light and accommodation. No nystagmus, strabismus or -ptosis. No weaknesses or paresis of facial muscles. The tongue projects -medially and shows no tremor. The triceps and biceps reflexes are -readily elicited, and are quite active, as are the knee-jerks and -ankle-jerks. On one occasion it was thought that the tendon reflexes -were slightly more active on the left than on the right. This was never -confirmed; always afterwards found equal. There was no tremor of -extended hands. Abdominal reflexes not elicited. Cremasteric present on -both sides. The plantar response is flexor. There is no Babinski, Gordon -or Oppenheim. No Romberg. Coördination tests well performed. No speech -defect. No sensory disturbances. Urine examination negative. - -Wassermann reaction in the serum: Positive, with cholesterinized -antigen; negative, with syphilitic fetal liver antigen. - -Wassermann reaction in fluid positive on two occasions. Examination of -spinal fluid, November 4: globulin +++, albumin ++, 100 cells per cubic -millimeter; large lymphocytes, 8 per cent; small lymphocytes, 90 per -cent; plasma cells, 0.7 per cent; endothelial cells, 1.3 per cent. -November 11, globulin +++, albumin +++, cells 18 per cubic millimeter. -November 26, globulin ++, albumin ++, cells 92 per cubic millimeter; -large lymphocytes, 13.1 per cent; small lymphocytes, 82.1 per cent; -plasma, 1.2 per cent; endothelial, 3.6 per cent. - -Gold sol, November 4, 5555432100. - -Gold sol, November 26, 3332100000. - -=Mental Examination.= On entrance to hospital patient seemed slightly -depressed and a bit irritable. This condition lasted two days, after -which he was agreeable and apparently entirely over his depression. Even -during his mild depression, however, he talked freely. There was no -evidence of retardation. He told his story readily. Orientation was -intact. Memory excellent. Educational knowledge well retained. There was -no evidence of any hallucinations or delusions. - - 1. Was Richard Lawlor insane? - - There was, then, on the mental and physical examination nothing to - make a definite suggestion of a psychosis, and the most one could - think of was a psychoneurosis or a cyclothymia of at least ten - years’ duration. The findings in the cerebrospinal fluid and the - Wassermann reactions, however, give us material for thought. - Certainly one cannot call the man insane; all who saw him agreed - on this point. - - 2. If Richard Lawlor should some day develop mental symptoms, what - would be the genesis of the new psychosis? Though writers such - as Fildes and McIntosh, and Swift, have suggested an - anaphylactic or hyperallergic explanation for the development of - symptoms after a normal interval; such a hypothesis could hardly - obtain in the present case. The hyperallergic hypothesis for the - development of tertiary neurosyphilis would run to the effect - that in the secondary stages there had been a definite disease - of the nervous system, which, however, absolutely cleared up, - leaving no inflammatory vascular or parenchymatous relics of its - existence. Nothing would on this hypothesis remain except a - hypersensitisation of the tissues. In some later period of the - now clinically normal person, one or more spirochetes from a - lesion outside the nervous system are carried into the nerve - tissues and there set up an anaphylactic or hyperallergic - reaction. It is obviously difficult to prove the correctness or - incorrectness of the hyperallergic theory without numerous - examinations of the spinal fluid, in clinically normal persons - after the secondaries have passed. The present case, so far from - demonstrating a normal fluid, demonstrates a highly pathological - fluid, even though there are absolutely no clinical symptoms - which could be regarded as of nervous origin. The burden of - proof at the present time would seem to lie with those who claim - hyperallergy in neurosyphilis. We prefer on present evidence to - think that at the conclusion of the secondaries a disease - process often remains in the nerve tissues despite clinical - quiescence. - - 3. What is the prognosis in the case of Richard Lawlor? The - prognosis _re_ neurosyphilis is doubtful. We have, however, boldly - termed the condition _PARESIS SINE PARESI_, meaning thereby to - suggest that the patient is in considerable danger of the - efflorescence of a true diffuse or paretic neurosyphilis. We have - no means of telling, however, whether the positive symptoms would - be those of a paretic or a non-paretic neurosyphilis. As data - accumulate regarding these cases of _paresis sine paresi_, we may - be able finally to come upon some case in which trauma shall bring - out the clinical symptoms of neurosyphilis. For discussion of this - matter, see the case of Bessie Vogel (52) in Part III of this - book. - - 4. Should Lawlor have been brought to a psychopathic hospital? It is - a safe working rule to have any person who attempts suicide - observed. A large percentage of suicides occur in psychotic - individuals and a suicidal attempt is not infrequently the first - recognized abnormality. Immediate observation is a necessary - safeguard against another more successful attempt. - - - =Demonstrates SYMPTOMS and LESIONS of PARETIC NEUROSYPHILIS - (“general paresis”). Autopsy.= - - -=Case 26.= John Morrill, 49, an operative in a mill town in Essex -County, Mass., was described as a “Saturday night and Sunday drinker,” -with a history of very serious long sprees at the age of 43. It seems -that he had had what was called “sciatica” at 35, and was treated in -hospital for seven weeks at that time. The nature of this sciatica is in -doubt, but there was a history of syphilitic infection at 36 years (scar -of glans). - -Morrill had been married twice, and two of the children were dead; one -daughter was described as “very nervous,” but there were four children -under ten years of age, all regarded as perfectly healthy. - -Morrill had been a mill operative of average capacity, was industrious, -and had supported his family despite alcoholism. The syphilis had been -treated with reasonable thoroughness. - -Aside from alcoholism, there had been no symptoms up to two months -before admission to Danvers Hospital. Then there had been insomnia, -fatigue, agitation, eruption on foot, loss of ten pounds in weight, -hypochondriacal fears, apprehensiveness for the future of the children, -incoherent talk; and just before admission, his talk was described as -foolish. He had taken to running away and hiding in bushes by a pond and -in the cellars of other people’s houses. - -The patient was of medium height and weight, with thin grayish hair and -grayish irides; musculature was slender. The face was blank in -expression, the teeth poorly preserved with atrophy of gums, the tongue -coated, and the breath foul. There was a gummy secretion of the eyelids, -an area of brownish branny eruption over both clavicles, a number of -depressed scars over the limbs and back, and another area of scaly -eruption on the right heel and the sole of the foot. The heart area was -increased, and the sounds were faint at the base, with the first sound -accentuated at the apex. The urine showed a trace of albumin. - -=Neurologically=, the Romberg position was maintained with a general -tremor and fluttering of the eyelids. In complicated movements, the -patient was slightly ataxic. The pupils were irregular, the left being -much larger than the right. There were no light reactions to be obtained -in window light. The reaction to accommodation was present, though -slight. Vision was poor, ¼-inch capitals could not be read by left eye -at reading distance. The knee-jerks were diminished equally; the -Achilles jerks were absent; the other reflexes were normal. Upon the -sensory side, the patient gave a history of pains in the legs at -irregular intervals for several years. These pains he described as of a -darting character. There was little or no sensory disorder, although the -outer surface of the right leg required a deeper pressure to elicit -sensation. There were no disorders of muscle sense. - -If Morrill was to be trusted, he had been born in Ireland, and had come -to the United States at the age of 17. He married at 18; there had been -seven pregnancies by the first wife, with one stillborn child; one child -had died at five weeks. The four children by the second wife were -healthy. The first signs of neuritis had occurred at 45 and had received -the diagnosis neuritis, although no connection between the neuritis and -the syphilis had been noted. - -The patient entered the hospital July 26, 1904, and was discharged, -improved, January 5, 1905. He returned a little more than a year later, -January 15, 1906, and died March 21, 1906. The total duration of the -disease from the onset of mental symptoms may therefore be stated as -somewhat under two years. When the patient appeared at the hospital the -second time, he showed a positive Romberg sign, an unsteady gait, an -ataxia that still was moderate, and somewhat more marked tremors, -involving fingers, tongue, and face. He was now unable to read ½-inch -type with the left eye. The knee-jerks, formerly diminished, were both -exaggerated, the left slightly more so. The Achilles reaction, not -obtained formerly, now appeared on the right side. The pupils reacted as -before. The sensory loss had become more marked, since sharp and dull -points could hardly be distinguished. Deep pinpricks were not felt in -the leg, and heat could not be told from cold. - -The speech in 1904 had been somewhat defective (“truly rural” rendered -as “tooly lualal,” “sifted soft thistles” as “thoft thsistles”), and -there had been little further development of the speech defect. The -handwriting had lost appreciably in legibility and had become much more -tremulous. During the first period of hospital observation Morrill had -what might possibly have been visual hallucinations, but it was -impossible to tell whether his story of seeing his wife and children -trying to get in through the window was hallucinatory or a matter of -fabrication. Memory was decidedly imperfect and few details of recent -events could be produced. The association of ideas was almost a -so-called “flight” of apprehensive, fearful ideas, loosely connected, -incoherently expressed, and dealing chiefly with his work and his -children. Judgment was imperfect; the height of the room was estimated -as 24 feet, but the height and weight of persons were estimated with -fair accuracy, and also the length of small objects, whose lengths were -doubtless remembered rather than estimated. The estimate of time -elapsing during a medical examination was accurate, but the estimate of -longer durations involving over-night memories was hopelessly imperfect. -Emotionally, there was a dulling of sensibility, an appearance of -suspicion and apprehensiveness; the patient fancied himself to be in a -hopeless condition as a result of syphilis, but at the same time -accompanied his statement of his hopelessness with laughter. A sample of -his hypochondriacal ideas: “I am all gone; I am good for nothing; I am -all gone now; I can’t drink now; can’t write or talk at all; worse than -when you saw me first; nothing in my inside; all wrong through me again; -I aint got no swallow now; I can’t die even; my heart aint much good; I -can’t hear it beat; I don’t think it flutters; no life in these hands; -they are all cold and dead” (pointing to his arms and moving them -about). During such a portrayal the patient laughed in a silly way. - -During the second hospital stay, Morrill was at first restless, -sleepless, profane, imperfectly oriented for time, possibly for place, -and also for the attendants. A few weeks later he became stuporous and -confused, and his feebleness and physical exhaustion were finally ended -by death, March 21, 1906. Death was preceded by a semi-comatose -condition; a left otitis media had developed. - -At the =autopsy=, it appeared that death was due to an early -bronchopneumonia associated with acute splenitis and doubtless related -to the otitis media of the left side. The body at large showed, aside -from these acute lesions, a few chronic lesions, including slight scars -of the left apex, and chronic adhesive pleuritis, chronic diffuse -nephritis, and aortic and coronary syphilis. The aorta showed slight -linear and nodular markings, with a single small dark ulcer in the upper -thoracic region, but the aorta did not show the characteristic scarring -which syphilitic aortas often show. The femoral marrow was of a dark red -chocolate color. The thyroid appeared to be smaller than normal. A -slight sacral decubitus had developed. - -The description of the head (E.E.S.) is given in full on account of the -encephalitic lesions shown. These encephalitic lesions may be summed up -as follows: - - Local cerebral =atrophy= and =sclerosis= of the frontal, orbital, - and central regions, especially of the left operculum and left - supramarginal gyrus. - - Extension of sclerosis to hippocampal gyri with effacement of - substantia reticularis alba. - - Slight chronic internal =hydrocephalus=. - - Granular =ependymitis= (especially of floor of 4th ventricle). - - Compensatory edema of frontal and central pia mater. - - Cerebellar sclerosis (culmen monticuli, lobus culminis, lobus - cacuminis). - - Spinal sclerosis (grossly evident in the posterior columns of the - upper thoracic region and of the lumbar enlargement). - -The details are as follows: - - Head:—Bald on top. Hair =gray=. Scalp normal. Calvarium thin, deeply - excavated by arachnoidal villi to right of vertex. Diploë absent. - Dura closely adherent in bregmatic region. Dura of usual thickness. - Sinuses contain cruor clot. Arachnoidal villi slight. Pia mater hazy - and over sulcal veins porcelain white over all of vertex except - occipital poles and over flanks (notably left). Thickened also - around circle of Willis, over culmen monticuli and in posterior - cerebellar notch. Edema of pia corresponding to atrophy of frontal - and central regions. Cerebral atrophy most marked in orbital - surfaces of both frontal lobes, in left area of Broca, and in left - supramarginal region. The ascending branch and the ascending ramus - of the posterior limb of the left Sylvian fossæ both readily admit - the thumb by reason of atrophy of adjacent substance. Induration - corresponds closely with atrophy, but is not more marked about the - left Sylvian fossa. There is sclerosis of both hippocampal gyri, - with loss of the substantia reticularis alba. The culmen monticuli - and lobus culminis are firmer than the clival regions, and the lobus - cacuminis is again slightly firmer than the clival region. - Cerebellum a little softer than usual. Pia strips with usual - readiness from all regions. The subpial region of the frontal lobes - is a trifle grayer than that of the rest of cerebrum. Ventricles - slightly dilated. Surfaces evenly sanded. Floor of fourth ventricle - shows numerous coarse, closely set granules. Brain wt. 1200 grms. - Cord shows a slight increase of consistence over one or two upper - thoracic segments and in lumbar enlargement corresponding with a - slight graying out of posterior columns. In places there is a - suggestion of graying out also in lateral columns. A few calcified - plaques in posterior lumbar pia. - -Analysis of these details shows a number of lesions that characterize -paretic neurosyphilis (among others, granular ependymitis, frontal -atrophy, chronic leptomeningitis), but the lesions are more than merely -frontal, extending as they do back as far as the postcentral regions on -both sides, and even as far as the left supramarginal gyrus. The -cerebellar involvement although frequent, can hardly be said to be -characteristic in paretic neurosyphilis. The spinal involvement is -characteristic of a case which is probably to be regarded as one of -taboparesis; that is, of paretic neurosyphilis following a number of -years after the establishment of tabetic neurosyphilis. The aorta is -almost constantly affected by sclerosis in paretic neurosyphilis. The -absence of diploë in the skull is not infrequent and the adherent dura -mater is often found. - -Microscopically, the tissues showed the characteristic lesions of -PARETIC NEUROSYPHILIS; nerve cell destruction, fibrillar and cellular -gliosis, lymphocytic and plasma cell deposits about the small vessels. - - 1. What are the clinical evidences of syphilis outside the nervous - system? The brownish branny eruptions of the skin, the depressed - scars and the scaly eruption on right heel and sole are very - suggestive of syphilis. Such clinical evidences of syphilis are - very important in systematic examination. Although the laboratory - tests are of the utmost assistance in the diagnosis of syphilis, - the clinical signs should not be neglected, and no physician - should rest satisfied with laboratory signs alone. X-ray diagnosis - of bone conditions sometimes succeeds when all other methods have - failed. - - - =GUMMA of cerebral cortex verified by operation; death.= - - -=Case 27.= The presenting picture in the case of David Tannenbaum was -that of deep dementia, in which condition the patient was brought to the -hospital. There was a meagre history to the effect that about four -months before admission, he had lost his job in a hotel through lack of -further work. We heard that at this time he had begun to suffer with -excruciating pains in the head; at first, worse at night, later, worse -by day. It appeared that this pain, though it came and went, was chiefly -localized on the left side of the head. For a fortnight, Tannenbaum had -been dragging his legs, until finally he had become unable to walk at -all. - -_Pari passu_ with these developments, Tannenbaum had become mentally -confused and irritable, and his memory had become untrustworthy. For -several days before admission, an appearance of marked dementia was -presented, with slow incoherent, or at all events, irrelevant words, and -a complete disorientation for person. However, his vision had become so -poor that it would have been hard for him to have recognized any one. - -It appeared that the family history was entirely negative; that the -patient was without education but had been physically very strong, and -had been fairly successful at first in the junk business, and later in -the clothing business; but latterly he had been less fortunate in the -clothing business, and finally had to resort to work as a laborer around -a hotel. - -His wife had had eleven pregnancies with but one miscarriage. -Nevertheless, out of the eleven pregnancies, there were now but four -living children. - -=Physically=, Tannenbaum was a rather small man; he was flabby and -looked as if he had recently lost weight. The skin showed areas of -pigmentation on the face and sides of the neck, and some dark -copper-colored circular areas, marble-size, in the neck (syphilitic?). -There was a slight radial arteriosclerosis. The heart was slightly -enlarged with distant and indistinct sounds. There was a small -pedunculated growth on the right side of the abdomen. - -The pupils failed to react to flash-light but they reacted to sunlight. -They both were slightly irregular but were equal in size, and reacted in -accommodation. There was apparently almost complete blindness and -extreme deafness. Arm-jerks and knee-jerks were absent; there was an -occasional slight response of the left ankle-jerk, but the right -ankle-jerk was absent; the left abdominal reflex was very feeble; the -right absent; the cremasteric reflexes were absent, but there were no -other abnormalities in the systematic examination. Hand grips weak; gait -awkward, with right leg held somewhat flaccidly. - -It was significant that percussion over the left frontal and parietal -regions was able to elicit great pain. Either through the patient’s -deafness or through sensory aphasia, spoken language was not understood. -The serum W. R. was positive, the fluid W. R. negative. - -=Diagnosis=: The clinical symptoms seem clearly to indicate syphilis. -The local skull tenderness and impairment of vision might well suggest -intracranial pressure. Uniting these suggestions, we might automatically -arrive at a diagnosis of cerebral gumma. We have learned to be rather -cautious of making a diagnosis of gumma of the brain through its mere -rarity. - -Decompression was suggested and executed. A deep growth resembling a -GUMMA, in the view of the surgeon, was discovered. No attempt could be -made to remove it. The patient died without recovering consciousness. - - 1. What is the significance of the negative fluid W. R. in this case - of cerebral gumma? The W. R. producing substances not infrequently - fail to appear in the spinal fluid from a gumma of the brain. The - serum W. R. was positive in this case, but even the serum W. R. - may be negative in cases of gumma, both of the brain and of the - body at large. It must be remembered that the serum W. R. may be - negative in paretic neurosyphilis (general paresis); the serum W. - R. is even more apt to be negative in cases of gumma. - -[Illustration: - - Gummatous meningitis. Compression of hemisphere. Tissue destruction of - underlying cortex. -] - - 2. Is operative procedure to be advised in cerebral gumma? There are - cases in which the acute and threatening symptoms of heightened - intracranial pressure require operative treatment simply because - the therapeutist cannot wait for the effect of antisyphilitic - treatment. Moreover, antisyphilitic treatment of cerebral gumma is - not always as successful as that of most syphilitic lesions. - - 3. Could the intracranial pressure be caused by other syphilitic - lesions than gumma? A heavy meningitis may cause symptoms such as - produced by an intracranial tumor. In such a case one will usually - find evidences of inflammation in the spinal fluid. Cysts caused - by syphilitic lesions may also produce identical symptoms. - - 4. What is the significance of cranial tenderness? Where - sensitiveness to cranial percussion is not due to a scalp lesion - it is very suggestive of a tumor underlying this point. A - gummatous lesion of the cranium itself, may occur without causing - pain or increased sensitiveness. - - - =CRANIAL NEUROSYPHILIS (focal syphilitic extraocular palsy) without - mental symptoms.= - - -=Case 28.= A chef, Paolo Marini, 28 years of age, reported that on -awaking one morning, everything appeared double to him and that his -right eyelid had begun to drop. In the following month Marini had begun -to feel weak and to have difficulty in swallowing, as well as at times -difficulty in breathing. The diplopia was found to develop when Marini -looked to the right. Mentally, the patient was in all respects normal, -and no other physical signs were found except the diplopia and ptosis -above mentioned. The W. serum test was positive, but the tests of the -spinal fluid were negative. - -=Diagnosis=: “CEREBRAL SYPHILIS.” - - 1. What is the anatomical cause of this condition? It is thought to - be due in a number of cases to a small diffuse gummatous lesion at - the _basis cerebri_. In the case of Marini this lesion appears to - have been a little more extensive and to have interfered with the - tenth and twelfth nerves also. - - 2. Why is the spinal fluid negative in such a case as that of - Marini? Head and Fearnsides believe that intracerebral lues is - characterized by a negative spinal fluid, under which circumstance - one has always to consider the possibility of brain tumor or - migraine in addition to the suspicion of syphilis. - - 3. What other causes besides syphilis should one consider for the - sudden diplopia? Brain tumor, multiple sclerosis, cerebral - arteriosclerosis, tuberculous meningitis, trauma and migrainous - ophthalmoplegia, are not infrequently at the bottom of this - condition. Cases also occur in which the etiology remains obscure, - even at autopsy. - -Under antisyphilitic treatment, Marini slowly improved. - - - =The SIX TESTS in TABETIC NEUROSYPHILIS (“tabes dorsalis”) may run - milder than in paretic neurosyphilis (“general paresis”) and - characteristically run somewhat like those of diffuse - (meningovascular) neurosyphilis; in particular, the fluid Wassermann - Reaction and the gold sol reaction are apt to run milder. The - clinical course of tabes dorsalis is protracted and the prognosis as - to life is good.= - - -=Case 29.= Mario Sanzi, 55 years of age, had been having what he called -rheumatism since his 43d year. This rheumatism affected only the hips -and legs, had at times been very severe, and for two years past had been -almost constant. Before that time, pains had come at intervals, lasted a -variable period, and suddenly disappeared. They were of knife-thrust -character, and could probably be called “lancinating.” In a given -attack, these pains would come at intervals of seconds or more. There -was also a certain unsteadiness in locomotion and inability to control -the vesical sphincter. - -=Physically=, the patient was entirely normal so far as could be made -out except =neurologically=. Argyll-Robertson pupils, absence of -knee-jerks, and ankle-jerks, Romberg sign, and characteristic gait, left -no cause for doubting the diagnosis of TABES DORSALIS. The blood and -spinal fluid both proved positive to the W. R., though the W. R. in the -fluid gave a negative reaction with 0.1 cm. and became positive with 0.3 -cm. or more. The globulin was somewhat increased though less markedly so -than in paresis. The gold sol reaction was “syphilitic” but weak. It is -to be noted that the disease had run a 12–years’ course before a doctor -had been consulted. The primary infection occurred at 32 years, namely, -11 years before the symptoms began. At the time of his primary -infection, Sanzi had received several years of treatment, chiefly in the -form of mercury by mouth. - - 1. What is the value of mercurial treatment of syphilis in the - prevention of tabetic or other forms of neurosyphilis? “Fournier - strove for many years to convince the medical profession that a - syphilitic patient should be treated for at least two years after - his infection, whether the syphilis seemed latent or patent. The - method of treating only the symptoms he characterized as the - opportunist method; treatment in the absence of definite symptoms - the preventive method, as preventing the later manifestations. - That prolonged treatment does prevent is shown by Fournier’s - figures analyzing 2396 cases presenting tertiary signs. These he - divides into three groups: Group I, comprising 1878 cases, or 78 - per cent of the whole number, having no treatment or inadequate - treatment—that is mercury for less than one year; Group 2, - comprising 455 cases, or 19 per cent, having moderate - treatment—that is, mercury for one to three years; and Group 3, - comprising the remaining 19 cases which represent only 3 per cent - of the whole number, having treatment for more than three - years.”[7] - - In the light of what we now know concerning latent neurosyphilis, - it would seem well for patients to be followed from time to time - with the W. R. on blood and spinal fluid after the supposed - completion of the treatment of primary and secondary syphilis. The - examination of the spinal fluid is not superfluous, as our - experience with the so-called _paresis sine paresi_ abundantly - shows. At the present day it is not good practice to assure a - patient that he is cured after two years of ordinary mercurial - treatment without resort to frequent spinal fluid tests, even - though the serum W. R. be negative. - - - =TABETIC NEUROSYPHILIS (“tabes dorsalis”) is often quite ATYPICAL - clinically and may even show no single symptom warranting the old - clinical name “locomotor ataxia.”= - - -=Case 30.= Stephen Green is a case of TABES DORSALIS with active -knee-jerks and without locomotor or muscle-sense disorder. When observed -at the age of 45, it appeared that there were but two complaints: lack -of control of the vesical sphincter and shooting pains in the legs. It -appeared that the urinary disorder dated back ten years, when there had -been difficulty in passing the urine. Sounds had been passed at the -time; occasionally there had been incontinence during after years, -ascribed by Mr. Green to the passing of the sound. However, the -physician at that time stated that the incontinence was a symptom of -tabes dorsalis. The incontinence had recently become worse, especially -marked at night, though also occurring in the day; much worse during -excitement, and very much worse after taking alcoholic drinks. Besides -incontinence, there is also difficulty at times in passing the urine, as -well as dysuria. - -As for the pains in the legs, they had been first noticed some three or -four years ago and considered to be mild rheumatic effects. Now, -however, they have grown progressively worse and have been the effective -cause of giving up business. The pains are sharp, darting, pinching, and -burning, and last, say, about a second with an interval of about the -same length. The attack will continue sometimes for many hours. - -There is a strabismus of the left eye, ascribed by the patient to an -accident with an umbrella (there had been operation without relief). The -pupils showed the Argyll-Robertson effect and were markedly irregular. -Despite the divergent strabismus with diplopia, the eye movements were -well performed although not in parallel axes. Ankle-jerks could not be -obtained even on reinforcement, but the knee-jerks were lively, and the -other deep and skin reflexes proved normal. The blood and spinal fluid -tests were characteristic of tabes dorsalis. - -It appears that the syphilis was acquired by this patient 15 years -before; that is, 5 years before neurological symptoms began. Three -courses of treatment had been taken at a well-known watering-place, and -mercury pills had been taken for two years by mouth. The patient is -married; has no children; there have been no pregnancies. - - 1. What causes may be assigned for the absence of children in the - family of a tabetic? There may be lesions of the genital apparatus - (orchitis, or more specialized toxic lesions). But impotence such - as characterized the present case must also be taken into account. - - 2. What is the therapy for tabetic pains? Pyramidon is nowadays much - in favor; morphine may be used; some authors recommend that the - patients be instructed to chloroform or etherize themselves - slightly for relief of the pain. Surgery of the nerve roots may be - resorted to in extreme cases. Intraspinous therapy, suggested by - various authors, seems to exert beneficial effect in many cases. - - 3. Is the lack of control of the vesical sphincter an unusual - initial symptom? On the contrary, the more careful the clinical - observation, according to some observers, the more likely is the - examiner to find that vesical symptoms were the earliest or among - the earliest complaints of the patient. Baldwin Lucke found - sphincter disturbances to be initial in 8¼% of his long Blockley - series. He found sphincter disturbance to occur in some stage of - the disease in 67.6%, being exceeded in frequency only by - staggering gait (87.2%) and lancinating pain (71.6%). According to - Lucke, the most frequent _initial_ symptom is lancinating pain in - the lower extremity, which, it will be noticed, occurred also in - our case of Stephen Green as an initial symptom along with vesical - disturbance. Lucke’s figures show that paresthesia of the lower - extremities (17.6%) and weakness of the extremities (16.4%) are - the next initial symptoms in frequency. - - 4. Could the early treatment in the case of Stephen Green be - considered as adequate? No better answer can be given to this - question than by quoting from Dr. Joseph Collins,[8] who probably - has done more than any other one man in this country in insisting - on the need of proper treatment of syphilis. As to the adequate - treatment of syphilis he says: - - “It consists in the proper use of salvarsan and mercury begun at - the earliest possible moment after infection and kept up till all - biochemical evidence of the disease has ceased, while the - metabolism of the individual is maintained as nearly normal as - possible. But the physician does not do his whole duty when he has - accomplished this. He must solicitously watch the individual to - see that no evidence reappears for months and even years after the - apparent cure. As an index of such reappearance the Wassermann - test of the blood serum and of the cerebrospinal fluid is the - safest guide. - - “Until there is a definite unanimity of belief among physicians as - to when the treatment of syphilis shall be begun, and some concert - of action as to what constitutes the adequate treatment of - syphilis, we cannot hope to make any considerable progress in the - prevention of syphilis of the nervous system, save by educating - the individual toward infection.” - - - =TABETIC NEUROSYPHILIS may produce symptoms chiefly if not entirely - in the region supplied by the CERVICAL plexus (“cervical tabes”).= - - -=Case 31.= Paul Halleck, 35, was a salesman who had begun to find it -hard to carry his sample case, since he was unable to tell whether or -not he had it in his hand. There was not only an anesthesia of the -hands, but they felt numb and there was often a tingling sensation. Of -late it had become hard for Halleck to dress himself or to write, and -these symptoms had been slowly growing worse. There was no other -complaint. There was, however, a history of a chancre about 7½ years -before, which had been followed by a rash and a sore throat. There had -been treatment with mercury and potassium iodid alternating for a period -of two years. - -=Physically=, there was no evidence of disease except =neurologically=. -The pupils were unequal (the right larger than the left) and reacted -slowly to accommodation and not at all to light. A marked ataxia of the -hands was shown in coat-buttoning. The finger-to-nose test showed a -marked dysmetria. Arm-jerks as well as knee- and ankle-jerks were -absent. There was a slight swaying in the Romberg position but no true -Romberg sign. There was no difficulty in locomotion. Both blood and -spinal fluid proved positive to the W. R.; globulin and albumin were -increased. The gold sol reaction was syphilitic, and there were 85 cells -per cmm. - -This case is probably not a pure example of CERVICAL TABES, since the -knee-jerks are also absent, and we may suppose a degree of lumbar spinal -cord changes in addition to the cervical changes. It well illustrates, -however, that the tabetic involvement of the cord may be quite -generalized and that it may strike high as well as low. - - - =ERB’S SYPHILITIC SPASTIC PARAPLEGIA.= - - -=Case 32.= Margaret Neal, a maid-of-all-work, 36 years of age, was -committed to a home for inebriates on account of her excessive -alcoholism, but she was shortly transferred to the Psychopathic Hospital -on account of difficulty with locomotion. We found a very marked -spasticity in walking, with a characteristic scissors gait. The pupils -were somewhat irregular, and although both reacted to light, the left -reacted far more slowly than the right and the reaction failed to hold -well. The arm reflexes were very active, and the knee-jerks and the -ankle-jerks were particularly exaggerated. There was a double Babinski -reaction, as well as Oppenheim and Gordon reflexes and a bilateral ankle -clonus. There seemed to be tenderness over the nerve trunks in the back -of the leg, below the knee. There was no evidence of incoördination, no -Rombergism, no disturbance of sensation, no disorder of the special -senses, and not even a tremor of the tongue or hands. - -=Mentally=, the patient was entirely negative. - -=Diagnosis=: Symptomatically, it is entirely clear that the patient was -suffering from SPASTIC PARAPLEGIA. One would have to consider besides -spinal syphilis, also amyotrophic lateral sclerosis, syringomyelia, and -spinal cord tumor. However, there appeared to be no definite wasting of -muscles, and the fact that the sensations were intact seems to rule out -also syringomyelia. There was none of the characteristic pain associated -with a cord tumor. There was, in fact, a strong clinical premonition -that the case was one of spinal syphilis, simply because syphilis is the -most common cause of spastic paraplegia in the adult. The pupillary -anomalies were also highly suggestive. - -The serum W. R. proved to be weakly positive, as was also the gold sol -reaction in the zones characteristic of syphilis. The spinal fluid -examination yielded 14 cells per cmm. There was a positive globulin test -and a moderate increase in albumin. The W. R. of the spinal fluid was -negative. - - 1. Why was the spinal fluid W. R. negative in this case of spinal - syphilis? The explanation of negative W. R.’s in spinal syphilis - is not easy. Possibly, however, in the course of years the - intensity of the process has been reduced and possibly the W. R. - has been one of the first tests to disappear. - - 2. How shall we explain the nerve trunk tenderness? We might - consider this to be due possibly to an inflammation about the - posterior roots. On the whole, partly on account of the situation - of the pains below the knee, it seems probable that the nerve - trunk tenderness of this case is the residuum of an alcoholic - neuritis. - -=Treatment=: Under injections of mercury salicylate, there was a rapid -improvement. In fact, in the course of several months, the patient -regained an ability to walk long distances. There still remains a -certain spasticity, but the abnormal spinal reflexes above mentioned are -no longer present. - - - =SYPHILITIC MUSCULAR ATROPHY, probably due either to spinal - parenchymal lesions, or to root neuritis, or to both.= - - -=Case 33.= Joseph Graham, now 50 years of age, seemed no longer to be -able to do good work as a teamster. His arms had become weak and the -muscles had become tremulous and apparently wasted. There was also pain -in the left leg and hip. It appears that this latter symptom had been -thought to be rheumatism, having begun about 8 years before with a -sudden sharp shooting pain in the left hip, about the region of the -sciatic notch. Graham had rubbed the hip with liniment, but without -reducing the so-called rheumatism. The trembling of the hands had begun -some years later, but no wasting had been noticed except during the past -year. The pain in the leg had suddenly become so severe that a month -before medical observation he had quit work. The question immediately -arose whether Graham was not suffering from some familial form of -muscular atrophy; but according to his representations, there was -nothing of the sort in the family. - -=Physically=, there was little to note. =Neurologically=, there was -more. The pupils were somewhat irregular in outline, and the right was -larger than the left. The left pupil failed to react to light, and the -right pupil reacted very slowly and with but a slight excursion. There -was no tremor of the tongue and no evidence of facial palsy nor was -there smoothing of the nasolabial folds. It was somewhat remarkable, -that in the absence of these signs, there was a marked speech defect. -The atrophy of arms, forearms, and hands was well marked, especially the -atrophy of the thenar and hypothenar eminences of the right hand. The -extended hands, especially the right, showed a marked coarse tremor. -Fibrillation was found in the muscles of the hands, forearms, arms, and -pectoral muscles. There was no dysmetria, and the diadochokinesia was -normal. Strength was diminished (dynamometer right hand, 32 kg., left 31 -kg.). There was little or no atrophy of the legs, although the left -thigh was perhaps slightly atrophic and the gluteal muscles of the left -side were somewhat flabby. The patellar and Achilles reflexes were -absent on both sides. There was a slight swaying in Romberg position. -Gait was normal. There was a marked tenderness on the left side of the -sciatic notch, as well as over the entire distribution of both external -and internal popliteal nerves. This area of skin was also hyperesthetic. -There were no other neurological signs on systematic examination. - -=Diagnosis=: The sensory disorder, the speech defect, and the pupillary -abnormalities seem to render the diagnosis of progressive muscular -atrophy doubtful. Nor was there any dissociation of sensations to -suggest a syringomyelia. Under such circumstances, one must fall back -upon the question of syphilis. Both blood and spinal fluid proved to be -positive to the W. R.; the globulin was increased and the albumin -markedly so; there were 61 cells per cmm., and the gold sol reaction -read 4 4 4 4 3 2 1 0 0 0. - - 1. Is there a relation of SYPHILITIC MUSCULAR ATROPHY to amyotrophic - lateral sclerosis? Spiller, some years since, claimed such a - relation, and it would seem with some justice. - - 2. How shall the present case be classified? There is evidence of - root pains (left hip). We may naturally suppose that these root - pains are reasonably good clinical evidence of a meningitic - lesion, of which the spinal fluid clinically gave a confirmation. - The fibrillation in this case somewhat suggests, however, a - central origin for the muscular atrophy. Accordingly, it would be - difficult to definitely classify the present case as either one of - meningovascular syphilis or one of central syphilis. It will be - remembered that Head and Fearnsides classify muscular atrophy - under both these headings. - - - =The period of SECONDARY SYPHILIS is frequently (over a third of all - cases?) MARKED BY approved signs of NEUROSYPHILIS precisely like - those of full-blown paretic or diffuse (meningovascular non-paretic) - neurosyphilis. These signs occur sometimes in association with - severe clinical symptoms, sometimes without clinical symptoms.= - - -=Case 34.= John Bennett, 28, was brought to the Psychopathic Hospital -much confused. His brother, who came with him, said that he had been a -very heavy drinker but had given up drinking about four months before. -He had recently had a cold but was otherwise in good health up to the -night before admission. On this night, Bennett had become suddenly -excited and went into his mother’s room, at the common home, and began -to curse her. However, he was put to bed safely, but on the next morning -began to moan continuously. After some hours of moaning, he was brought -to the hospital. Here he remained difficult to manage, being irritable, -noisy, and resistive. Questions he either would not or could not answer, -and there was even no evidence that he understood questions. However, -within a few hours, it was clear that he was slowly coming out of the -confused state. On the following day, it was possible even to rouse him -and get his name. The confusion gradually cleared still further and, by -the end of three days, he had become mentally absolutely well so far as -could be determined. - -He then informed us that he had had a chancre about five or six months -before, followed by a secondary skin eruption; that he had received four -injections of salvarsan (the last, a month before admission) and three -injections of mercury. At about the time of the last injection of -salvarsan, he had developed headache with pain and slight stiffness in -the back of his neck; and a fortnight later, he began to have dizzy -spells, followed during the last week by difficulty in hearing. There -was amnesia for everything that happened after his spell of sudden -excitement on the evening before admission, and this amnesia was never -lifted for the four days that followed. - -=Physically=, Bennett was very well built and muscular. Nor were there -any evidences of disease outside the nervous system. There was some -slight stiffness of the neck and slight pain on movement of the head, -which probably ought to be attributed to meningitis. The =neurological -examination= showed tendon reflexes all normal, and normal sensations. -There were, in fact, no neurological signs except that both pupils were -dilated; the left was larger than the right. Both pupils reacted to -light but reacted very poorly. They reacted much better to -accommodation. - -The W. R. proved to be positive, as might well be expected in a man -whose infection had taken place less than six months before. The -globulin and albumin of the cerebrospinal fluid were in great excess, of -a degree which we clinically express by ++++. The W. R. of the fluid -also was strongly positive down to 0.1 of a cmm. The gold sol reaction -was the “paretic” type, and there were 228 cells per cmm. - - 1. How early may clinical evidence of neurosyphilis set in after - infection? Craig found one case of “brain syphilis” occurring one - month after infection. Frye claims a case of tabes dorsalis - developing six weeks after infection. Craig states that he has had - three cases of brain syphilis occurring within six months, and six - within a year of infection. - - 2. What effect did the salvarsan injections have in causing or - preventing the symptoms in this case? Nonne sums up the - neurorecidive question as follows: Since the introduction of - salvarsan therapy for neurosyphilis, paralyses of various cranial - nerves are seen more frequently. This higher frequency is in part - only apparent since more attention has been paid of late to - auditory and labyrinthine disorders. On the whole, however, it - must be considered that salvarsan does mobilize spirochete foci - which without salvarsan therapy would perhaps have remained - latent. Probably we are here dealing in some instances with fresh - infections of neurosyphilis, in other cases with a Herxheimer - reaction. Ehrlich believed that these latent foci occur - particularly in places with stagnant blood current; as, for - instance, in the narrow bony canals. This hypothesis, sufficient - in some instances, is less satisfactory for cases of peripheral - neuritis, for example. - - 3. What treatment is indicated? Intensive antisyphilitic treatment - is strongly indicated. Whatever may be the truth concerning the - production of neuro-recurrences (“neurorecidives”) it is certain - that the symptoms usually vanish with a continuance of salvarsan - therapy. The important point is to give efficient treatment, and - in a case like Bennett’s improvement is fairly certain unless some - serious insult occurs before the remedial efforts have been given - time. It is still an open question whether intraspinous treatment - is more efficient in such cases than intensive intravenous - injections of salvarsan. In Bennett’s case diarsenol was injected - intravenously twice a week in 0.6 gm. doses, reënforced with - intramuscular injections of mercury salicylate and potassium iodid - by mouth. Under this treatment improvement began slowly and in a - few months he was symptomatically well and after three months his - tests were practically negative. - - - =JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”) with OPTIC - ATROPHY.= - - -=Case 35.= Mary Coughlin, a blind girl of 16 years, was brought to the -hospital in a state of great excitement, laughing and crying -alternately. The neurologist is entitled to think of blindness, and -particularly of the optic atrophy which Mary showed, as probably due to -syphilis. However, there was no history of syphilis in the father, who -died in an accident at the age of 40, or the mother, who died at 45, of -heart trouble. An elder sister was married and well; two younger sisters -were living and well. The fifth sibling, a boy, had died in infancy. -There had been no miscarriages. In fact, the only point in favor of -syphilis was the somewhat far-fetched point that the younger brother of -the patient had died in infancy. - -The patient’s history was rather suggestive of some other diagnosis. Her -birth had been normal, she walked and talked at 13 months, was at school -from six to twelve, reaching the seventh grade, and was considered -bright. At three years of age, she had been run down by a car and -dragged under the fender for a considerable distance. Her head was hurt -but the patient did not lose consciousness in the accident. Fainting -spells began at 11, in which spells the patient would lose consciousness -for a minute or two. About this time, the patient’s eyesight had begun -to fail, and for some four years she had been entirely blind. Headaches -had come on of late. - -The Coughlin case, except for the above-mentioned suspicion of -syphilitic optic atrophy, might be regarded as an unusual example of a -post-traumatic disease. - -We found her to be fairly well developed and nourished; there was a -deformity of the lower half of the sternum and of the third and fourth -ribs on the right side. There were no other physical phenomena found -upon systematic examination. The left pupil still reacted to light; the -right failed to react, but this lack of reaction could not be regarded -as of Argyll-Robertson nature on account of the finding of optic atrophy -with the ophthalmoscope. - -=Mentally=, it appeared that the patient’s retention of school knowledge -was poor, though her blindness for four years had doubtless given her -little opportunity to keep such information fresh. Rather strangely, -Mary gave utterance to many delusions: first, expecting to receive her -sight by an operation on the head; second, to write a book of her -doings; third, to buy a house for the children; fourth, would pay $3000 -for the house, earning the money by working at a tailor’s or as a -trained nurse; fifth, to go on the stage to earn money by dancing; -sixth, will have lots of money. - -One of Mary’s characteristic statements is as follows: “Won’t it be -lovely when I can see Dr. H.’s face in heaven or some other lovely -place? Dr. H. was a grand doctor to me, and when we get together again -we are going to Tremont Temple and keep us together. I am going to do -some dancing and play the piano. I am going to graduate at the high -school and go to Trinity College in Washington, and I hope I shall be a -faithful keeper of mother’s tomb.” - -The patient was at times euphoric and expansive. - -At this stage, what with optic atrophy, euphoria, and expansive -delusions, we should perhaps be entitled, had Mary been an adult, to -offer the diagnosis GENERAL PARESIS. In fact, on the whole, any other -than a syphilitic cause for the optic atrophy was exceedingly doubtful. -Brain tumor of a nature to produce optic atrophy might very improbably -last so long as five years. There was no evidence of any intoxication at -the time when the blindness occurred. - -The W. R. was positive in the blood and spinal fluid; there was a -positive globulin test, and an excess albumin as well as 15 cells per -cmm. - - 1. What is the significance of Mary’s trauma at three years? So far - as we are aware, none. - - 2. What light could be thrown by a W. R. study of the family? In - some instances, much light is thrown; in the present case all - three living sisters of the patient have been examined and their - serum W. R.’s have been found negative. - - 3. What is the prognosis of juvenile general paresis? Death within a - few years, as in general paresis in adults. The patients live - rarely more than four or five years after the onset of symptoms. - Mary Coughlin died a year and a half after the above examination, - namely, in her eighteenth year, some seven years after the onset - of symptoms. - - 4. What can be said of treatment? A few favorable results have been - reported after intraspinous therapy (Swift-Ellis). Too little work - has been done with systematic treatment of juvenile neurosyphilis, - both paretic and non-paretic, to permit important conclusions at - this time. - - 5. How can we explain the infection of this sibling whereas the - others, both younger and older, escaped? It would seem that we - would have to discard the hypothesis of a congenital infection and - consider that it was acquired accidentally during the lifetime of - the patient. Considering the prevalence of syphilis it is rather - to be wondered that more such cases of “innocent” infection do not - occur in children. We may recall how many instances of juvenile - gonorrhea occur. In a case as this where the symptoms calling - attention to syphilis necessarily occur so long after the original - infection it is practically impossible to trace the origin of the - infection. - - - =The diagnosis of JUVENILE PARESIS is often easy.= - - -=Case 36.= Theresa Mullen, an under-sized girl of 12 years, presented a -remarkable appearance due to congenital amputations of the fingers and -toes. She lay in bed, drivelling and making unintelligible cries. It -appeared that the patient weighed about 12 pounds at birth and was very -fat; that she had been fed on condensed milk, had survived cholera -infantum, whooping cough, and, as the parents said, “two kinds of -measles.” - -Theresa had gone to school at 5 years, reaching the third grade at the -age of 9; but at this time, she began to lose ground and was put in a -class for backward children. Moreover, at about this time, the teachers -noticed spells of causeless laughter and meaningless twisting back and -forth. Theresa would also scream at night, looking about the room; once, -rising and crying, “Take him away, that black thing,” though no -appropriate object was present. There had been little or no complaint of -headache. Theresa had been deteriorating for some time, and for a year -past had been having increased difficulty in walking. For two months the -child had not spoken intelligible words; for the last week, she had been -incontinent. - -The =diagnosis= was almost obvious from the manual and pedal deformities -taken in connection with the saddle-back deformity of the nose. It was -interesting in connection with the contentions of W. W. Graves, that the -scapulae were scaphoid in type. - -Accordingly, the history given by the parents seemed consistent enough. -The parents were both 36 years of age, having married at 23. The first -pregnancy was a miscarriage at two months, of unknown cause. Theresa -came next; thirdly, came a miscarriage at three months; fourthly, a -girl, who is not strong or well physically, has suffered much from -headaches and sore throat, but is fairly bright. The fifth pregnancy -resulted in a boy, who is bright but of under-size. Three more -pregnancies resulted in miscarriage. - -Taking into account the above-mentioned physical characteristics, the -personal history, and the family history of Theresa, the diagnosis could -hardly be in doubt even in the absence of a lack of pupillary reaction -to light on the right side, infantilism of genitalia, positive W. R.’s -of serum and spinal fluid, positive globulin, and excess albumin, 34 -cells per cmm. and the paretic type of gold sol reaction which were -found. - -The =prognosis= of this case appears to be rapid deterioration, -terminating in death within a few months. Now and again, however, some -such cases spontaneously improve. Such a case as that of Theresa Mullen -is always disheartening in itself but suggests the social value of -Wassermann tests in the other members of the family. The other children -of the Mullen family proved to be suffering also from syphilis, since -their blood sera all showed a positive W. R. - - 1. What is the characteristic age of onset in JUVENILE PARESIS? An - impression has prevailed in some quarters that the typical onset - of juvenile paresis is in the adolescent years, and Clouston’s - first case (1877) developed in a boy of 16. Thierry’s 58 cases, - developing from the 8th to the 20th year, averaged 14 years of age - at onset. Mott’s 22 cases from the 8th to the 23d year, averaged - 17 years at onset. According to Clouston, juvenile paresis - develops most often at puberty (15 to 17 years). It is sometimes - claimed that cases developing symptoms early live longer, and that - juvenile cases developing symptoms after the 20th year run a short - course. For a case developing in the 5th year, see John - Friedreich, Case No. 77. - - 2. What may be concluded from the physical signs (congenital - amputations) present in this case before the development of mental - symptoms? Some cases of juvenile paresis appear to show no - physical signs whatever in childhood. While these amputations - might be the accidental result of a difficult delivery, it is more - probable that they are due to a syphilitic process. - -[Illustration: - - Juvenile paresis—congenital amputation of digits. This case reached - fourth grade in school before deterioration. -] - - - =CONGENITAL SYPHILIS is apparently capable of producing simple - FEEBLEMINDEDNESS (that is, a form of disease non-paretic, - non-tabetic, without special tendency to progression, and without - tendency to vascular insults).= - - -=Case 37.= Isaac Goldstein was a small boy of six years and seven -months, with a father known to be suffering from general paresis. The -child was very irritable and nervous and very difficult to manage, but -would hardly have been the subject of medical attention except in a -family study suggested by the paresis of the father. - -The child had been born at term and had apparently undergone a normal -development. Physically, he showed no definite signs of congenital -syphilis. In fact, the physical examination was to all intents and -purposes negative. The W. R. of the serum, however, proved to be -positive. Mental tests showed that his mental age was that of a child of -a little over five years. Taking all things into account, it is probable -that he should be regarded, therefore, as somewhat retarded mentally. - - 1. Is syphilis answerable for the mental retardation in this case? - Provided that the family is free from feeblemindedness and mental - disease, it would seem that the retardation of a congenital - syphilitic should perhaps be regarded as syphilitic in origin. Of - course, the institutions for the feebleminded have not shown - exceedingly high percentages of syphilitic children in various W. - R. surveys; still, the percentage of positive reactions in - institutions for the feebleminded is clearly higher than the - incidence of congenital syphilis shown in the population at large. - Hence, we may conclude that syphilis is one of the etiological - factors in the production of feeblemindedness. Dr. W. E. Fernald, - of the Waverley School for the Feebleminded, has recently pointed - out that the syphilitic cases belong rather in the lower grades - (idiots and imbeciles) of feeblemindedness than in the higher - (morons). - - 2. Can we guess what the pathological anatomy and histology of the - brain may be in such cases? The Waverley studies now in process - seem to indicate that some cases have little or no gross - alterations, but show a few slight traces of lymphocytic - accumulations discovered upon extended search, and a certain - tendency to the appearance of rod cells in various foci. But the - whole matter is still _sub judice_. It is a question whether these - traces of chronic inflammation are the residuals of a more active - process or the beginnings of a process that is about to be more - active. - - 3. How characteristic is a positive W. R. in the serum of a child - without physical stigmata of congenital syphilis? If we limit the - term _stigmata_ to the major and more important signs, we must - reply that it is not unusual to find positive W. R.’s in sera of - physically normal-looking children. Except in family studies, such - cases will often escape notice, either because there are no - stigmata whatever, or because such stigmata as exist are of a - minor nature and regarded as unimportant anomalies. Some of these - cases occur in the clinics later in life as so-called _syphilis - hereditaria tarda_. If one wishes to discover these cases with - late development of symptoms before their full bloom, the most - obvious method is to examine carefully the children of known - syphilitics. - -[Illustration: - - Scaphoid Scapulae. -] - - - =JUVENILE TABETIC NEUROSYPHILIS (“juvenile tabes”); TREATMENT.= - - -=Case 38.= The point in presenting Archibald Sherry, a JUVENILE TABETIC -of 12 years on admission, is perhaps to exhibit pride in therapeutic -results. - -There was little or no doubt of the diagnosis; in an adult, the -phenomenon would be called tabes dorsalis with a question of general -paresis. The right pupil was larger than the left and reacted neither to -light nor to distance. There was a slight tremor of the tongue and of -the outstretched hands. The knee-jerks and ankle-jerks could not be -obtained, nor could the periosteal reflexes in the legs. There was a -slight unsteadiness in the gait and in various finer movements, and a -slight ataxia of the legs. There was not a classical Romberg sign but -there was slight swaying in Romberg position. The teeth were -Hutchinsonian. For the rest, the physical examination was practically -negative. - -The family history was of interest. On the paternal side there was -nervousness as well as alcoholism and degeneracy. The maternal -grandmother had cancer. Archibald’s father was immoral and alcoholic. -There was a girl four years older than Archibald, who, though nervous -and unstable, has shown no signs or symptoms of syphilis and does not -yield a W. R. in blood or spinal fluid. - -Archibald himself was born at term, a large child, who, however, lost -weight rapidly, developing a marked skin eruption on head and back three -weeks after birth. This skin disease lasted for a month and a half and -then spontaneously disappeared. Archibald remained weak and sickly, not -walking until three years of age. However, he did well in school up to -the end of his 11th year, when he failed to keep up with the children. -He had been an amiable child and had gotten on well with his playmates. -Some time in his 10th year physical disability had begun; there was -numbness in the legs with weakness; at times, actual inability to walk. -The right pupil was noticed by the mother to have increased in size; the -eyelashes had turned white. There was pain over the left eye and a -feeling of weight on top of the head. Speech became difficult or even -confused. - -Consistently enough, the W. R. both in blood and spinal fluid was -positive. Globulin and albumin were present in large amounts; there were -150 cells per cmm. - -Granting that this be in some sense a case of juvenile tabes we may -raise a doubt whether the case is one of congenital syphilis. The W. -R.’s of the blood of both father and mother are negative. Syphilis is -denied by them. The nervous and unstable older sister failed to show -definite symptoms of syphilis or a positive W. R. There had been no -miscarriages or stillbirths. The question arises whether the -Hutchinsonian teeth do not indicate congenital syphilis. It appears, -however, that it is possible to develop Hutchinsonian teeth if syphilis -is acquired before the teeth are formed. We have no data as to how or -why this particular baby should have acquired syphilis, if he did so -acquire it, at the age of three weeks. On the whole, sceptics may doubt -our suggestion that the case is one of acquired juvenile tabes. Possibly -the question is academic so far as treatment is concerned. - -=Prognosis=: The rarity of juvenile tabes is such that little can be -said as to prognosis. Three and a half years have passed since a few -injections of salvarsan were made. The pains above mentioned rapidly -disappeared, the gait became steadier, the attacks of confusion ceased, -and the speech improved. Unfortunately, on account of a lack of -coöperation on the part of Archibald’s mother, we have been unable to -continue treatment. However, we have from time to time followed the -patient in his home and he seems to have shown no falling back after the -initial improvement. It would be of great value could we know the -situation in the spinal fluid at the present time. - - 1. Is there any explanation why paresis should occur in some - juveniles and tabes in others? There is no available explanation - for this difference nor any for the characteristic early optic - atrophy of juvenile tabetics. - - - - - Be frustrate, all ye stratagems of Hell, - And, devilish machinations, come to nought! - - Paradise Regained, lines 180–181 - - - - - III. PUZZLES AND ERRORS IN THE DIAGNOSIS OF NEUROSYPHILIS - - -This part of the case collection, dealing with puzzles and errors, is -ushered in by six cases (39–44) drawn from a group of errors in -diagnosis made some years since at the Danvers Hospital. These six are -autopsied cases. Attention is called to the fact that modern methods of -diagnosis might have prevented the errors. - - - =DIFFUSE NEUROSYPHILIS (“cerebrospinal syphilis”) versus PARETIC - NEUROSYPHILIS (“general paresis”). Autopsy.= - - -=Case 39.= Caroline Davis, dead at 49 years, was a case of error in the -diagnosis of general paresis. Like Cases 40 to 44, Case 39 was -diagnosticated by the full Danvers staff as a case of general paresis; -however, it must be added, before the days of the W. R. and the modern -methods of systematic diagnosis. As will transpire in the sequel, there -is a large question whether Case 39 is not after all really a case of -neurosyphilis, possibly not of the paretic group. The details are as -follows: - -Caroline Davis was a normal school girl till 15, apt in studies, mill -worker till marriage at 18; one child, dead (cause unknown). Habits -good. Moderate deafness set in in the forties and in 1901 patient became -completely deaf in three months’ time. In 1905 she became unable to take -care of her house and had a shock in which the right leg was affected. - -On commitment patient showed good development and nutrition with slight -enlargement of capillaries of cheeks, redness and roughening of skin of -right ankle. Teeth absent. Slight radial and brachial arteriosclerosis. -Urine negative. Sluggish pupil reactions to light both directly and -consensually. Deafness absolute, bone conduction defective. Arm reflexes -brisk, knee-jerks equal, brisk. Bilateral Babinski reaction more marked -on the right side, tremor of tongue, Romberg’s sign, gait defective. -Speech stumbling, writing clear, without tremor. - -Communicated by writing only. Consciousness normal, disorientation for -day of month, for place (misnames hospital) and for persons (recognizing -nurses, not patients). - -Patient wrote many letters complaining of pain, headaches and especially -of pain in the abdomen and side. The patient was thought to show a -slight defect of memory, but her deafness rendered diagnosis difficult. -The patient died suddenly on May 23, 1908, shortly after supper, falling -backwards, and dying in five minutes with marked respiratory distress. - -=Post Mortem Findings.= The =cause of death= was not clear. The heart’s -blood and cerebrospinal fluid were sterile. There was a small hemorrhage -in the anterior part of the right ventricle derived from a small artery -of the caudate nucleus. There was about 400 cc. of blood between the -dura mater and the pia mater. There was a slight sclerosis of the basal -and Sylvian arteries. The brain substance was uniformly softer than -normal. - -It is possible that the hemorrhage had taken place some time before the -patient’s fall and that the brain substance had swollen in consequence. -Just before the fall she had a weeping spell. - -The =anatomical diagnoses= were as follows: - -Obesity, unequal pupils, fresh wound near left ear, edema of legs, -slight focal adhesive pleuritis, hypostatic congestion of lungs, chronic -endocarditis, chronic myocarditis, congestion of kidneys, congestion of -pancreas, subacute splenitis, chronic adhesive pelvic peritonitis, -hematoma and cystic condition of Fallopian tubes, =calvarium dense= and -thick, subdural hemorrhage, slight =chronic leptomeningitis=, general -=cerebral atrophy=, marked in tips of =frontal lobes=, old =cyst of -softening= between left corpora albicantia and optic chiasm, small -punctures of left ear drum, drums opaque, =chronic spinal -leptomeningitis=; brain weight, 1190 grams. - -There were marked firm interadhesions between dura and pia throughout. A -lumbar puncture soon after admission in 1907 had shown: - - Per cent - Endothelial cells 10 - Lymphocytes 30 - Plasma cells 0 - Phagocytes 0 - Polymorphonuclear cells 51 - Unclassified 9 - Fibroblasts 0 - Cells in 100 fields 125 - -It will be noted that the lumbar puncture yielded no plasma cells and -yet showed 30% of lymphocytes. Alzheimer, in 1904, attempted to -distinguish the histology of the cerebral syphilitic from that of the -general paretic, maintaining that _lymphocytosis was the characteristic -feature of the ordinary neurosyphilitic_, _whereas plasma cells were -associated with the lymphocytes in the paretic_. This case showed -=lymphocytic= deposits. To be sure, they were decidedly subordinate in -the cerebral cortex, cerebellum, and basal ganglia, to the marked -evidences of nerve cell destruction, although there were perivascular -infiltrations about a few of the larger vessels in the white matter of -the cerebral cortex. - -The spinal cord, however, showed a most severe infiltration, especially -in the gray matter, where the infiltration accompanied severe nerve cell -changes and arterial changes. The pia mater of the spinal cord was also -packed with mononuclear elements, among which, however, no plasma cells -could be found. - -But although the inflammatory changes in the shape of lymphocytosis were -relatively more prominent in the spinal cord than in the cortex, yet the -cortex yielded evidence of an exceedingly marked destructive process. -Perhaps no layer of any of the areas of the cortex examined failed to -show some atrophic alteration. The upper layers of the cortex were -everywhere more severely diseased than the lower layers. Here we are -dealing with an instance of an active meningomyelitis and subcortical -encephalitis. It is, of course, probable that the W. R., had it been -performed, would have been positive in this case. On the basis of the -histology, we are inclined to regard the clinical picture in this case -as belonging among cases of NON-PARETIC DIFFUSE NEUROSYPHILIS. - -This case, as also the next several, is especially instructive in -teaching the difficulty in differentiating paretic and non-paretic -neurosyphilis. Not only is this difficulty met in clinical diagnosis, -but in pathological diagnosis as well. - -The histological diagnosis depends in large part on the work of the -Nissl-Alzheimer school, which has received great recognition. At the -present time, however, there is beginning to be considerable doubt as to -the entire validity of this teaching. At any rate there are many -borderline cases in which the differentiation is well nigh impossible. -In this case note chronic meningoencephalitis, with cortical -degeneration, in the absence of plasmocytosis. - -From the clinical standpoint the intensity of the W. R., the character -of the gold sol reaction, and the result of therapy have added new -points in differentiation. Much more work controlled by autopsies is -still needed, however, to put us on sure ground in borderline cases. - - - =VASCULAR NEUROSYPHILIS(?) versus PARETIC NEUROSYPHILIS (“general - paresis”). Autopsy.= - - -=Case 40.= Case 40 like Case 41 was an error in the diagnosis of general -paresis which might be regarded as academic rather than practical. Both -were cases of arteriosclerotic brain disease with severe cerebellar -involvement. Case 40 had a spinal cord that was not quite normal. There -was a tabetiform lesion in the cervical spinal cord (not elsewhere), -together with a unilateral degeneration suggesting in some respects a -radicular origin. The most striking feature, however, of Case 40 as in -Case 41, was a lesion of the cerebellum. In Case 40 the dentate nuclei -were in large part destroyed by cysts of softening, although the -cerebellar cortex was fairly well preserved on both sides. The details -of Case 40 are as follows: - -H. F., male, gear maker, born 1850. - -=Heredity.= Maternal grandmother insane. Mother insane at 52, became -demented and lost use of limbs, died at 71. Aunt insane. - -=Personal History.= Common school education. Capable workman till within -a few months. Early in life alcoholic. Drunk almost every week until -1899 or 1900. Irritable, nervous, selfish, loose in relations with -women. Venereal disease denied by wife. Married in 1883. Three frail -children. No miscarriages. Neuralgia in 1901 or 1902. - -January, 1904, patient left carriage shop on account of mistakes in -work, became more pleasant, childish, fearful, talkative, did funny -things, later became vagrant, stole from fruit stores, smoked cigarettes -picked up in the street, and became restless and irritable. - -Committed to Danvers, June 24, 1904, with slightly enlarged heart, -somewhat heightened blood pressure, and a slight sediment of epithelial -cells in urine. - -Romberg’s sign was present, but there was little or no demonstrable -incoördination otherwise. Very slight tremor of fingers. Left knee-jerk -absent, right obtained on reinforcement. Achilles jerk absent. Triceps, -wrist and normal plantar reflexes present. Pupils react to -accommodation, but very slightly, if at all, to light. Sensations normal -except in legs. The legs show preservation of tactile and temperature -senses, but abolition of pain sense except over dorsum of foot. - -Speech showed slurring of syllables and “brigrade” for “brigade.” -Disorientation for time, place and in part for persons. Admitted that -his work had been deficient but regarded himself as well. Emotionally -variable, crying at times and suddenly becoming jocular. Eloped July 3 -and somehow reached his wife’s house in a neighboring city. - -Euphoria persisted. The pupils continued Argyll-Robertson, and the -knee-jerks remained absent. Became oriented for place and partially as -to time (month and day of week correct). - -During 1905 failure became rapid, with ataxia of legs, persistent -euphoria, and loss of weight. - -Convulsions, regarded as general paretic, developed in 1906. Death -sudden, December 7, 1906. - -=Post Mortem Findings.= The =cause of death= was streptococcus -septicemia, probably derived from a gangrenous bronchopneumonia or -related with a small thrombus of the right auricular appendix. There was -also an acute purulent otitis media, mastoiditis and sphenoidal -sinusitis, as well as extensive decubitus. From this decubitus or from -the intestinal tract may have been derived the numerous colonies of -_bacillus coli communis_ which developed on plates from the -cerebrospinal fluid. - -=Arteriosclerosis= was little in evidence, being confined to the -coronary, right vertebral and carotid arteries (slight in all). _Cysts -of softening existed in the posterior part of each dentate nucleus_ and -may probably be interpreted as indicating vascular disease. - -=Chronic disease outside the nervous system= was prominent and in part -suggestive of senile findings; milky patches of pericardium, adhesions -about liver and gall-bladder, adhesions about spleen, adhesions and -fibrous thickening of parietal peritoneum, adhesions in both pleural -cavities, chronic diffuse nephritis, hypertrophy of bladder wall, dense -calvarium, dural adhesions. - -The =nervous system= showed several unexpected features. The _absence of -chronic leptomeningitis_ was striking: the pia mater was everywhere -delicate and transparent except that the walls of the cerebellar and -chiasmal cisternæ were thickened and that there were slight opacities -along the sulcal veins of the convexity. Brain weight 1090 grams. There -was a generalized =sclerosis and pigmentation of the cerebral cortex=. -The sclerosis varied in degree and was most marked in the prefrontal -regions, the anterior halves of the superior frontal gyri, the middle -third of the right precentral gyrus, the region of the splenium on the -left side, and the sagittal rami. If the _bacillus coli communis_ found -in the cerebrospinal fluid had any effect upon the consistence of the -brain, obviously hard to prove in a brain of leathery consistence at the -outset, it was shown only in the right Rolandic area in the vicinity of -the sclerotic part of the precentral gyrus. =Granular ependymitis= of -all ventricles. Weight of cerebellum, pons and bulb, 135 grams. - -Perhaps the most remarkable feature of all in the case was the -occurrence of =cysts of softening= in the posterior part of each -=dentate nucleus=. For discussion, see Case 41. - - - =VASCULAR NEUROSYPHILIS (?) versus PARETIC NEUROSYPHILIS (“general - paresis”). Autopsy.= - - -=Case 41=, like Case 40, was one of arteriosclerotic brain disease with -severe cerebellar involvement. Here is another case in which the Danvers -staff made a diagnosis of general paresis without dissenting voice. -There were some tabetic symptoms, and the spinal cord at autopsy did -show a moderate lymphocytic infiltration of the meninges, entirely -consistent with the picture in the spinal fluid. In this case, the -dentate nuclei of the cerebellum were not destroyed as in Case 40, but -were affected by cell atrophies of variable degree in different parts of -the nuclei. There was also a severe gliosis of the cerebellar cortex. -The left hemisphere of the cerebellum was more severely diseased than -the right. The cortex showed far more marked and generalized cell -atrophies throughout the layers than did Case 40. The details of this -case, which was that of a colored coachman, Samuel North, are as -follows: - -He was born in 1871. Learned to read and write at school. Stableman and -coachman. Alcoholic till 1902. Took much quinine, possibly impairing -hearing thereby. Memory impaired and growing worse since 1902. Gait -unsteady for a longer but unknown period. August 13, 1907, wandered -about, instead of attending boot-black stand, muttered, talked -incoherently. In the next few days talked about religion and apparently -had hallucinations of hearing. Committed August 16, 1907. - -On commitment stoop-shouldered, flat-chested. Gait staggering. -Unsteadiness in Romberg’s position. Incoördination of arms and fingers. -Coarse tremor of tongue. Tremor of lower jaw. Exaggeration of left -knee-jerk and diminution of right. Exaggerated Achilles jerks. Spurious -left ankle clonus. Questionable Babinski reaction of left side. -Abdominal and epigastric reflexes present but cremasteric absent. Left -pupil smaller than right and fails to react to light. Reaction of right -pupil sluggish. Moderate defect of hearing of both sides. - -During the first week the patient developed hallucinations of sight and -hearing, but of no other senses. Disorientation for time, place, and -persons. Answers to arithmetical problems given with assurance but as a -rule incorrectly (as 17 and 32 are 90; 18 divided by 3 is 88). -Handwriting scarcely legible. Memory poor, especially for recent events -(recalled a lumbar puncture as an exercise in baptism). Impressibility -and attention poor. Euphoria. - -Death after gradual failure July 29, 1908. - - Lumbar puncture showed: Per Cent. - Endothelial cells 9 - Lymphocytes 81 - Plasma cells 6 - Phagocytes 0 - Polymorphonuclear cells 4 - Unclassified 0 - Fibroblasts 0 - Cells in 100 fields 700 - -=Post Mortem Findings.= The cerebrospinal fluid showed a pure culture of -_Bacillus coli communis_, and the heart’s blood showed many colonies of -an unidentified bacillus. Culture from mesenteric lymph nodes sterile. - -The =cause of death= is somewhat in doubt. There was an early pneumonic -process with fibrinous pleurisy, and there was an early acute -hemorrhagic ileitis with a very slight overlying peritonitis and slight -corresponding enlargement of mesenteric lymph nodes. There was an -infection of the meninges with _Bacillus coli communis_. - -Evidences of =chronic disease outside the nervous system= were: coronary -and pulmonary arteriosclerosis, chronic fibrous endocarditis, mitral -sclerosis, aortic sclerosis with calcification, chronic splenitis, -chronic interstitial nephritis, hepatic atrophy (wt., 900 grams), -thickening of cartilaginous portion of right auricle (old trauma), scars -of apices of lungs. - -The =calvarium= was dense and the =dura mater= everywhere adherent. The -=arachnoidal villi= were but slightly developed, but there was one small -focus of cortical herniation through the dura mater of the left middle -cranial fossa. The =pia mater= was delicate except for slight opacities -along sulci. There was some pial thickening over the region of the -interparietal sulci on both sides. There was pial pigmentation -anteriorly and superiorly. - -There is no gross evidence of intracranial arteriosclerosis, except (1) -that afforded by the lesions of the dentate nuclei of the cerebellum -mentioned below and (2) the swerving to the right of the basilar artery, -possibly due not to arteriosclerotic lengthening of the artery but to an -unusual shape of the pons (see below). - -The =brain= weighed 1245 grams (cerebellum and pons 165 grams). =The -anatomical diagnoses of central nervous system= were: - -Slight general encephalomalacia (post mortem imbibition of fluid, 31 -hours). Slight gliosis of right prefrontal and frontal gyri. Slight -gliosis of right optic thalamus. Generalized granular ependymitis, -especially near fornix and about foramina of Monro. Anomaly of pons (not -gliotic, but possessing far more white matter on the left side than the -right). Severe arteriosclerosis confined to the dentate nuclei of the -cerebellum. - -As we now look over the data in Cases 40 and 41 we are inclined to ask -the question, whether modern systematic diagnosis would not have shown -these cases to be NEUROSYPHILITIC? One is inclined to answer this -question in the affirmative, on the basis that Case 40 showed somewhat -questionable Argyll-Robertson pupils, and Case 41 showed unilateral -Argyll-Robertson effect. Both cases showed Romberg sign, but the dentate -nucleus and other cerebellar disease in each case may in some way have -contributed to or imitated this phenomenon. Whether Case 40 was a -tabetic must remain a question, but Case 41 must be regarded as a case -with spinal and meningeal changes highly characteristic of syphilis. - - - =VASCULAR NEUROSYPHILIS plus TABETIC NEUROSYPHILIS (“tabes - dorsalis”) simulating paretic neurosyphilis (“general paresis”). - Autopsy.= - - -=Case 42.= The case of Elizabeth Brown was at one time carefully studied -by Dr. A. M. Barrett in his work on mental diseases associated with -cerebral arteriosclerosis and, like Case 43, was one in which tabes -dorsalis was a factor. Elizabeth Brown’s maternal grandfather and mother -were insane; there had also been insanity in a sister. Mrs. Brown was -struck on the head at 44, and was unconscious for an hour, but there -were no sequelae to this accident. At 48, there was a shock, or -apoplectiform attack, followed by unconsciousness for two hours and by -left hemiplegia, right ptosis, and thick speech. Mrs. Brown began to -walk again after two weeks, but was found to be forgetful and -fabulatory. She seemed at times to be hearing music, and somewhat -repeatedly became helpless and unable to walk. She could not remember -from day to day, showed incontinence of urine and feces, and was brought -to the Danvers Hospital. The physical and mental deterioration was -progressive. There were some signs of organic brain disease. The -musculature was especially flabby on the left side. The left angle of -the mouth drooped, and the left nasolabial fold was smoothed out. The -arm movements were ataxic, the tongue protruded to the left, the right -pupil reacted but slightly to light (eye blind from cataract), the -knee-jerks, Achilles, wrist, and elbow reflexes, were absent. The -patient was unable to stand, and there was a marked tremor of the hand, -tongue, and lips. There was a zone of anesthesia for pain and tactile -stimulation extending round the body, from the 3d to the 6th rib, and -there were symmetrical areas of anesthesia on the inner surface of the -forearms and the legs. - -The =autopsy= showed a =general arteriosclerosis= with =chronic= and -=acute meningitis=. The brain weighed 1110 grams; the =pia mater= was -moderately thickened; the basal vessels were highly arteriosclerotic. -The brain itself, however, normal externally, upon dissection, showed a -number of small cysts irregularly scattered in the white substance. The -basal ganglia were porous, and there were several small cysts in the -pons. =Microscopically=, there was evidence of severe vascular disease, -involving not only the arteries but also the veins. It was the -superficial rather than the deep arteries that were more often attacked. -There was a marked =perivascular gliosis=. Extensive search yielded _no -evidence of lymphocyte infiltrations_, either in the brain or in the -spinal cord. - -The spinal cord showed degenerations in both the lateral and posterior -columns, of which the explanation may possibly be like that in our -paradigm, Case 1. - -Is the case of Elizabeth Brown one of neurosyphilis? We cannot -definitely say on account of the non-availability of the modern -systematic tests, but it may well be that the case, although certainly -not one of paretic neurosyphilis, was one of TABES WITH VASCULAR -COMPLICATIONS. - - - =TABETIC NEUROSYPHILIS (“tabes dorsalis”) with symptoms of cerebral - origin producing a picture resembling taboparetic neurosyphilis - (“taboparesis”). Autopsy.= - - -=Case 43.= Robert Allen was the fifth case of error in the diagnosis of -general paresis analyzed some years since from the staff meeting records -of the Danvers Hospital. The Allen case resembles the case of Elizabeth -Brown in that there was a combination of tabetic phenomena with cerebral -lesions of a non-paretic character at autopsy. But although there seemed -to be an utter absence of inflammatory cells (lymphocytosis) in the case -of Elizabeth Brown (42), there were some slight perivascular cell -accumulations in the Allen case, with a few mononuclear cells suggestive -of lymphocytes. The cerebrum, however, failed to show plasmocytosis. It -was seriously diseased, showing a marked neuroglia proliferation about -the atrophic nerve cells. - -Robert Allen was a printer coming from a long-lived race. The following -are the main facts: - -Married in 1875 (two children, healthy); again married in 1893 (one -child, healthy). Compositor from 1890. In 1898 and 1899 girdle and -lancinating pains. Thereafter for several years gait was unsteady. -During 1904 and 1905 freedom from pains and improvement in gait but -gradually increasing irritability and nervousness. Stopped work on last -of March, 1905, owing to sudden increase of irritability, emotionality, -boastfulness, expansive schemes, and ataxia. - -Habits: no tobacco, very little alcohol at long intervals. No drug -habits, no sexual irregularity known. - -Committed to Danvers April 3, 1905, with slight muscular development, -poor nutrition, acne, irregular, poorly preserved teeth, gingivitis, -flat-foot, slight radial arteriosclerosis, slight arcus senilis, a few -hyaline casts, leucocytes, epithelial cells, and trace of albumin in the -urine, scar in sulcus, and enlarged inguinal lymph nodes. - -Ataxic gait, Romberg’s sign, fibrillary twitching of chest, abdominal -and facial muscles when standing; right pupil slightly larger than left, -pupillary margins irregular, light reactions (electric bulb test) both -consensual and direct absent, slight pupillary reaction in -accommodation; biceps, triceps and wrist reflexes lively and equal; -abdominal, cremasteric and plantar reflexes normal, knee-jerks, Achilles -and front taps negative even on reinforcement. - -The patient himself stated that his ataxia began in 1904, that he had -been under treatment for swelling of legs and feet and pain in limbs -since 1903, and that there had been some trouble with limbs since 1895. -He had been told that his disease was lead-poisoning. About three weeks -before commitment patient said he had had an attack of unconsciousness. - -The patient’s speech showed considerable defect. Words were pronounced -slowly with slurring and tripping especially of the labials. Orientation -perfect. School knowledge well retained. The easier arithmetical -problems were accurately performed. Memory imperfect for minor recent -events. Estimations of space and time often very imperfect. Variability -of mood, sometimes euphoric, sometimes tearful and irritable. Occasional -expansive estimates of personal powers (“Can lift three five-hundred -pound weights with one finger”). Indistinct expansive financial ideas. - -The patient continued oriented, euphoric, expansive, untidy, till -October, 1905, but on October 12 developed an infection at the site of a -callus on the sole of the foot and died with pyemic symptoms, October -17. - -=Post Mortem Findings.= The =cause of death= was streptococcus -septicemia with acute ulcerative colitis, acute splenitis, bilateral -purulent pleuritis, multiple infarctions of lungs. - -There were no signs of =chronic disease outside the nervous system= -except a moderate thickening of the mitral valves, and slight dural -adhesions. - -The brain weighed 1450 grams. The vessels at the base showed a slight -degree of sclerosis. There was a slight opacity of the frontal, -parietal, and temporal pia overlying slightly atrophied convolutions, -whose surfaces showed in a few places slight cuppings. The ependyma over -the thalami and the floor of the fourth ventricle was finely roughened. -The spinal cord showed a typical TABES DORSALIS. - -Although we probably cannot regard either Case 42 or Case 43 as a case -of paretic neurosyphilis, and although it must remain doubtful whether -they are cases of any form whatever of neurosyphilis (in the absence of -the modern tests), yet it seems clear that both these cases may very -well have been cases of neurosyphilis on account of the existence of a -definite tabetic process in each. The symptoms of these cases, like -those of Cases 38 to 41, suggest how difficult it must be _to make a -clinical diagnosis of general paresis safely without employing available -laboratory tests_. Yet how frequently in the past have neurologists -brought data concerning various phenomena in long series of so-called -paretics in which the error of diagnosis was certainly between 5 and 15% -and frequently still greater. The entire question of the symptomatology -of paretic and non-paretic neurosyphilis, therefore, needs re-opening -and revision. - - - =CEREBRAL GLIOSIS (probably non-syphilitic) producing the clinical - picture of paretic neurosyphilis (“general paresis”). Autopsy.= - - -=Case 44.= John Hughes was a hostler, and later assistant with a -wholesale drug company, with which he remained for 32 years. He had been -moderately but constantly alcoholic all his adult life up to 50 years of -age, and at 45 had had an attack of so-called nervous prostration, in -which his head had troubled him and he had been seclusive. At 49, he had -a serious attack of otitis media, associated with delirium, swelling of -the feet, and what was called rheumatism. After this attack of otitis -media, Hughes appears to have been not altogether right. - -At 53, after a quarrel with his employer, Hughes quit work, began to -trade a little in hens and pigs, became forgetful, especially of recent -events, and did “a variety of peculiar things.” He was a married man but -he had no children. There had been miscarriages but of unknown origin; -venereal disease was denied. At 55, a week before admission, Hughes had -a spell of unconsciousness for several hours, after which his speech was -thick, and restlessness, insomnia, and a wandering tendency set in. -Visual hallucinations, fabulation, tremors, “excited-looking” eyes, are -described. He would sweep things from the dining-room table, pulled a -hot stove into the middle of the floor, attempted to sweep paint off the -floor, and cut up a carpet with a knife. - -The patient on commitment November 5, 1904, was well developed and -nourished. The mucous membranes were rather pale. Bruises and -excoriations of limbs. Harsh breathing at the base of each lung. -Enlargement of heart; sounds irregular. Accentuation of aortic second -sound; tension fair, rate 80. Slight brachial arteriosclerosis. Abdomen -slightly distended. The urine contained a faint trace of albumin and -many hyaline casts. - -Moderate tremor of extended hands. Slight tongue tremor. Romberg’s sign -absent (slight swaying). Considerable ataxia of extremities (inability -to stand with foot on opposite knee). Vision poor. Hearing could not be -tested accurately. Prompt pupil reactions with direct light. Slight -consensual reaction in left pupil, absent in right. Deep reflexes equal -and lively. - -Quiet and orderly at first. Later restless and noisy. Questions were -answered at times relevantly, more often irrelevantly. Patient -irritable, intractable. Required repeated urging to take nourishment. -Consciousness clouded. Orientation imperfect. Attendants are possibly -“officers.” Date September, 1995. Slight errors in repeating alphabet. -Mistakes in Lord’s Prayer with rhyming tendency. Simple arithmetical -tests answered automatically with many mistakes. More complex -combinations incorrect. Handwriting tremulous (noted as “typical of -general paresis”). Auditory hallucinations (answering invisible -persons), “All right, I’m coming.” Amnesia and confabulation. Q. “Have -you had breakfast?” A. “No,” (later) “Yes, I had a very light -breakfast.” Q. “What did you have?” A. “Anything that came along. A few -green peas and beans that were left, bread and butter and pie. I had a -good breakfast. Guess feed is very high.” Q. “Give names of your sisters -and brothers.” A. “There are three or four I never see. I will have to -think them up.” (Later)—“Lillie, Abbie, Julia, George.” On repetition of -question, “Elizabeth, Julia, Annie and Lizzie.” - -Delusions somewhat doubtful. At no time euphoria. - -The patient remained only nine days in the hospital, developing diarrhea -a week after admission. - -=Post Mortem Findings.= The =cause of death= was bilateral -bronchopneumonia of hypostatic distribution, accompanied by bronchitis -and acute splenitis. The intestinal tract was normal (despite the -diarrhea). No cultures. The heart showed acute myocarditis. - -The vessels in general showed no sclerosis, except that the aorta showed -a few patches with calcification near bifurcation. There was a moderate -degree of mitral sclerosis. The kidneys showed a moderate degree of -chronic interstitial nephritis. The heart weighed 530 grams and there -was moderate dilatation of all the valves. - -There were some evidences of chronic disease outside the nervous system, -namely, an obliterative pleuritis on the right side, chronic -perisplenitis, and chronic external adhesive pachymeningitis. - -The =nervous system= showed a pia mater thin and transparent, with a -moderate congestion of larger and smaller vessels. No noteworthy change -of the brain substance or of the ventricles was found, except that the -cerebral substance was of unusual firmness (autopsy twelve hours after -death). - -It is clear that the brain was not wholly normal, exhibiting a general -induration due in part to subpial gliosis and in part doubtless to -perivascular gliosis. =Microscopically= the tissues showed features of -great interest, especially multiple focal neuroglia cell proliferations -of a perivascular distribution, considerable subpial fibrillar gliosis -of an unusually focal type, and a rather general subpial cellular -=gliosis=. Histologically, it seemed that this chronic progressive -process had started, not so much in relation with dying nerve cells, as -in relation with blood vessels. The =perivascular= deposits of neuroglia -cells were confined almost exclusively to the infragranular cortex -layers. It seems plain that the diagnosis of general paresis was not -justified. It is probable that the diagnosis of neurosyphilis is not -justified. The explanation may be that now and then cases of cerebral -sclerosis may clinically imitate the neurosyphilitic process. It must be -borne in mind that the diagnosis in this case was made, like the other -cases at head of Part III, without the advantage of modern systematic -methods. Clinically speaking, of course, there was no definite -Argyll-Robertson pupil, although the consensual reaction, slight on the -left side, was absent in the right pupil. The general picture appeared -to be one of the so-called demented form of paretic neurosyphilis. - - - =Differential diagnosis between NEUROSYPHILIS and NEURASTHENIA.= - - -=Case 45.= Albert Robinson, a man of 28 years, was shipwrecked on one of -the Great Lakes. The ship was on the rocks for eight days, and Robinson -was under a great strain. Ever after the wreck, Robinson had felt severe -pain in the head, neck, and back, and a feeling of great weakness -whenever he exerted himself physically or mentally, and seven months -after the wreck, he had several attacks of fainting. - -For a number of weeks he had worried a good deal about his inability to -make money, especially as money was badly needed on account of his -wife’s approaching confinement. A few days before entrance, Robinson had -become very forgetful, and was unable to recall, the night before -entrance, where he had been during the day. On the whole, however, on -mental examination no actual evidence of memory defect could be shown to -exist. - -=Physically=, Robinson was entirely negative, except for some hard -glands in each groin. =Mentally=, there was little to show except -depression, worry over his financial condition, and his inability to -work. The serum W. R. proved negative. - -=Diagnosis=: On the whole, the diagnosis of psychoneurosis (see case -Harrison (9)) due to the shock at the time of the shipwreck seemed to be -proper. To be sure, the patient gave a history of a chancre at 25, -treated for two years, after which he was declared cured. - -However, following up the clue of admitted syphilis, rigorous -questioning elicited the fact that a few months before there had been -diplopia, lasting part of a day. - -=Lumbar puncture= seemed desirable. The fluid was clear but contained -125 cells per cmm. with appropriately increased amounts of albumin and -globulin. The spinal fluid W. R. was positive. The diagnosis of -CEREBROSPINAL SYPHILIS seemed established. - -The lesson of this case appears to be that perhaps we should never -exclude syphilis until we have made an examination of the cerebrospinal -fluid. The W. R. of the blood in meningovascular (non-paretic syphilis) -is negative in many cases (the figure is sometimes set as high as 40%). - -=Treatment=: After a half dozen injections of salvarsan, all symptoms -disappeared and Robinson went back to work, claiming to be in a better -condition than for some time past. - - 1. How shall we explain such a symptom as the transient diplopia? - This diplopia is probably an example of a neurorecidive, but it - will be observed that it occurred without salvarsan therapy. See - discussion above under the case of Bennett (34), where the general - result of the neurorecidive inquiry launched by Ehrlich early in - the history of salvarsan therapy showed that precisely similar - phenomena had always occurred in neurosyphilis, whether under - treatment or not. The anatomical and histopathological explanation - of such phenomena is, of course, doubtful, but a review of the - findings in the case of Alice Morton (1) will show how many - apparently serious symptoms in neurosyphilitics are actually - irritative or at least due to lesions which are entirely - recoverable. We may suppose, first, a local proliferation of - spirochetes; second, a local over-formation of toxic substances, - directly or indirectly the product of spirochetosis; thirdly, a - local exudation; fourthly, a local proliferation; fifthly, a - combination of these phenomena, any or all of which may be - regarded as but transient. We have sometimes found at autopsy very - little exudate except in small areas; sometimes not more than a - few mm. or cm. in superficial extent. Note, for example, the small - areas of lymphocytosis demonstrable in but two foci in the case of - Alice Morton, the paradigm placed at the beginning of this book. - - - =NEUROSYPHILIS(?) in the SECONDARY STAGE of syphilis. HYSTERICAL - symptoms. Diagnosis?= - - -=Case 46.= Alice Caperson was a colored girl of 18 years. She had -acquired syphilis five months before admission to the hospital, and the -secondary symptoms of this syphilis had just disappeared before -admission. - -Very shortly after acquiring syphilis, the young negress began to act -peculiarly. She describes herself as having a sort of nightmare, both -when asleep and also when awake. For instance, she saw her dead -grandmother. It appeared at first like a seraph; then it came nearer to -her and seemed to fill out; and then was dressed precisely as her -grandmother had been. This seraph appeared as though trying to tell her -something, but she could not make out what the something was. The vision -had appeared on two or three occasions. - -Our examination detected little beyond instability and irritability of -mood with some depression. The patient readily fell to weeping. She soon -made friends in the wards, however, and got on well. =Physical -examination= was entirely negative but the W. R. of the blood serum was -positive. The W. R. of the spinal fluid was negative, as was the gold -sol reaction; there was an excess of albumin and a positive globulin -test; there were seven cells per cmm. - -The psychiatric diagnosis of a case like that of Alice Caperson would -waver between hysteria and dementia praecox. However, as for dementia -praecox there are hardly any typical symptoms. There is insight into the -hallucinations, which are hypnagogic. There are, however, no hysterical -stigmata. - -The spinal fluid reaction is typical of the secondary stage of syphilis. -It is commonly said that in every case of syphilis the nervous system is -involved at some period, if only to the degree shown in the present -case. However, such involvement tends to disappear both with and without -antisyphilitic treatment, just as do the secondary skin symptoms. So far -as syphilis is concerned, the prognosis under radical treatment is as -good as usual. We are inclined to regard the case as one of the -HYSTERICAL or PSYCHOPATHIC group and inasmuch as cases occurring in the -developmental stage of a patient’s life are of fairly good general -prognosis, we are inclined to regard the prognosis in this particular -case as good under proper therapy and hygiene. - - 1. What is the relation of neuroses to syphilis? Neurasthenia, - chorea, hysteria, and epilepsy are often grouped (for example, by - Nonne) as neuroses bearing at times important relations to - neurosyphilis. (For the relations of neurasthenia, chorea, and - epilepsy, see cases of Greeley Harrison (9), Margaret Green (72), - and David Borofski (49), respectively.) As for the hysteria shown - in Caperson, Charcot enumerated syphilis among _agents - provocateurs_ of hysteria along with alcohol, lead, arsenic, and - the like. Fournier has also considered the problem. It is clearly - necessary to show that before infection there were no hysterical - symptoms, and that the hysteria developed during the operation of - the syphilitic process, and it is probably necessary to show that - the symptoms will clear up under antisyphilitic treatment, if we - are to concede the existence of a syphilitic hysteria. - - 2. What are the evidences of neurosyphilis in the secondary and - primary stages of syphilis? As above stated, the findings in - Caperson are typical enough. Wile and Stokes at first stated that - 60 to 70% of the secondary syphilitics show changes in the spinal - fluid; in a further article they maintain that probably every case - shows such changes and that clinical symptoms of neurosyphilis of - the secondary period can probably be determined. They claim that - it is probable also that the same holds for primary syphilis - itself. The importance of these claims lodges partly in the - relation of these early signs of neurosyphilis to the whole - question of latency and to the question of _paresis sine paresi_. - For a discussion of _paresis sine paresi_ see cases Lawlor (25), - Vogel (52). - - - =Differential diagnosis between NEUROSYPHILIS and MANIC-DEPRESSIVE - PSYCHOSIS.[9]= - - -=Case 47.= As in other instances (compare Martha Bartlett (21) and Annie -Monks (85)) so also in the case of Ethel Hunter, a woman 61 years of -age, there was no initial suspicion of neurosyphilis. Mrs. Hunter was -brought to the hospital stuporous as a result of an overdose of -paraldehyd. The paraldehyd had been administered by a physician to -combat insomnia and agitation. As soon as Mrs. H. had recovered from the -drug stupor, this agitation appeared once more, and it was clear that -she was suffering from marked depression. There was tremendous worry -over the sickness of a woman with whom the patient lived. The patient -was very self-accusatory, blaming herself for many things that had -happened in the household. Besides her agitation, depression, -self-accusations, and insomnia, the patient showed a good deal of the -symptom frequently termed “retardation”—a kind of lagging of all mental -processes found, according to Kraepelin, in manic-depressive psychosis. - -Accordingly, the diagnosis of manic-depressive psychosis might well have -been rendered. The fact that the psychosis so far as known began in the -involution period was not against the diagnosis since the so-called -involution-melancholia of this period is at least in a certain fraction -of cases nothing more or less than a form of manic-depressive psychosis. -However, the =physical examination= made the diagnosis of -manic-depressive psychosis a little doubtful. There was a superficial -thickening of the arteries (blood pressure: systolic, 170; diastolic, -104), which thickening would not in itself be against the diagnosis of -manic-depressive psychosis. (In point of fact, arteriosclerosis is -rather common late in this disease and previous attacks could not be -excluded on the basis of available history.) The contracted pupils were -irregular and both reacted sluggishly to light, although better to -accommodation; the right pupil was larger than the left. The arm -reflexes were pretty active. The left knee-jerk could not be obtained, -nor was the right knee-jerk more than very sluggish. The Achilles -reflexes could not be obtained. Although there was not a positive -Romberg sign, there was a considerable swaying in Romberg position. -There was no speech defect. The other reflexes showed nothing abnormal. -On the whole, we had to conclude that, although Mrs. Hunter might be an -instance of manic-depressive psychosis, still there was much of -neurological interest in the case. - -This conclusion was emphasized when the W. R. of the blood serum was -found to be positive. The spinal fluid W. R. was also positive, and the -gold sol index was of the “paretic” type. There were 74 cells to the -cmm. Globulin stood at ++++, and albumin at ++++. - -This case, therefore, again illustrates, as well the protean nature of -GENERAL PARESIS (the diagnosis rendered), as the doubtful value of -making a psychiatric diagnosis without due consideration of the physical -examination and laboratory findings. How easy might it have been, at -least some years ago, to consider that this patient of 61 years had -suffered a slight shock at some previous time (left knee-jerk absent), -but was as a matter of fact a case of manic-depressive psychosis with a -vascular complication! - -Note: We must again duly insist that the merely sluggish light reactions -of the pupils in such a case as this do not especially point to general -paresis. The literature seems to establish that sluggishness of light -reaction precedes the classical Argyll-Robertson pupil. Yet it does not -do to say that, if the Argyll-Robertson pupil pretty conclusively points -to neurosyphilis (for exceptions see cases Falvey (55), Murphy (60)), -then a sluggish pupillary reaction to light looks in the same direction. -Sluggishness may precede stiffness in many, or perhaps all, cases, but -sluggishness of pupils is a frequent phenomenon outside the syphilitic -group of cases. - - 1. What part is played by emotional shock and psychic causes in the - starting up of general paresis? The answer to this question cannot - be definite. That a paretic process can be started up after trauma - is admitted on all sides; but we here suppose actual physical or - chemical brain disturbance permitting increased spirochetosis or - inflammatory reaction. In the case of psychic shock, or what might - be called _psychogenic general paresis_, our best resort will be - to the indirect effects of hormone action, or of vasomotor and - other autonomic disturbances produced directly or indirectly by - emotion. We are clearly here dealing with material too speculative - to be of practical service at this time. - - 2. Was the depressive drug therapy in the case of Hunter - justifiable? The paraldehyd had been administered by a physician - apparently on purely symptomatic grounds to combat the insomnia - and agitation of this woman of 61 years. With all due - acknowledgment of the difficulties of private practice, we must - insist that when ordinary measures in the relief of insomnia and - agitation are insufficient to curb these conditions, then a - positive danger ensues with the larger doses. As a rule, with - these larger doses and with the withdrawal of sensory stimulation, - the patients relapse into a stupor of grave moment. We need only - recall the situation in delirium tremens where adequately - depressive drugs often tend to kill the patient. - - - =Case for diagnosis. Errors in the diagnosis of NEUROSYPHILIS are - possible even when abundant clinical and laboratory data are - available.= - - -=Case 48.= The first error chosen for demonstration is that in the case -of the machinist, Milton Safsky. - -Safsky, about 8 months before his entrance to the hospital in the 42d -year of his life, had begun to lose strength, to grow thin and pale, and -to suffer from an extreme and continuous thirst. He was said to have -drunk as much as 6½ gal. in a day, and passed appropriately large -quantities of urine. After a time, his management at a general hospital -became difficult, as Safsky became confused, cried “hysterically,” and -was at times very noisy. He sustained a marked memory loss, seemed to -show visual hallucinations, and complained of headache, both frontal and -occipital, and of pain about the eyes. Sometimes the patient was very -euphoric and expressed what seemed to be delusions of grandeur, saying -he was wealthy and owned many machine shops. - -Some symptoms, e.g., polydipsia and polyuria amounting to a diabetes -insipidus, associated with headache and arrested attention, suggested -possibly a new growth in the pituitary region. The mental symptoms might -naturally be supposed to be due to some infiltration or pressure effect -of intracranial growth. After admission to the Psychopathic Hospital, -the patient was found difficult to arouse, although he could eventually -be aroused. His orientation proved to be as poor as his memory. From -time to time, the patient became a bit more intelligent and able to -execute requests. - -The =physical examination= was in general almost entirely negative. -=Neurologically=, the pupils were markedly contracted and reacted slowly -to light, though they were otherwise normal. The deep reflexes were all -somewhat lively, though equal. The umbilical and cremasteric reflexes in -particular were present. Systematic examination revealed no other reflex -disorder, nor any disturbance of sensation. There was a coarse tremor of -the extended hands. There were no phenomena of importance in the visual -fields. - -As against the diagnosis of growth, pituitary or extrapituitary -(diabetes insipidus and headache), a hypothesis of neurosyphilis had to -be considered. Not only were the contracted, slowly-reacting pupils and -the active deep reflexes suggestive, but the euphoria with grandiose -ideas looked entirely consistent. As for the polyuria, one had to think -of the so-called syphilitic polyuria of the textbooks, which is regarded -as a more or less characteristic result of syphilitic involvement of the -_basis cerebri_. Moreover, the W. R. in the spinal fluid proved to be -slightly positive; 146 cells per cmm. were found therein; there was a -large quantity of globulin, and a very marked increase in albumin. These -observations seemed to be exceedingly suggestive of a cerebral syphilis. - -However, as the case progressed, the diagnostic situation changed. The -W. R. upon a second puncture fluid proved negative. After some weeks, -characteristic symptoms of intracranial pressure developed; the -diagnosis of BRAIN TUMOR had to be taken as established, and there is no -doubt of its correctness. - - 1. What is the explanation of the weakly positive W. R. in Safsky’s - spinal fluid? An explanation is not easy to find. Possibly we may - regard the reaction as an example of error in technique. It is - even possible that it may have been produced by exudative products - in the spinal fluid. - - 2. What precautions may be taken against an error in diagnosis such - as was first made through the positive spinal fluid Wassermann in - the case of Safsky? First, repetition of the W. R.; secondly, it - is very unusual to find a weakly positive W. R. in a case with - such marked excess of albumin and such very marked increase of - globulin as was shown by this case. - - 3. How can we explain the inflammatory products in the puncture - fluid? Superficial brain tumors are frequently associated with a - so-called _meningitis sympathica_. The products of such meningitis - are exhibited: _viz._, globulin, albumin, and pleocytosis, exactly - as shown in Safsky. - - - =Can PARETIC NEUROSYPHILIS (“general paresis”) appear clinically - EARLY (e.g., two years) after the initial syphilitic infection?= - - -=Case 49.= David Borofski, a street car conductor, 27 years of age, -suddenly had a convulsion while at work in his car. For four months -Borofski continued to have rather numerous convulsions, was finally -compelled to discontinue work, and resorted to the Psychopathic -Hospital. It appears from his own story that, about two years before, he -had had a chancre, for which he had been treated at a general hospital -syphilis clinic, and of which he was told he was cured. With a -progressive loss of memory and with convulsions, Borofski became much -concerned about himself, and was finally persuaded by his fellow-workers -to come to the Psychopathic Hospital. - -The convulsions were described as follows: The patient gives a short -cry, has convulsive movements for about ten minutes, remains unconscious -for perhaps half an hour, and wakes with headache, dizziness, and a -feverish appearance. Sometimes the attacks were more severe, with -frothing at the mouth, biting of lips, and loss of sphincter control. -There were also slight attacks, occurring almost every day, without loss -of consciousness; these latter attacks consisted of dizziness, inability -to speak for a few seconds, and some arm twitching. - -=Physically=, Borofski was well developed and nourished, with a blood -pressure of 160. The only abnormal phenomena =neurologically= were -absent knee-jerks and ankle-jerks, sluggish pupillary reactions, and -slight tremor of the hands. - -=Mentally=, despite suggestive complaint of amnesia, the memory was -found to be fairly good but knowledge of current events and school -knowledge was poor. The simplest problems in arithmetic Borofski gave -up. - -The first diagnosis in such a case would naturally be epilepsy. However, -when an epileptic or epileptiform attack occurs for the first time in -adult life, the chances are probably against an idiopathic epilepsy. -(This is not a universal rule but will serve.) Borofski himself, -moreover, gave a history of syphilis. And the very nature of the -attacks, with arm twitching and without loss of consciousness, would not -readily fit into the frame of the idiopathic group. The absence of -certain reflexes and the sluggish pupils are naturally also suggestive -of syphilis, although not convincing. - -The W. R. of the serum proved positive, as did that of the spinal fluid. -The gold sol reaction was characteristically “paretic”; there was an -excess of albumin and a positive globulin, and there were 15 cells per -cmm. There could be little or no doubt of the diagnosis of some form of -neurosyphilis. The laboratory picture was consistent either with general -paresis or with cerebrospinal syphilis. So far as we are aware in the -present stage of knowledge, the two conditions can hardly be -differentiated unless we choose to rely on therapeutics. However, it is -exceedingly rare for general paresis to occur only two years after the -original infection. If we can trust this statistical fact, we shall -perhaps be wiser to term the case of Borofski one of DIFFUSE -CEREBROSPINAL SYPHILIS, and not one of paresis. - -=Treatment=: Borofski was put on antisyphilitic treatment consisting of -0.6 gram of salvarsan twice a week and potassium iodid, together with -intramuscular injections of mercury salicylate. The convulsions then -ceased. After four months Borofski returned to work, and he has remained -at work for a year. He has never regained his former health. - -Fifteen months after beginning of treatment the laboratory tests were -again made (there had been more than 60 injections of salvarsan), and -the cell count and gold sol reactions were found to be negative. -Globulin and albumin were also in smaller amounts than in the original -examination. However, the W. R. of the serum and the spinal fluid -remained positive. - -Head and Fearnsides state that cases of cerebrospinal syphilis should -return negative spinal fluid tests after six months of treatment. Upon -this criterion of Head and Fearnsides, Borofski would not be a case of -cerebrospinal syphilis; but it is probably impossible to separate -various forms of neurosyphilis into categories on any such grounds. - - 1. Shall case David Borofski be regarded as one of paretic - neurosyphilis (“general paresis”)? He has returned to work and has - remained at work, though without regaining his former health. In - any event, however, he does not offer the typical picture of - inevitable decline and death presented by the typical case of - Pietro Martiro (15) presented in our discussion of systematic - diagnosis. However, we could not upon laboratory grounds, or even - upon the ground of clinical observation, distinguish Borofski from - Martiro; Borofski has greatly improved; Martiro is dead. Borofski - developed his obvious neurosyphilis only two years after the - original infection. The conservative syphilographer might, - accordingly, reply that David Borofski is not a typical case of - paretic neurosyphilis (“general paresis”) either in the length of - the incubation period for his neurosyphilitic symptoms, or in his - outcome. - - 2. What is the cause of such convulsions as those developed by David - Borofski? Evidence from clear cases of general paresis with - convulsions leads to the hypothesis that such convulsions as those - developed by Borofski are not necessarily based upon frank - destructive lesions such as would be produced by the plugging of - terminal arteries. They may well be produced through the - activities of minor lesions, only demonstrable by microscopic - methods, either through properly disposed cell losses or by the - pressure of exudate, or even by endotoxins or other substances - derived from the bodies of dead or living spirochetes. - - 3. Aside from the well-known syphilitic epilepsy due to meningitis, - is there a non-meningitic epilepsy (such a disease as Fournier - formerly described under the term parasyphilitic epilepsy)? We - dismiss from discussion the so-called symptomatic epilepsies which - are the result of a gross organic disease of the brain substance - or its membranes, and which do not differ so far as we are aware - from organic epilepsy produced by other gross lesions of an - identical size and structure. These symptomatic epilepsies may be - partial, or even may present the appearance of generalized - epilepsy. We may also leave out of account those epileptic - pictures which are produced in general paresis itself, and which - may be viewed as nothing but partial phenomena of general paresis. - The kind of so-called “parasyphilitic” epilepsy that Fournier - described is a kind of epilepsy that cannot be distinguished from - genuine epilepsy, in which the sole disease-phenomenon throughout - a long period of time consists of epileptic convulsions. It - appears that these “parasyphilitic” imitations of genuine epilepsy - occur in individuals with a very long post-infective “incubation - period,” but that there are some cases in which the epilepsy - appears, on the contrary, in the very earliest stages of syphilis. - The attacks are a little less common than those of idiopathic - epilepsy; they have the same apparently causeless beginning; are - associated with complete amnesia; and are followed by - characteristic dazed states. The patient’s intelligence, however, - suffers little. Now and then a case reacts well to antisyphilitic - treatment energetically pushed. (Spontaneous long remissions in - non-syphilitic epilepsy must be remembered.) Petit mal attacks - occur sometimes between the more severe attacks. In short, it - would appear that there is a group of syphilitic epilepsies in - which the brain shows no gross structural lesions, which - accordingly do not exhibit any Jacksonian appearances, and which - last a comparatively long time without changing their character, - and often without being especially altered for the better by any - form of antisyphilitic treatment. This condition is sometimes - known as a post-syphilitic epileptic neurosis. Nonne had been able - to collect up to 1902 some 12 cases from his own service. - - 4. Would it be proper to call Borofski a case of taboparesis? Absent - knee-jerks in a victim of paretic neurosyphilis should not be used - to suggest a diagnosis of taboparesis. This question of - terminology has been discussed above, under Sullivan (16). - - 5. What is the mechanism by which the amnesia of a case like - Borofski is produced? The answer runs in the same terms as the - answer to the questions concerning the cause of convulsions. The - amnesia in general paresis has surprising functionality. A study - of autopsied cases of general paresis has shown that amnesia is - practically as common in cases without marked destruction of brain - tissue as in cases with atrophy of classical extent and depth. The - clinical recovery in this case was practically complete in respect - to memory. We must regard the amnesia as not due to the - destruction of storage cells bearing the so-called neurograms - (Morton Prince). - - 6. What is the explanation of the persistently positive W. R.’s of - the serum and spinal fluid associated with diminished globulin and - albumin tests, a negative gold sol reaction, and normal cell - count? See discussion under Case Martha Bartlett (21). - - 7. How atypical is the early development of paretic symptoms in - David Borofski? C. B. Craig has collected, in 100 cases of brain - syphilis (a list including both paretic and non-paretic cases), - some data on this point. The shortest period reported by Craig was - in a case in which the neurosyphilitic symptoms appeared one month - after infection. Craig found three cases where symptoms appeared - in six months, and six cases within a year. The longest - post-infective period of Craig’s list was thirty years. Our case - of Chatterton (73) developed symptoms 33 years after infection and - Washington (66), forty years after infection. Nonne casts some - doubt on statements to the effect that tabetic symptoms may occur - three to four months after infection. It seems to be admitted that - pupillary anomalies and reflex changes may occur in the early - secondaries and may recover under antisyphilitic treatment. - Nonne’s case of longest post-infective interval, like that of - Craig, was one of 30 years. - - Myerson has reported a 20–year old patient who acquired chancre - April 1, 1911 (spirochetes demonstrated); salvarsan was - administered April 20th. There were no secondary symptoms, but in - May, headache, visual disturbance, vertigo, and other symptoms - developed (neurorecidive). Upon June 20th, that is, 11 weeks after - development of the chancre, aphasia and astasia developed, with - numbness of the left side. At this time, the pupils were slightly - irregular and unequal but reacted normally. The signs in the fluid - were positive. Upon this question see our cases of Bright (121) - and Bennett (34). - - - =Hemitremor following hemiplegia in PARETIC NEUROSYPHILIS (“general - paresis”). Autopsy.= - - -=Case 50.= Achilles Akropovlos, 39 years, had symptoms six months before -commitment to Danvers Hospital. There were attacks of confusion, -difficulty in walking, and speech defect, resulting in an entire -incapacity to work and eventual commitment. Rather unusual and striking -was a very marked tremor, apparently limited to the right side of the -body. =Physically=, Akropovlos was normal, but =neurologically= he -showed, in addition to the marked right-sided tremor, a marked speech -defect, and a degree of ataxia. The tendon reflexes were very active, -but there were no abnormal reflexes, and the pupils reacted normally. -According to the history, the difficulty had followed a slight attack of -apoplexy. =Mentally=, there was a marked confusion. The blood serum and -the spinal fluid were both positive to the W. R.; globulin was present, -and albumin was increased; there were 43 cells per cmm. There was hardly -any diagnosis to make except general paresis. - -Death followed 18 months later, or two years after onset of symptoms. -Increasing weakness, emaciation, and dementia preceded death. Autopsy -confirmed the diagnosis of PARETIC NEUROSYPHILIS. - - 1. What is the usual cause of death in general paresis? Intercurrent - disease very frequently occurs in general paresis, and such - intercurrent disease is then given as the cause of death. As a - matter of fact, however, one feels that in many of these cases the - intercurrent pneumonia or infection—frequently of the - bladder,—bedsores, sepsis, and the like, are merely accidental - incidents in a condition that is leading to death, and which has - caused a lowered resistance to infection. In certain instances - where nursing is exceptionally good and where no such infection - occurs, the patient continues to grow weaker and weaker, paralyses - of all the muscles follow and finally paralysis of deglutition or - respiration may lead to death. The emaciation and paralyses may be - of such a grade that the patient is entirely devoid of fat and - unable to move at all. Not infrequently vascular crises occur, and - one of these may be responsible for death. - - 2. What was the cause of the hemitremor? The hemitremor suggested an - irritative or destructive lesion in the motor path. Delving into - the history it was learned that the patient had had a shock - followed by a right hemiparesis. This had cleared up leaving the - tremor as a residuum. The autopsy disclosed a reddish-brown - pigmentation and fibrous thickening of the pia over the left motor - area, confirming the idea of a previous hemorrhage. As a rule the - shock phenomena occurring in paresis clear up more completely and - no gross lesion is visible post mortem. However, cerebral - hemorrhage must be expected in any person suffering from syphilis, - and is no rarity in paretic neurosyphilis. - - - =PARETIC NEUROSYPHILIS (“general paresis”) with NORMALLY REACTING - PUPILS. History of trauma. Autopsy.= - - -=Case 51.= Daniel Wheelwright, a barber of English extraction, 57 years -of age, had had a sunstroke at 15. At 42, there had been pneumonia, -after which an attack of rheumatism was said to have kept the patient -from work for a year. There was trauma of head (falling wrench) at 44. -This blow on the head was the assigned cause of the mental disease, -symptoms of which, however, did not develop until about the first of -September, 1905, about three months before entrance, January 9, 1906, -and about six months before death, March 20, 1906. - -It seems that the patient had begun to change in manner; he had become -despondent and apathetic, silent, and somnolent. Two weeks later, he -stopped working, began to read the papers once more, and became somewhat -more cheerful. - -About Thanksgiving, Wheelwright got up at midnight, and remained up, -lighting all the fires and talking continuously. During the next two -weeks, he talked much to himself, laughing out at times. About two weeks -before Christmas he went out and started to make a sidewalk of old -boards, working in his shirtsleeves, without a hat. He would work until -midnight making screens for windows. During the day, he would go out and -give money to passing children; would offer to pay the grocer twice as -much as articles were worth. - -On the day before Christmas, he put out all the fires and lights in the -house, sent all the family to bed, and opened all the doors. Christmas -morning, he rose early and got the washtubs ready. He helped his -compliant wife to do the washing, then put out all the fires and opened -the windows. After Christmas, he began to tell how rich he was going to -be through starting a garden and by making butter. He bought six or -seven quarts of milk daily, and procured carrots and oranges, grinding -them up to color the milk. January 9th he was committed to Danvers -Hospital. - -=Physically=, there were few symptoms. =Neurologically=, there was a -tremor of tongue, fingers, and face. The knee-jerks were lively. The -pupils reacted normally; the patient was restless, pacing up and down. -There was a speech defect demonstrable with test phrases. Orientation -was imperfect for time and for place. Handwriting was poor, memory -impairment was marked, but the patient was given to fabrication as to -past events. A characteristic sample of statements: - -“Do you know that this is an insane hospital?” “Yes; there are two or -three men here out of their heads. I could cure them with my hands but -they won’t let me. I could get all the sick men on their feet just by -rubbing them. I can do anything with my hands. I can build a house by -just sitting down and thinking about it. I can whip all the men in this -place. I have better sense now than I ever had in my life.” - -Again, “How long have you been here?” “Over three months; they have put -me in heaven three times since I have been here. They killed me, crushed -my heart, and turned my blood to water. I am all right now. I let the -sun shine on my heart and it brought it together. I can whip every man -in here as fast as they come up.” - -Again, “I will make a million dollars on my garden when I get it. I can -make a million dollars on half an acre. I can do anything. I can move -this house by just thinking of it.” - -During a special examination, the patient told how he had fastened wings -on his hands and feet, and how he had gone to heaven; he told how he had -soared high above the earth, and how differently the stars look when up -near heaven than they do from the earth. He spoke of seeing angels and -of the beauties of heaven. - -The diagnosis of PARETIC NEUROSYPHILIS was confirmed at autopsy. - - 1. What is the significance of the normally reacting pupils? While - it is usual to find pupillary anomalies in neurosyphilis, these - changes are not an essential part and it is not rare to find - normal pupils in all forms of neurosyphilis. It is less frequent - to find a normal pupil in tabetic than in diffuse or paretic - neurosyphilis. In paretic neurosyphilis it is the rule to find - pupillary changes during some stage of the disease, but not - necessarily early. At times the pupillary sign may be one of the - earliest signs of neurosyphilis—again it may occur only as a late - symptom, if at all. One of the most important of the pupillary - signs is irregularity of contour. While this does not always mean - neurosyphilis it is highly suggestive and certainly indicates - careful examination even though the W. R. in the blood be - negative. - - 2. What was the relation of trauma to the development of the - neurosyphilitic symptoms? It is, of course, the rule in all forms - of mental disease to have some factor offered by the patient or - relatives as the cause of the psychosis. Often these assigned - causes are minor events thought of only after the later appearance - of symptoms. In this case it was not thought that the trauma had - any causal effect. For a discussion of trauma and neurosyphilis - see cases Joseph O’Hearn (90), Levi Sussman (91), and Joseph - Larkin (92). - - - =NEUROSYPHILIS, probably PARETIC, with symptoms highly suggestive of - MANIC-DEPRESSIVE PSYCHOSIS.= - - -=Case 52.= Bessie Vogel[10] was admitted to the Psychopathic hospital -New Year’s day, 1915, in a very much excited condition. The family -history is very meagre, and all that is of significance is that mother -has always been very “nervous.” The records in part: - -=Past History.= Very healthy as a child, and except for occasional -throat trouble and headache had no physical ailments until eight years -ago, when she had an operation for appendicitis, and two and one-half -years ago was operated upon for hernia and adhesions. Following this she -began to show a lack of energy, neglected her housework, was much -depressed, wept frequently, complained constantly of pain in various -places, and was ill-tempered. In about five months she improved, and -then after a couple of weeks at the shore seemed entirely well. - -=Present Illness.= In November, 1914, that is, about seventeen months -after the recovery from the previous depression, she again began to show -practically the same symptoms. She was depressed, could not sleep, and -would get up in the night and sew; was self-centered and hypersensitive, -then became restless and nervous; wanted to go shopping and out for -dinner; went to New York and then to New Bedford. Symptoms became more -marked; she became very ill-tempered, threatened her husband when angry -over trifles, threatened suicide, then began to get active and spent -money extravagantly. At the end of two months, that is, Jan. 1, 1915, -she was admitted to the hospital. - -=Physical Examination.= A small, thin woman, appearing to be about 45 -years old (actual age 37). Aside from the absence of teeth and the -operation scars, the general examination is negative. =Neuromuscular -system=: The pupils are round, regular, equal, and react to light and -accommodation, but do not hold very well. Extraocular movements well -performed, no palsies of facial muscles, tongue protruded medially -without tremor. Uvula is raised symmetrically. Biceps and triceps and -supinator reflexes are present and brisk. Patellar and Achilles reflexes -are equal on the two sides and brisk. Abdominal skin reflexes not -obtained. Plantar reflex active and flexor in type. No Babinski, Gordon, -or Oppenheim. No tremors. - -Wassermann reaction serum positive. Examination of spinal fluid: clear, -globulin ++++, albumin ++++; cells, 130 per cmm.; small lymphocytes, -79.9%; large lymphocytes, 14.1%; polymorphonuclear leucocytes, 4.6%; -plasma cells, 0.7%; endothelial cell, 0.7%. W. R. positive. Gold sol -reaction, 55555522 +-. - -=Mental Examination.= On admission patient showed great psychomotor -activity, was very playful, marked flight of ideas, was expansive, very -emotional, very erotic. She slept very little, appetite was poor, and -she lost weight rapidly. Orientation and memory intact. No -hallucinations elicited. In about three weeks improvement began, and at -the end of eight weeks she appeared practically recovered. On April 9, -1915,—that is, 13 weeks after admission,—she was allowed home on visit. -On leaving, she appeared normal in every way. There was no evidence of -psychotic symptoms, she had good insight, and physically there was -absolutely nothing of a neurological nature that was abnormal. - -This case, with the history of a previous depression and its clinical -picture during the acute stage, and its recovery, is certainly in every -respect typical of manic-depressive insanity, and only the positive -result of the six tests causes us to put it in the group of GENERAL -PARESIS. Only the further course will shed any light as to the correct -significance of these findings, and even then we shall not be too sure -that we had not been dealing with a manic-depressive psychosis in a -latent neurosyphilitic. We would strongly emphasize the point that at -the present time this patient presents no mental or physical signs of -cerebrospinal syphilis or general paresis; but the six tests are still -positive. This case differs from the ordinary general paresis remission -in that there is not a single physical sign of paresis present. - -There are many transitional cases between this case which shows no -symptoms or signs of neurosyphilis except the laboratory tests, and the -typical case of general paresis. Thus we have cases with slight -character change and no physical signs except rare “seizures.” On the -other hand, in many cases the presence of abnormal neurological -phenomena without definite mental signs is first noted. Certain remitted -cases show only some slight pupillary or reflex abnormality. We believe -we have here added the last link in the chain between the primary and -quaternary symptoms. - -This case is illustrative of several which we have published elsewhere -under the name of _paresis sine paresi_ or latent neurosyphilis to -illustrate how all the laboratory signs of neurosyphilis may be present -in a patient without any physical or mental symptoms that may be -correlated with these findings. - -We summarize our discussion of this as follows: - -1. There is a group of cases showing the laboratory signs characteristic -of central nervous system syphilis: (_a_) positive W. R. in the serum, -(_b_) positive W. R. in the spinal fluid, (_c_) pleocytosis, (_d_) -excess of albumin, and (_e_) of globulin in the spinal fluid, (_f_) gold -sol reaction of central nervous system syphilis, and which show no sign -or symptom of neurosyphilis. - -2. We believe these cases represent a form of chronic cerebrospinal -syphilis, probably paretic in type. - -3. They have the greatest theoretical and practical significance in the -consideration of the life history of neural syphilis, in the concept of -_Allergie_, in regard to results of treatment, and finally as to the -evaluation of the laboratory tests. - -4. Here is perhaps offered the last link to form a complete chain -between the symptoms of the primary stage of syphilis and its final -termination of life as the result of the diseases cerebrospinal syphilis -or general paresis. - - - =SYPHILIS (?); EXOPHTHALMIC GOITRE; neurosyphilitic old lesion of - optic thalamus; unilateral induration and atrophy of left cerebral - cortex. Autopsy.= - - -=Case 53.= Carrie Pearson, a housewife 25 years of age, died at Danvers -Hospital less than a week after admission, and it was at first stated -that her symptoms had lasted but two weeks before admission. In point of -fact, a further investigation showed an important succession of -symptoms, lasting some four years. - -Carrie had been considered a healthy child, going to school at the usual -age, and progressing well with her studies. She however, left school in -the ninth grammar grade, at the age of 15, and went to work in a -milltown. She married a worthless person at the age of 18, and lived -with her husband for three years. There was one child born a year after -marriage. Two years later, however, a tremendous goitre had developed -such that her neck was described as “out square with the face,” and at -the same time the patient’s eyes had become prominent. - -About two weeks before admission, she had gone to a neighboring town to -take care of a sick woman, but during her endeavor to be a nurse, she -had broken out into a mania, tearing up furniture and bedding, and -talking irrelevantly for a period of four days. She also showed insomnia -and continually tore off her clothing from her body. - -Upon =examination=, the marked enlargement of the thyroid gland together -with the prominent eyeballs, husky voice, and pulse rate of 150 per -minute, were entirely consistent with the diagnosis of exophthalmic -goitre. The patient described herself as “Carrie Nation.” Asked to write -her name, she took the pen and tried to spatter ink, wrote hurriedly and -carelessly her maiden name and several words without apparent meaning. -Asked to write, “God save the Commonwealth of Massachusetts,” she wrote: -“God save the common pal U S Spe Manor Gen, or til pat. Since Lord, or -no prime in Hear to the God Tel. Ho. n and or Mabel, or gal.” After -this, she took paper and wrote meaningless scrawls, saying that it was -Japanese writing. There was much motor restlessness with -distractibility, pointing and grimacing, mimicking the actions of those -about her. - -Death occurred from exhaustion, and the case might not have been -regarded as unusual except for the autopsy, which showed a peculiar -brain lesion, described below. The point of greatest interest in the -case was the fact that syphilis is, although not proved to exist by -laboratory tests, beyond question a factor in the case. Although the -woman had given birth to a normal child, who is still alive, yet in the -period of a few years her breasts had atrophied, her hair had -disappeared from the axilla and from the pubes; varicose veins had -developed in both legs. Whereas there was little or no fat over the -chest or back, the omentum and mesentery were very plentifully supplied -with fat. It is probable, then, that we are dealing with a case of -exophthalmic goitre somehow of syphilitic origin. The brain lesion is -consistent with this hypothesis. - - =Autopsy=, March 3, 1907. Four hours post mortem. - - Body length, 165 cm. Body of a well developed and well nourished - young woman. Lividity in dependent parts. Purplish discoloration of - left thigh to knees. Skin rough and scaly. Petechial eruption over - chest. Neck thick, protrudes anteriorly. Varicose veins over upper - parts of calves on both legs. Eyes protruding, not covered entirely - by lids. Pupils equal, dilated. Subcutaneous fat very deep over - lower part of body. Very little fat over chest and back. Breasts are - very small, apparently atrophied. Normal amount of hair on head, - slight amount over pubes. Axillary hair absent. Fat on section of a - light yellow color. Omentum extends to pubes, plentifully supplied - with fat. Large amount of mesenteric fat. Appendix normal. - Intestines smooth and glistening. Slightly injected. No fluid in - peritoneum. Uterus small, retroverted. - -[Illustration: - - Cortical hemiatrophy—A, relatively normal right precentral (“motor”) - cortex; B, atrophic left precentral. - - Note in B: - - 1. Absence of giant pyramids of Betz (corticospinal, upper motor - neurones). - - 2. Superficial (subpial) condensation of tissues with sclerosis - (gliosis). The tissues in all areas examined _on the left side_ - yielded this effect. -] - - HEAD: HAIR in good quantity. SCALP normal. CALVARIUM shows diploë. - DURA MATER over left cerebral hemisphere inseparably adherent to - calvarium, over right hemisphere normal. Arachnoidal VILLI - moderately developed. PIA MATER shows injected veins, notably in the - sulci of the right hemisphere. Pia mater everywhere thin and clear. - VESSELS at base of normal appearance. - - BRAIN weight 1180 grams. Spread on a board, the right hemisphere - tends to flatten so that it measures 1.5 cm. more from side to side - than its fellow. Besides more marked venous injection, the right - hemisphere shows also flatter and slightly more plastic - convolutions. The posterior poles of the hemispheres are a little - firmer than the parts anterior. The orbital and hippocampal gyri on - the right side are a little firmer than the surrounding parts. On - section the gray and white matter shows no lesions, excepting the - slight plasticity of the tissues at large on the right side and a - well marked induration, with retraction under the knife, of the - occipital and hippocampal white matter. The basal ganglia of the - left side are normal. On the right side a sagittal section - demonstrates a rounded area of induration, with ill-defined borders, - measuring perhaps 1.5 cm. from above downwards by 2 × 2 cm., - situated largely in the lenticular nucleus and involving the greater - portion of the globus pallidus, a small segment of the putamen below - and behind and the regionary part of the anterior commissure with - surrounding tissues. The most striking feature of this lesion is the - occurrence in the middle of a cluster of vacuoles or cystic clefts, - with smooth pale interiors, ranging from pinhead to 0.25 cm. or even - 0.5 cm. in greatest diameters. There are six to eight clefts to a - surface of section. The color of the lesion differs little from that - of the globus pallidus itself, but the tissue is a trifle - translucent. It is impossible to demarcate the lesion with the eye. - Induration is demonstrable several mm. beyond the visible part of - the lesion. The consistence of the lesion slightly surpasses the - usual consistence of the olivary bodies. - - CEREBELLUM, PONS and BULB weight 165 grams. Cerebellar tissue a - trifle more plastic than usual. The right olive is not so prominent - as usual. - - =Note.= THYROID: Weight 125 grams. Both lobes and isthmus enlarged. - One lobe more than the other; lobe on one side measuring 6 × 4 cm. - - Anatomical Diagnoses - - Enlargement of thyroid gland. - - Exophthalmos with dilated pupils. - - Fatty degeneration of thoracic muscles. - - Slight aortic sclerosis. - - Dilatation of right heart. - - Hypertrophy of left ventricle. - - Slight tricuspid endocarditis. - - Bicuspid aortic valve. - - Hypostatic pneumonia. - - Acute and chronic splenitis. - - Fatty liver (central necroses?). - - Acute nephritis. - - Chronic gastritis. - - Small breasts. - - Axillary hair absent. - - Petechial eruption of chest. - - Varicose veins. - - Chronic external adhesive pachymeningitis of left side. - - Moderate swelling of right hemisphere with venous injection. - - Slight occipital gliosis of both sides. - - Slight gliosis of orbital and hippocampal gyri of right side. - - Sclerosis with atrophy of occipital and hippocampal white matter of - right side. - - Gliotic lesion (1.5 × 2 × 2 cm. of right lenticular nucleus - involving anterior commissure). - - 1. Was the exophthalmic goitre in Carrie Pearson due to syphilis? - Unfortunately we have no clear proof that Carrie Pearson was - syphilitic. She was stated to have been syphilitic by the - physician who treated her before her commitment to Danvers - Hospital. There is, however, no proof of syphilis, inasmuch as the - patient died in the pre-Wassermann period. - - 2. Is the thalamic lesion probably syphilitic? No lymphocytosis or - plasmocytosis characterizes the lesion, which is the only lesion - of the sort in the Danvers collection. It would not do to call a - lesion syphilitic just because it is _sui generis_. In any event, - the clinical analysis of the case faced the claim of syphilis as - an actual factor in the patient’s life and as a possible factor in - the goitre. - - - =It is well known that the ARGYLL-ROBERTSON PUPIL is characteristic - of the so-called “PARA-SYPHILITIC DISEASES” (“general paresis” and - “tabes”); does this sign occur in other neurosyphilitic conditions?= - - -=Case 54.= Julius Kantor was a shoemaker of 35 years, who came to the -hospital for treatment because his family physician had found a positive -W. R. in Kantor’s blood serum. He had had a cough for a number of years, -and during the last year a little blood had been found in the sputum; -whereupon Kantor had been placed under active anti-tuberculosis -treatment. The enterprising family physician had found the positive W. -R. in the first days of his treatment for tuberculosis. There was, in -fact, a history of a chancre nine years before, which had not been -followed by any secondary or tertiary symptoms, and which had been but -scantily treated. - -There were no mental symptoms. - -Kantor was =physically= fairly well developed and nourished. There were -a few piping râles in the left upper chest, both in front and back, and -also a slight dulness with increased vocal and tactile fremitus. No -tubercle bacilli, however, could be found on repeated sputum -examination. - -=Neurologically=, the pupils were myotic and both showed the -Argyll-Robertson reaction. There were no abnormal reflexes whatever, and -there was neither ataxia nor speech defect. Not only the blood but also -the spinal fluid W. R. proved to be positive; there was a marked -increase in the albumin and globulin; there was a gold sol reaction of -the syphilitic type, and there were but three cells per cmm. - - 1. In view of the headache in case Kantor, what other causes of - headache are to be considered? It is certain that irritations of - the dura mater can produce headache, and the physiological - observation of the sensitiveness of the membranes and the - non-sensitiveness of the brain substance is an ancient and - classical observation. Internal hemorrhagic pachymeningitis - produces severe headache. The relations of this disease to trauma, - to arteriosclerosis, and possibly to syphilis (alcohol perhaps - should also be considered) in certain instances have not been - entirely cleared up. Syphilitic headaches are, according to - Lewandowski, dependent also upon a dural affection or upon a - periosteal affection. The headaches of brain tumor are also - commonly related to dural conditions, either directly due to the - pressure of the tumor itself, or indirectly to the heightened - intracranial pressure consequent upon the tumor. It is clear that - the tension under which the dura mater lies is not always - localized in the region of a brain tumor or a syphilitic lesion. - Head has claimed that brain tumor produces headaches of two kinds, - according to whether the disease affects the dura mater or is - dependent upon an increase of pressure in the brain. It does not - appear that the pia mater has any relation to headaches, but - meningitis, in which the inflammation is confined to the pia - mater, is nevertheless associated with headache; the headache is - here supposed to be due to the increase in brain pressure, and - thus actually to an effect wrought upon the dura mater. Vasomotor - disorders and various types of cephalic hyperemia are thought to - produce a kind of headache, but Lewandowski calls this kind of - headache somewhat in question. Reflex headaches are stated to be - produced indirectly by a process of radiation from interior - lesions in the brain. There are certain headaches called nodal - headaches (_Schwielen-Kopfschmerz_). Hypermetropia, caries of the - teeth, adenoids, and diseases of the nose and axillary cavities, - to say nothing of thoracic and abdominal diseases, are also - counted among conditions that may produce headaches. In this - connection, Head has claimed differential zones of headache - corresponding to certain diseases. - - The brain itself may produce headache through intoxications, - through conditions produced by a variety of diseases; may follow - neuroses. Alcohol may produce headaches in some persons even when - it is taken in very small doses. Certain uremic cases yield - headaches, as do also gouty and chlorotic conditions. According to - Lewandowski, the headaches of arteriosclerotics are due possibly - to vasomotor disturbances in the membranes, or one may think of - nutritive cerebral disorders. A peculiar form of headache is that - of fatigue after mental work, allied to which is the neurasthenic - headache; constitutional headaches have been assumed to occur, to - say nothing of hysterical headaches. There remains also the - important question of migraine, for which a vasomotor explanation - has been proposed. - - 2. Was Kantor suffering from tuberculosis of the lungs? The - hypothesis of lung syphilis ought certainly to be very seriously - considered. Upon repeated sputum examination, no tubercle bacilli - have yet been found. - - 3. Is Kantor a case of general paresis? In the absence of mental - symptoms, and in consideration of the mildness of the reactions, - it is certainly not easy to make the diagnosis of general paresis. - However, the diagnosis of tabes dorsalis is not justified either. - Accordingly, we may answer our question: whether the - Argyll-Robertson pupil occurs in other neurosyphilitic diseases, - by pointing out that in the case of Julius Kantor, as in the case - of Henri Lepère (105) and Frederick Stone (106), the - Argyll-Robertson pupil has been found in syphilitic conditions - that are neither typically paretic nor typically tabetic. - - - =Does the Argyll-Robertson pupil necessarily indicate - neurosyphilis?= - - -=Case 55.= Daniel Falvey, 44 years of age, was an almshouse transfer to -the Danvers State Hospital in the year 1904, when the principle of state -care was adopted in Massachusetts. As in most of the almshouse transfers -of that day, little could be discovered as to antecedents. He had been a -mill-worker from the time of his immigration in 1890, at 30 years of -age. He had been somewhat alcoholic. There was a shock some 17 months -before his death, which occurred about seven weeks from the date of -transfer. - -Not only was he unable to walk unsupported, but when supported there was -a slight dragging of the left leg and the gait was noted to be somewhat -propulsive. The tongue and hands were tremulous, and the left grasp was -somewhat weaker than the right. Both knee-jerks were increased although -neither more than the other. There was no sensory disorder. - -Although but 44 years of age, Falvey presented the appearance of a much -older man. His heart was somewhat enlarged and there was a degree of -peripheral arteriosclerosis. On the whole, no special attention was -attracted to this case clinically and he was regarded as an example of -arteriosclerotic dementia, like many another among the transfers. -However, we owe to Dr. H. M. Swift the important observation of the -Argyll-Robertson pupils. The case was studied long before the Wassermann -method was available, and is here reported merely to call attention to -the fact that the stiff pupils may have other neural origin than -neurosyphilis. - -The autopsy material in the case was worked up by one of the -authors.[11] The autopsy had been performed by Dr. A. M. Barrett, who -found on section through the brain stem at the anterior border of the -pons a mass springing from and continuous with the pineal gland, lying -in the third ventricle and the aqueduct of Sylvius. Upon further study, -this mass was found to begin posteriorly in the pineal body itself, from -which the mass could hardly be told in the gross except by an injected -border. - -This mass proved upon microscopic examination to be a psammoma, which -histologically resembled a glioma rather than a sarcoma. Throughout the -mass there was a variable content of fibrillary intercellular substance -having the histological reactions of neuroglia fibrillæ. The -histological details (mitosis, large giant cells with multiple nuclei, -etc.) do not here concern us. We deal with a neoplasm springing from the -pineal gland growing on the posterior half of the third ventricle, the -anterior orifice of the aqueduct of Sylvius, and the space between the -velum interpositum as far back as the posterior corpora quadrigemina. -There is no evidence in the body of old syphilis; although it is -possible that the stiff pupils were neurosyphilitic, it seems probable -that they were related to the pineal tumor. At all events, there are in -the literature evidences that the pineal-quadrigeminal group of tumors -and other lesions may bring about pupillary disturbances. On this -account, we here include the case. The tumor hardly led to an error in -diagnosis since neither neurosyphilis nor brain tumor was at all -expected clinically. - - 1. Can alcoholism produce identical results? See Case Murphy, (60), - one of alcoholic pseudoparesis. - - 2. What is the nature of stiff pupils? A pupil is called stiff in - the sense of the Argyll-Robertson pupil if it fails to react to - illumination either of itself or of the other eye and at the same - time if it reacts properly in convergence and accommodation. Of - course the stiffness of a blind eye must not be regarded as an - Argyll-Robertson pupil. In a case of right-sided Argyll-Robertson - pupil, therefore, the left pupil reacts properly both to direct - illumination of itself and to illumination of the right eye, but - the right eye fails to react to illumination of either eye. Such - an Argyll-Robertson right pupil will remain of the same width both - in darkness and in light. Clinicians agree that the - Argyll-Robertson is diagnosticated rather too frequently than too - seldom, and this by reason of the fact that a sluggishness of - light reaction is interpreted as stiffness. The sign, as is well - known, has come to be regarded as almost pathognomonic of tabetic - or paretic neurosyphilis. Nonne, however, has found among 510 - cases of alcoholism, nine instances of Argyll-Robertson pupil and - 19 cases of sluggish light reactions. The pathological anatomy of - this sign is still doubtful although a number of schematic - accounts are available; among hypotheses, one may think of an - elective effect of the tabetic or paretic degeneration upon reflex - collaterals. The explanation would then resemble that for absent - knee-jerks and kindred reflex disorders. We should then - hypothesize a loss of the finer processes of the terminal - arborizations about the cells of the nucleus of sphincter nucleus - iridis. However, the situation of the sphincter iridis has not yet - been absolutely determined. - - When a pupil is said to be entirely stiff it means that it reacts - neither to light nor accommodation. This condition not - infrequently follows the partial stiffness or Argyll-Robertson - reaction. - - 3. Is the Argyll-Robertson pupil more tabetic than paretic? This has - been claimed at times, but in point of fact, the Argyll-Robertson - pupil is very frequent in paresis, and so also are posterior - column changes. According to statistics of Bumke, 36% of tabetics - fail to show the Argyll-Robertson pupil, and 38% of paretics. - When, however, finer methods, such as those standardized by - Weiler, with photographic records, are employed, the number of - cases without at least a tendency to the Argyll-Robertson pupil - becomes much smaller. - - In connection with the important question as to the classical - Argyll-Robertson pupil and pupillary sluggishness to light, it may - be inquired what are the ocular signs in neurosyphilis? Joffroy - has tabulated the signs in 300 general paretics as follows: - - Sign. No. of Per - cases. cent. - Alterations of light reflex 235 78 - Inequality 205 68 - Abolition of light reflex (bilateral or unilateral) 156 52 - Abolition of light reflex (bilateral) 133 44 - Irregularity of pupil 117 39 - Irregularity of both pupils 109 36 - Diminution of light reflex 108 36 - ditto (bilateral) 79 26 - Alteration in accommodation reflex 79 26 - Diminution of accommodation reflex 52 17 - Mydriasis 41 13 - Myosis 40 13 - Diminution of light reflex (unilateral) 35 11 - Abolition of accommodation reflex 35 11 - Diminution of accommodation reflex (bilateral) 29 9 - Abolition of accommodation reflex (bilateral) 26 8 - Diminution of accommodation reflex (unilateral) 23 7 - Fundus changes 21 7 - Vascular changes 16 5 - Abolition of accommodation reflex (unilateral) 12 4 - Paresis of the third nerves 10 3 - Ptosis 9 3 - Irregularity of one pupil 8 3 - Nystagmus 7 2 - Visual acuity lost 7 2 - Atrophy of disc 6 2 - Total blindness 5 2 - Paralysis of the fourth nerves 1 1 - - - =Can neurosyphilis exist in the absence of positive findings in the - spinal fluid?= - - -=Case 56.= There was no great difficulty in setting up a diagnosis of -general paresis in the case of James Burns, a mechanic of 31 years of -age, who came voluntarily to the Psychopathic Hospital for treatment. -The point in Burns’ case was that the spinal fluid proved entirely -negative in all respects despite the fact that the serum W. R. was -positive, and despite the following facts of history and mental -examination. - -The patient claimed syphilitic infection seven years before, namely, at -24 years of age, and also claimed that he had infected his wife, who was -in fact at the time undergoing antisyphilitic treatment. He complained -of insomnia, worry, depression, hypersensitivity to noises (such as -those made by his own children), thoughts of suicide, and amnesia. The -amnesia, however, might be regarded as subjective since our tests failed -to show amnesia. Nor was there any diminution in arithmetical ability. -Despite the patient’s claim that he had been “way off in his way of -thinking,” there appeared to be no delusions. Beyond a certain -flightiness in conversation, we could hardly get any evidence of -psychosis unless of the neurasthenic order. - -=Physically=, however, the left pupil failed to react to light though it -was found to react to distance, and the right pupil exhibited a -diminution of its reaction to light. There was no ataxia of gait, yet -there was a complete Romberg reaction. There was a moderate tremor of -the hands and of the tongue. Otherwise there were no reflex disorders -upon systematic examination, nor was there any demonstrable disorder in -the rest of the physical examination. - - 1. What is the diagnosis in the case of James Burns? On the whole we - agree with Nonne, that negative spinal fluid findings (of course, - in the absence of treatment) preclude the diagnosis of general - paresis. The symptoms might possibly be explained, however, by - means of a localized syphilitic involvement of the cerebrum, no - cells or products of inflammation having penetrated to the spinal - fluid. According to Head and Fearnsides, this condition may be - found especially in the anterior or middle fossa. Accordingly, - going upon these views of Nonne and of Head and Fearnsides, we - should be entitled to make, perhaps, a diagnosis of cerebral - syphilis. - - 2. What is the significance of the Argyll-Robertson pupil in James - Burns? Nonne states that if one follows cases with - Argyll-Robertson pupil over a sufficient period of years, they one - and all eventuate in active symptoms of cerebrospinal syphilis - (not necessarily of the cortical type), and this despite the fact - that the pupillary change may have been present a number of years - before any other symptom had developed. - - - =Neurosyphilis (“DISSEMINATED ENCEPHALITIS”) within seven months of - initial infection. Autopsy.= - - -=Case 57.= We borrow the main features of a remarkable case examined at -the Danvers State Hospital clinically by Dr. H. W. Mitchell and reported -elaborately by Dr. A. M. Barrett. This case, whom we shall call John -Summers, acquired syphilis at about the end of the third week in May, -1902, and consulted a physician on June 12, at which time a -characteristic initial lesion of syphilis was plain. Summers was -excessively alcoholic at times and was not seen by a physician again -until July 2, just after an alcoholic debauch. At this time there was -ulceration of the primary lesion, and a papillary eruption had developed -over the arms, chest, abdomen, and legs. Mercurial treatment and mixed -treatment were given. Arthritis occurred but disappeared with increased -dosage. - -About six months after infection, the patient developed severe -headaches, hardly controllable by treatment. Amnesia and a certain -stupidity, with neglect of personal habits, and even of eating, -developed, whereupon Summers was admitted to the Danvers Hospital, -December 11, 1902. He weighed 124 pounds, was extremely feeble, with -dull and expressionless face, coarse purposeless movements of arms; left -pupil larger than right; right external strabismus and ocular ptosis; -increased knee-jerks, crossed adductor reflex, coarse tremors of arms -and hands; and extreme clouding of consciousness. It was doubtful -whether the pupils were stiff to light or not. - -The patient died on the ninth day, December 18, in a state of coma. -After admission, his stupor had become more marked; there had been -incontinence of urine and fæces, and the patient could be aroused only -by loud tones. Difficulty in swallowing had developed; the right-sided -ptosis had become more marked, and muscular twitchings had developed on -the right side. When the left leg was pinched, there was twitching of -the left leg and arm. There was slight spasticity of the right arm and -leg. An examination upon the day of death definitely showed a lack of -reaction of the pupils to light. - -[Illustration: - - 1. Exudate in pia mater—mononucleosis. -] - -[Illustration: - - 2. Superficial (subpial) cellular reaction of neuroglia tissue - (expanded cell bodies). -] - -[Illustration: - - 3. Cellular gliosis of deeper layers of cortex. Apparent increase in - capillary supply, possibly relative to loss of neural elements. -] - - Case 57. Neurosyphilis (“disseminated syphilitic encephalitis” of A. M. - Barrett), fatal seven months from initial infection. (Photographs by A. - M. Barrett.) - -Dr. Barrett was able to find in the literature a case of Bechterew which -histologically resembled his own case, but though in the instance -reported by Bechterew the first symptoms developed within the year -following infection, death did not occur until two years later. - -In view of a total duration of symptoms clearly not over seven months, -it is interesting to inquire how far microscopic brain changes could -have proceeded. Neither calvarium nor dura mater showed changes. There -was a slight haziness of the pia mater over the convexity, but the pia -mater over the base (especially below the cisterna and from thence -spreading out over the pons and into the fissure of Sylvius) was not -only hazy but definitely thickened and hyperæmic. The thickening was -most marked about the root of the right third nerve (corresponding with -the eye findings in life). There was also a macroscopic thickening of -the left Sylvian artery. Section of the brain showed nothing abnormal -except a small area among the pyramidal fibres of the right side of the -pons, where there was a single hemorrhagic area about 7 mm. in diameter -around which there were small punctiform hemorrhages. (Compare -twitchings of left leg and arm upon stimulation of left leg, and note -also the muscular twitchings and slight spasticity of right leg and arm -noted just before death.) This case was examined and reported upon in -1905. We learn from Dr. Barrett that a re-study of the case with modern -methods has failed to demonstrate a spirochetosis. - -The meninges show infiltration and destructive and proliferative changes -of the blood vessels. Condensed extracts from Dr. Barrett’s full report -follow: - - There were local variations in the severity of the meningitis. The - sulci showed the most marked infiltration. The slighter degrees of - exudation were made up largely of lymphocytes with a few plasma - cells, occasionally large mononuclear cells, and rarely a - polymorphonuclear leukocyte. Where the exudation was more extensive, - the large mononuclear cells became more common and the - polymorphonuclear leukocytes increased in number. The large - mononuclear cells were often phagocytic, containing from one to six - leukocytes. The exudate was always most abundant about the blood - vessels. The plasma cells were always most numerous in the - adventitia of the veins, here greatly outnumbering the leukocytes. - The polymorphonuclear leukocytes were relatively infrequent except - where there were necrotic areas, which areas were usually continuous - with an infiltration of a vessel wall. - - As to vascular changes, the media was not often involved, nor was - the adventitia so often affected as the intima. Such lesions as - appeared in the intima and adventitia were infiltrative rather than - proliferative. The elastica of the blood vessels proved to show but - slight changes. - - A characteristic change was the endarteritis,—of a focal nature with - a few large mononuclear and lymphocytic cells pushing the intima - inward at the edge of a lesion. In the more marked portion of the - focal process, the thickness of the intima was greatly increased by - proliferation. Great numbers of large mononuclear cells could be - seen between the intima and the elastica. About these cells and - interlacing among the other elements of the proliferating tissue was - an excess of connective tissue fibres. - - The meningeal veins were more often diseased than the arteries; - there was adventitial infiltration with lymphoid and plasma cells; - sometimes the vein walls had become necrotic and infiltrated with - polymorphonuclear leukocytes. - - It will be remembered that the left Sylvian artery was grossly - thickened, and microscopic section of this vessel showed a partial - thrombosis. - - The brain showed diffuse and focal changes. The _diffuse_ process - was one of nerve cell degeneration and proliferative changes in the - neuroglia and blood vessels, and no section of the many examined - proved to be free from such changes, although in the majority of - instances, these diffuse changes were slight. The cortical layers - showed more of these diffuse changes than did the white substance. - Barrett considered that the glial cell changes were more delicate - indicators of the cortical changes than the nerve cell changes. He - found rod cells, satellitosis, superficial gliosis, and a large - gamut of changes in the neuroglia. There were two rather - characteristic nerve cell changes: a shrinkage change going on to - almost complete destruction, and a type of cell swelling, also - apparently proceeding to complete destruction. - -[Illustration: - - 4. Arteritis of pia mater. -] - -[Illustration: - - 5. Focal vascular lesions. - - Case 57. Seven months from infection. “Disseminated syphilitic - encephalitis,” Barrett. (Photographs by Barrett.) -] - -[Illustration: - - Paretic neurosyphilis (“general paresis”)—cerebral atrophy, _without_ - meningitis. Therapeutics cannot hope to restore lost tissue. - - Duration. 3 years from beginning of well marked symptoms; 6 years from - beginning of obvious symptoms; 12 years from a so-called “nervous - prostration.” -] - - Among _focal_ changes, there were four main types: Areas of - encephalitis, having the general appearance of granulation tissue, - areas of simple necrosis or softening, apparently directly related - to vascular changes near by, hemorrhages, and certain foci regarded - as gummatous. - - Save for pial infiltration and a few vascular changes, there was - very little change in the medulla and spinal cord. There was a - hypertrophic gliosis of the margin of the medulla and cord - throughout, and a focal lesion of well marked gliosis at one point - in the bulb. There were no fibre degenerations in the medulla or - cord, nor were there any coarse fibre degenerations in the cortex - itself except in one locus, the left gyrus rectus. This case is of - peculiar value in showing to what extent lesions may proceed in a - period of six to eight months after primary infection. - -Of course the case is in one sense entirely atypical. The lesions were -not confined to the nervous system. Aside from the maculo-papular -eruption and ecchymosis of the skin, there was a diffuse hemorrhage of -the inner half of the conjunctiva of the left eyeball, a small -hemorrhagic focus in the mitral valve, a caseous nodule, one cm. in -diameter, in the apex of the left lung whose tuberculous or syphilitic -nature is left in doubt; a broad milk-colored patch of thickening of the -capsule of the liver. It is to be noted that there were no gross lesions -of the aorta. - - - =On the classical assumption that PARETIC NEUROSYPHILIS (“general - paresis”) is a fatal disease, is there a disease PSEUDOPARETIC - NEUROSYPHILIS (“pseudoparesis”) which may recover or pursue a long - course like that of a case of diffuse neurosyphilis (“cerebrospinal - syphilis”)?= - - -=Case 58.= Peter Burkhardt had been an efficient highway inspector, but -in his forty-fifth year he had begun to be unable to do his work -satisfactorily. His wife had become somewhat afraid of him. He had had -somnolent spells in his chair and had squandered money. The mental -symptoms had lasted for some six months, but had become more marked -during the month preceding admission. Burkhardt would at times fail to -recognize his friends. - -The general =physical condition= of Burkhardt was very good. The pupils -were irregular and reacted sluggishly both to light and to -accommodation. The knee-jerks and ankle-jerks were absent. There were no -other neurological disorders upon systematic examination. There was a -speech defect. =Mentally=, little could be determined except a certain -sluggishness. - -History and physical examination at once suggested general paresis. The -serum W. R. was doubtful, but the spinal fluid reaction was positive, as -was the gold sol reaction (which was “paretic”); the globulin and -albumin were greatly increased; there were 48 cells per cmm. -Antisyphilitic treatment, consisting of salvarsan twice a week and -potassium iodid by mouth, was followed by a rapid mental improvement. -After two months, Burkhardt was discharged apparently normal, with all -the blood and spinal fluid tests negative. He has been taken back into -the highway service. - - 1. What is the proper definition of pseudoparesis? Fournier termed - pseudoparesis certain cases that looked like paresis but were not - syphilitic in origin. Of these cases the most characteristic group - is that of alcoholic pseudoparesis. It is clear that there will be - no difficulty in the definition of a disease pseudoparesis whose - entity is presented in the adjective that precedes the term - (_e.g._, alcoholic pseudoparesis). According to this usage, a case - of pseudoparesis would be one in which the symptoms and possibly - some of the signs somewhat resemble the symptoms of paresis itself - but for which another etiology could be fairly established. - - 2. Are there any cases of syphilitic pseudoparesis? We are of the - opinion that the term should be dropped. It is true that there are - cases which clinically look like general paresis and exhibit the - appropriate laboratory signs of general paresis but seem to differ - from paresis in their course even when they receive no treatment - whatever. In the present phase of doubt as to the classification - of paretic and non-paretic forms of neurosyphilis, it seems to us - of doubtful utility to characterize a case as pseudo simply - because it differs in its course, particularly as the literature - has always duly recognized that a number of cases of general - paresis have had long courses and sometimes very long remissions. - - There is also another group of cases that have been termed cases - of pseudoparesis, namely: certain cases of neurosyphilis which - clinically look like general paresis and seem to be following its - classical course but are interrupted by treatment. Here again it - seems to us doubtful whether the designation pseudo should be - attached to this group of cases, particularly while the whole - therapeutic question in the paretic group of neurosyphilis cases - remains _sub judice_. Accordingly we are tempted to include in the - group of paretic neurosyphilis cases that either get well of - themselves, or get well under treatment, or pursue a very long - course, or are subject to very long remissions. But we make this - decision in terminology without prejudice to the therapeutic - question and it is open to any critic to throw these cases into an - atypical non-paretic group of neurosyphilis cases. - - 3. How shall we explain the absence of ataxia of case Burkhardt when - knee-jerks are absent and when, therefore, we are entitled to - conclude a certain degree of spinal disease? As stated in - connection with case Sullivan (16), the absence of knee-jerks is - not a warrant for terming a case—paresis of the tabetic form. The - fact is that the lesion in paresis tends to be intraspinal, just - as the higher brain lesions tend to occur within the brain - substance. The meninges are relatively spared both within the - cranium and within the spinal canal. The characteristic - degeneration of posterior nerve roots which we find in tabes - dorsalis is not necessarily found in general paresis even when - there are somewhat extensive spinal lesions. Accordingly the - absence of sensory returns by way of the posterior nerve roots - which characterizes tabes dorsalis is not necessarily a phenomenon - of general paresis. The mechanism by which the knee-jerks are lost - depends upon histological detail. They may be lost when under - tabetic conditions the posterior roots are severely diseased and - when under paretic conditions only intraspinal collaterals or a - small portion of fibres are affected. The whole question hinges - upon where and to what degree the various reflex arcs are cut in - the disease. The tabetic phenomena are, as so commonly stated, - intradural; that is, the sensory or gangliospinal neurones at - certain levels are affected all the way in from the points at - which they pierce the dura mater. The affection of these and other - neurones in general paresis is an intraspinal and parenchymatous - affection. - - - =Neurosyphilis; auditory hallucinations; ideas of persecution; - attacks of excitement. SYPHILITIC PARANOIA (Kraepelin)?= - - -=Case 59.= Bridget Curley was a case that was discharged from the -Psychopathic Hospital, recovered, after 26 days in hospital. The -symptoms so resembled those of alcoholic hallucinosis that the diagnosis -was made despite the fact that the patient consistently denied the use -of intoxicants. There was, in fact, no proof that she drank alcohol. The -case was, however, not clearly one of alcoholic hallucinosis or of any -other well-defined form of mental disease. A provisional diagnosis of -manic-depressive psychosis, manic phase, had, in fact, also been made. - -The illness had begun with depression and inactivity, Bridget’s friends -accounted for these conditions on the ground that a lover had departed -for Ireland. A few days after the depression began, Bridget became dizzy -and refused to give a boarder his breakfast, stating that she had lost -her memory and had begun to hear bells ringing and people talking. She -then became greatly excited and was brought to hospital, where the -prolonged baths quieted her. - -It seems that Bridget had had stomach trouble and headaches at the top -of her head or sometimes in her temples. =Physical examination= showed -the left pupil to be larger than the right, a slight tremor of the lips, -a slight systolic murmur at the apex, slightly irregular pulse, and -moderate edema of ankles. The blood serum was negative to the W. R., but -lumbar puncture was executed and the fluid showed a positive W. R. - -The patient was tested by the Binet and other methods, and although 35 -years of age, seemed to be by the mental tests hardly over 11 years old. -She was inclined to be feverish, somewhat restive, and pugnacious; -rather slow of speech, sometimes refusing to answer and grimacing. Her -pugnacity was, however, easily controllable, and the excitement was -largely at night. This excitement subsided rapidly in the course of a -few days. - - 1. What is the diagnosis in this case? The following diagnoses and - suggestions for diagnosis were made at the staff meetings: - - Unclassified mania. - Manic-depressive psychosis, manic phase. - Toxic delirium. - Dementia praecox. - Bacterial infection of the brain. - Unclassified delirium. - Acute delirium. - Infectious psychosis. - Acute confusional psychosis. - Psychopathic personality by use of alcohol. - Mental deficiency with atypical mental state. - Syphilitic paranoia. - - 2. Is this a case of syphilitic paranoia? The so-called syphilitic - paranoia of Kraepelin is a rare and uncertain type of syphilitic - mental disease. Delusions and hallucinations are prominent. As a - rule, the onset is stated to be slow and insidious, or at any rate - there are a variety of indefinite prodromata. Jealousy is a - prominent feature, sometimes attended with marked sexual - excitement. Auditory hallucinations and ideas of persecution are - particularly in evidence. The most striking feature in Kraepelin’s - group was a sudden occurrence and equally sudden disappearance of - violent excitement, with or without external cause. Thus, an - excitement would be produced by a few words spoken, and - immediately after, the phase of excitement would pass and the - patient would become entirely friendly and accessible once more, - as if nothing had happened. About half of Kraepelin’s cases showed - a positive serum W. R. He does not report lumbar puncture - findings, and grounds the existence of disease upon certain - autopsied cases. The speech and writing disorder of paresis as - well as the characteristic disorientation for time and muscular - weakness of general paresis were absent in the group. It appears - that most cases of the group have hitherto been placed in dementia - praecox. - - - =The clinical symptoms of CHRONIC ALCOHOLISM are sometimes largely - identical with those of PARETIC NEUROSYPHILIS (“general paresis”): - differentiation by means of the laboratory findings.= - -To demonstrate this proposition, the cases of Francis Murphy (60) and -David Collins (61) are in point, being sharp foils to one another. - - -=Case 60.= A laboring man about 44 years of age was brought to the -Psychopathic Hospital one summer day, in a stupor. This patient, Francis -Murphy, had been at his regular work as axeman in the Park Service, when -he suddenly fell in a heavy convulsion. He was carried to a general -hospital, still in convulsions, and ether was administered to quiet the -movements. The convulsions shortly ceased, but the patient’s -consciousness failed to clear; hence his transfer to the Psychopathic -Hospital. - -Here he remained much disturbed and was placed in a room with a mattress -on the floor. On this mattress he would crouch on all fours for a -considerable time, looking fixedly downward as if at an object on the -floor, unresponsive to questions but compliant with efforts to place him -on his back. He gave the impression of daze and either disorientation or -confusion. - -Within twenty-four hours the patient became more tranquil and -consciousness became clearer, but the patient was at a loss to bring to -memory either recent or remote events. However, he replied to questions, -giving some different story each time he was approached. Curiously -enough, the patient seemed very contented and good-natured and would -even laugh foolishly at times, saying that he felt fine and all ready to -go out to work. - -The general impression conveyed by Francis Murphy at once suggested the -possibility of neurosyphilis. Convulsions, perhaps initial in middle -age, with a post-convulsive stupor, followed by a partial clearing up, -with persistent amnesia and a suggestion of fabrications with euphoria, -bore out the suggestion. - -The =physical examination= strengthened the impression of neurosyphilis. -Well developed and nourished, florid, with a manual tremor and sweating -of the palms, the patient was in general without physical symptoms. -=Neurologically=, however, whereas the left pupil was larger than the -right and reacted properly to light, the right pupil was a bit -contracted, somewhat irregular, and either reacted not at all to light -or very slightly so (reacting perfectly to accommodation). The -knee-jerks could be obtained only with reinforcement, and several other -reflexes could not be elicited (triceps, radial, ulnar, periosteal, -Achilles, umbilical). Moreover, the heel-to-knee test was poorly -performed; some of the common tests phrases were very poorly repeated; -there was marked tremor in writing; and the paragraphia seemed to be not -merely peripheral, for syllables were left out in words and ordinary -words spelled incorrectly (psychographic disturbance). - -We do not care here to insist that the right pupil was really an example -of the Argyll-Robertson phenomenon since the slightest tinge of doubt is -important if a positive diagnosis is practically equivalent to asserting -syphilis. Practically, however, the right pupil was regarded as an -Argyll-Robertson pupil under hospital conditions (flash-light reaction). -Argyll-Robertson pupil, areflexia, speech disorder, writing disorder, -memory disorder, conduct disorder, and euphoria, all with a history of -convulsions, certainly warranted the tentative diagnosis of -neurosyphilis. - -As usual, resort was made to the W. R. in the serum and in the spinal -fluid. One of the first results to come through from the laboratory was -the absence of globulin, normal albumin, negative gold sol reaction, and -a cell count of two cells per cmm. in the spinal fluid. Later the _W. -R.’s_ were returned _negative_ for blood and spinal fluid. - -In the meantime, an illuminating change had occurred in the patient, for -two days later,—three days after the first convulsion in the park,—the -patient had apparently quite recovered; his consciousness became nearly -clear; he could remember every event up to the time of the convulsion, -and his memory came back in appropriate degree for both remote and -recent events. - -The patient, it appeared, had for some time been drinking more and more -heavily. In recent days, he had been taking five or six whiskeys and a -half dozen beers daily on the average, and often much more. About ten -years before, the patient narrated, there had been a convulsion at a -ballgame, and this convulsion the patient himself called a “rum fit.” - -Here, then, is a case of ALCOHOLIC PSEUDOPARESIS. Without the W. serum -test and without the spinal fluid examination, it is probable that the -diagnosis of general paresis might have clung to the patient for some -time on account of the apparent Argyll-Robertson pupil, which had to be -accepted as such on the flash-light data. In point of fact, in this case -the pupil later reacted more normally to light, and the speech and -writing disorders measurably cleared up. - - 1. Can alcohol produce the Argyll-Robertson pupil? The majority of - neurologists would today answer, Yes. - - 2. If in the case of Francis Murphy, the W. R. in the blood had - happened to be positive on account of a non-neural syphilitic - infection (spinal fluid negative), would the diagnosis _general - paresis_ be warranted? Probably the diagnosis _general paresis_ - would have been made. If the patient had been lost to observation, - he might well have been regarded as an atypical paretic with - prodromal convulsions. - - 3. Would positive globulin and excess albumin in the spinal fluid - alone or in association with a positive serum W. R. warrant the - diagnosis _general paresis_ or _neurosyphilis_? The chances are - that most neurologists would advocate proceeding to treatment in - any case of positive serum reaction, whether or not there was - globulin or excess albumin; but the positive globulin and excess - albumin would probably not warrant the diagnosis _general paresis_ - or _neurosyphilis_ in the absence of excess cells and the - characteristic gold sol reaction and W. R. in the fluid. - - 4. Is the case of Francis Murphy one of alcoholic epilepsy (as - suggested by Murphy’s own phrase, “rum fits”)? It must be - remembered that epileptics become alcoholic and that epileptic - convulsions increase or become more severe with alcoholism. On the - other hand, the literature indicates that alcoholism can produce - convulsions, as can many other factors. The literature also - indicates that there is a condition of epilepsy in which the - convulsive tendency sets in as a result of alcoholism in a patient - not previously disposed to epilepsy; it appears also that - sometimes, though very rarely, the epilepsy continues after - withdrawal of alcohol, and even after giving up the habit. Francis - Murphy appears to have had but two spells of convulsions, both of - them following heavy bouts with alcohol. There is so far, then, no - warrant for calling Francis Murphy’s case one of alcoholic - epilepsy. - - 5. Does the use of alcohol by a subject destroy the value of the - W.R.? It has been held by some that alcoholism interferes with the - accuracy of the W. R. This has not been our experience and for the - present we are of opinion that the results have the same value in - alcoholics as in non-alcoholics. The next case (Collins, 61) is - one in which a positive W. R. occurred in an alcoholic. When - dealing with paretic neurosyphilis it is especially true that the - W. R. is disturbed very rarely, if at all, by toxins or drugs, - except antisyphilitic drugs. - - - =Alcoholism may cloud the diagnosis of NEUROSYPHILIS. - Differentiation by laboratory tests.= - - -=Case 61.= David Collins was a steamfitter of about 43 years of age, -picked up at 6.45 a.m. in the midst of convulsions and talking -incoherently, in a state apparently of fairly clear consciousness. On -arrival at the hospital, the patient was able to tell how he had always -been a hard drinker, and how during the past week of unemployment he had -taken large quantities of poor whiskey,—perhaps an average of a pint a -day. Collins also told how he had had delirium tremens several times, -but he said the present spell was quite unlike delirium tremens. There -was no disorientation or impairment of memory, and the patient did not -in any wise suggest a mental case a few hours after admission. - -It appears, according to Collins, that he had obtained some work the -night before, and had quit work about 6.30, whereupon he stepped into a -barroom, took one drink of whiskey, left the barroom, walked down the -street, and suddenly lost track of the world, coming to consciousness in -a carriage with two policemen, but remaining, as he said, “dopy,” -inattentive, and confused. After a meal, however, the patient began to -feel better and soon felt quite all right. - -The =physical examination= was quite negative except that -=neurologically= there was lingual and manual tremor, a speech defect, -apparent only with test phrases, unsteadiness of handwriting, left -knee-jerk greater than right, a left-sided Babinski reflex, and a -difficulty in executing rapid successive movements (dysdiadochokinesis). -This degree of neurological disorder in our experience warrants lumbar -puncture as well as a serum test. The lumbar puncture shortly disclosed -a positive globulin and excess albumin, and the returns from the W. R.’s -were positive for both spinal fluid and blood serum. The data of the -gold sol reaction were not available on account of technical -difficulties. However, it appears that the diagnosis of neurosyphilis -could hardly be avoided in this case. - -David Collins differs from Francis Murphy, then, in showing a positive -blood and spinal fluid reaction for syphilis as well as a positive -globulin and excess albumin. As above remarked, it is probable that the -positive globulin and excess albumin would not warrant more than a -suspicion of neurosyphilis taken by themselves. - -Unfortunately, we were unable to persuade the patient to submit to -treatment, and from the patient’s point of view possibly his decision, -not to submit to treatment, was a good one since he has had no symptoms -of any sort for a period of 18 months since his episode. However, as -abundantly elsewhere demonstrated, we feel that the patient is wrong, -and that the physicians are right in urging treatment. - - 1. Is not the convulsive episode an alcoholic phenomenon in David - Collins entirely separate from the patient’s general and - neurosyphilis? Possibly; however, an outbreak of neurological - symptoms with spontaneous recovery is not only consistent with the - diagnosis of syphilis, but somewhat characteristic of - neurosyphilis. We suspect that another attack will occur in David - Collins.[12] We shall from time to time make use of the social - service to suggest his going under treatment, and shall employ his - record of contact with a public institution to drive in our - suggestion. Still it is clear that there are numerous cases in the - community that are not accessible to social service initiated from - a public institution. Accordingly, educational propaganda is - necessary for salvage of the middle- and upper-class victims of - syphilis. It is a little unfortunate that the ethics of the - private practitioner make such salvage of middle- and upper-class - persons not very likely. Might it not be that an extension of - state medicine to this field would incidentally increase the - amount of successful private practice? - - 2. What may be the cause of such a convulsive episode as that of - David Collins? It would appear that the convulsions of general - paresis and of neurosyphilis in general often occur without gross - structural lesions of the brain. It may be suggested that vascular - irritation or parenchymal irritation by spirochetes, acting in - appropriate parts of the central nervous system, can produce such - convulsions. - - 3. What is the significance of the unilateral phenomenon in David - Collins (left knee-jerk greater than right; left-sided Babinski)? - The current explanation of hyperreflexia is that somehow - inhibitory impulses from upper portions of the nervous system have - ceased to influence the local arcs that mechanize reactions like - the knee-jerk and the normal plantar reflex. The phenomena are - commonly found in cases with pyramidal tract disorder, and in the - case of David Collins one may suspect, therefore, that there was a - central disorder affecting the right pyramidal tract above its - decussation. One might suspect that the convulsions were initiated - by a lesion (whether gross or microscopic in range) in the right - side of the cerebrum; but whether in the white matter or in the - gray matter must be left doubtful. The clearing up of all symptoms - suggests either that the lesion was microscopic in range or that - the phenomena were transient and functional. - - 4. Can the dysdiadochokinesis be used to indicate cerebellar lesion - in David Collins? Possibly; but it does not appear that the - difficulty in executing successive movements was unilateral. It - seems impossible to bring into close topographical relation the - basis for the Babinski and left-sided hyperreflexia, and the basis - for the dysdiadochokinesis. Alcohol is sometimes asserted to exert - an especial effect upon the cerebellum. - - 5. Must we suppose structural lesions, either (a) of the nature of - cell losses demonstrable microscopically, or (b) of the nature of - secondary degenerations demonstrable by Weigert myelin sheath - methods, in the case of David Collins? It appears that we do not - need to assert the existence of such lesions. - - 6. Could the hyperreflexia and the Babinski reaction be due to local - spinal cord disease? Possibly; but the existence of other - neurological symptoms (lingual and manual tremor, speech defect to - test phrases, ataxic handwriting, and dysdiadochokinesis) makes it - probable that there were lesions, or at any rate disordered - functions, within the cranium; and there appears to be no basis - for asserting local spinal cord disease. - - - =Differential diagnosis between NEUROSYPHILIS and ACUTE ALCOHOLIC - PSYCHOSIS.= - - -=Case 62.= Joseph Buck was a chef of 60 years who came in, seeking -advice because his memory was getting poor; he was unable to remember -names and what he was about to do. He was tremulous and had much pain in -his limbs. He had been drinking heavily for weeks,—probably ten weeks; -in fact, he described himself as having had “the shakes” and as having -lately seen animals and people that were unreal. He had had the shakes -before and the condition had lasted for two to three days after alcohol -was discontinued. - -=Physically=, Buck was tall, well developed, although poorly nourished, -with a skin suggesting alcoholism. There was a slight acne over the back -and chest; there was a slight enlargement of the heart, with blood -pressure, systolic, 180, diastolic, 120. There was a corneal opacity of -the left eye, which the patient said was the result of syphilis -following a chancre, which he had acquired at the age of 27. There was -also a ptosis of the upper lid of the left eye. The right pupil was -irregular and reacted to light sluggishly, and with a very small -excursion. The patient was slightly deaf in both ears. The deep reflexes -were all lively and equal. The tremor was most marked in finely -coördinated movements. There was a slight swaying in the Romberg -position but the sign could not be said to be present. The gait was -unsteady. There was a marked tenderness over the nerve trunks. - -So far as =mental examination= went, it seemed that the patient’s claim -of amnesia was subjective. There was certainly no more amnesia than a -slight difficulty in recalling details. The diagnosis of alcoholism with -convalescence from delirium tremens would certainly seem to have been -sufficient for the phenomena, and the suggestion of alcoholic neuritis -only confirmed the picture. To be sure, one might expect a diminution or -absence of deep reflexes; still, these reflexes may be overactive in an -irritative stage of the disease. - -Naturally, however, the history of syphilis and the pupillary phenomena -and ptosis, made the consideration of neurosyphilis necessary. Both -serum and fluid W. R.’s proved positive; there was an excessive amount -of albumin and globulin, the gold sol reaction was typically “paretic,” -and there were 377 cells per cmm. - -The patient improved upon a rest treatment and was given injections of -mercury for his syphilis. After a few months he felt well enough to -return to work, and continued at work throughout a season, receiving -mercurial treatment throughout this time. A spinal fluid examination -fifteen months later showed a weaker gold sol reaction, reduction in the -amount of globulin and albumin, and but 26 cells to the cmm. The W. R.’s -had remained positive. - - 1. What are the forms of syphilitic neuritis? According to Nonne, - syphilitic neuritis and polyneuritis have at last acquired - standing in neuropathology. The older claims depended upon - findings on palpation and recovery after antisyphilitic treatment. - Since the introduction of salvarsan, cases of ophthalmoplegia, - facial, acoustic, and optic nerve disease, as well as neuritis of - the extremities, have been reported in large numbers. These - phenomena are to be regarded as neurorecidives in the modern sense - of that term. The neurorecidive is not a salvarsan effect, but is - an effect of the syphilitic process itself, settling in the - peripheral nerves. Paresthesias are especially prominent in - peripheral mono- or polyneuritis, and this point is of some value - in differentiating the syphilitic peripheral neuritis from root - neuritis. Root neuritis is more often characterized by neuralgic - attacks. Objective hyperæsthesia of neuromuscular origin is also - found in these cases, demonstrated by pressure on the nerves. The - motor phenomena consist in a flaccid paresis or paralysis, - especially affecting the radial, ulnar, and peroneal nerves. Nonne - states that it is rare for syphilis to affect a single nerve - region, and he regards cases in which a single region alone is - affected as usually due to a local gummatous process. - - 2. What is the significance of 377 cells per cmm.? See discussion of - Washington (Case 66). - - - =Differential diagnosis between NEUROSYPHILIS and CHRONIC - ALCOHOLISM.= - - -=Case 63.= Albert Fielding, 46, was an insurance broker, who was brought -to the hospital for excessive alcoholism. Indeed, he showed all the -signs, both of chronic and acute intoxication, except that there was no -nerve trunk tenderness. Fielding was very loquacious though his speech -was rather thick. He showed tremor of hands and an alcoholic skin. -Physical and neurological examination proved entirely negative. - -Fielding claimed that he had had a nervous breakdown at about 36 years -of age, after disappointment in love. He had the drinking habit and -began to drink more and more. He had now become nervous and tremulous -and had to drink in order to brace himself. After a few days, the -patient began to be much better, having recovered from acute alcoholism. -=Mental examination= now showed good memory with orientation intact. -There was a certain tendency to reminiscence and to somewhat childish -actions. He had attempted to stop drinking but had been unable to quit. -As a matter of fact, his mother and father had been excessive drinkers -and he had inherited the tendency, etc. - -The =diagnosis= seemed to be plain. The routine W. R. upon the blood -serum was negative. However, the patient had remarked during the history -taking, that he had had a chancre and secondary symptoms of syphilis. -Accordingly, lumbar puncture was resorted to. The fluid showed a -slightly positive W. R.; the gold sol reaction was of the syphilitic -type; there was a considerable increase in albumin and globulin, and -there were 20 cells per cmm. The diagnosis of neurosyphilis seemed -clear. - -=Course=: The patient received six months’ treatment in a sanatorium but -the symptoms remained almost as before, and the patient showed the same -childishness and inability to take care of himself. Since the symptoms -continued six months after the withdrawal of alcohol, it might well be -suspected that the condition was more than a merely alcoholic one. -However, in a number of purely alcoholic cases, such long-standing -effects are found: even as long as six months or longer after the -withdrawal of the alcohol, and one might conclude therefore that -Fielding was actually a victim of alcoholic dementia. The spinal fluid -after these six months (during which period antisyphilitic treatment was -given) showed no change, and the prognosis was offered that the case -would probably develop into one of paresis. - -A year later, after six months sanatorial care and six months life in -the community, the patient returned to the Psychopathic Hospital in an -alcoholic condition. The lumbar puncture showed all signs negative -except the W. R. which was slightly positive. The W. R. of the blood was -negative. - -In connection with this case, see the case of _paresis sine paresi_ -(25). - - 1. What is the relation of the syphilitic and alcoholic process in - Robert Fielding? One does not like to break the so-called rule of - parsimony in diagnosis, but it would seem that the effects in - Fielding are the combined effects of syphilis and alcoholism. - - - =Differential diagnosis between NEUROSYPHILIS, DIABETIC - PSEUDOPARESIS and BRAIN TUMOR.= - - -=Case 64.= A large and imposing person, Calvin Hall, 55, had been -employed as a doorkeeper and guard, in which position he was on duty for -12 to 14 hours daily. Eventually, however, he had begun to have a good -deal of pain in the legs and a few months before observation, one day, -his legs gave way and he fell to the floor. There was, however, no loss -of consciousness, and he was carried to a general hospital. The result -of an examination there was that his family was informed that he had -some nervous trouble. - -Hall now began to be melancholy and wept a good deal. His appetite and -sleep remained intact. He felt too weak to walk. At the end of about a -year, he began to improve and again became able to do a little light -work. About a month before coming to the Psychopathic Hospital, about -two years after the onset of symptoms, Hall suddenly began to talk -excessively, in a rambling and rather senseless way. A fortnight later, -he began to suffer from insomnia and restlessness. - -Some medical facts were available: It seems that at 25 years this -patient had become infected with syphilis though there had never been -any secondary signs. He was married four years later but there had not -been any children. Moreover, for four years past, the patient had been -treated for glycosuria. - -Upon admission, the patient’s sensorium was clear, but his orientation -was only partial. He could give a fair account of his life, but it -appeared that his memory was somewhat impaired. There were auditory -hallucinations (voices of relatives). He often mistook the identity of -persons about him. He talked in a grandiose fashion of his great -strength and especially of a God-given power to read minds. His flow of -thought was rapid, rambling, circumstantial, and with traces of -irrelevance. He was rather continuously busy and at times restive. There -was a good deal of emotional agitation and apprehensiveness, and again -the patient would become suspicious and tearful. - -=Physically=, there was a discharging sinus connected with the right -humerus, close to the elbow. The pupils, though equal and regular, were -sluggish in reaction to light. The knee-jerks and ankle-jerks were -absent. There was no Romberg sign but there was some swaying in the -Romberg position. There was a moderate ataxia in walking. Glycosuria to -a moderate degree was determined. There were no casts or albumin in the -urine. The W. R. of the blood and of the spinal fluid was negative. The -albumin of the fluid, however, was considerably increased. X-ray -examination of the skull yielded a suggestion of absorption of the -posterior clinoid processes of the sella turcica. The X-ray examination -of the arm in the region of the sinus showed a chronic osteomyelitis, -possibly syphilitic (or diabetic?). - -The diagnostic problems in the case of Calvin Hall are extremely -intricate. There are clinical suggestions of general paresis, not -confirmed by the laboratory findings. - - 1. Are we dealing with a case of diabetic pseudoparesis? Is the pain - in the legs of like origin, and has a neuritic process led to the - absence of the knee-jerks? The Allen treatment appears to have had - no beneficial result in this case. - - 2. Is there a tumor of the sella region, which could account for the - mental symptoms and the glycosuria? The spinal fluid albumin might - be regarded as consistent with a variety of psychoses, including - that of brain tumor. We have to remember the definite history of - infection, the sterile marriage and the possibly syphilitic - osteomyelitis. - - - =DIABETES AND NEUROSYPHILIS, relations?= - - -=Case 65.= Donald Barrie, a man of 61, diabetic for several years, had -begun to worry about the diabetes, feeling that he was about to die, and -had gone so far as to make several threats of suicide. Hence he was -brought to the Psychopathic Hospital for observation. - -Barrie was rather well developed and nourished, although he looked far -older than he was. There was a marked arcus senilis; the skin was dry -and rough; the radial and other accessible vessels were markedly -sclerosed; abdomen obese; right testicle very low with thickened and -hard epididymis. - -=Neurologically= there was little abnormal to discover. The pupils were -irregular; both reacted fairly well to light. There was a slight tremor -of the extended hands, and still less of the tongue. The voice was -slightly thick and the patient stumbled somewhat on test phrases. Urine: -specific gravity, 1029; sugar; no acetone; no diacetic acid. Sugar 2 to -11 grams for 24 hours on ordinary diet. It proved impossible to get the -patient sugar-free, either by cutting down the carbohydrates or by using -the Allen method. - -=Mentally=, the depression with reiteration of wrong-doing and -self-accusation because of the contraction of syphilis, were the -striking features. There was, to be sure, a slight imperfection of -memory for remote events; memory for recent events and knowledge of -current events was very poor. Barrie claimed that his condition was -entirely hopeless, that his memory was exceedingly bad, and that he was -no longer capable of supporting his family. - - 1. What shall be said as to diagnosis in a man of 61 with - glycosuria, depression, amnesia, sluggish pupil, slight tremor, - slight speech defect, and a history of syphilis? The W. R. of the - serum proved positive, and also the W. R. of the spinal fluid. The - gold sol reaction of the fluid was of the syphilitic type. There - were 112 cells per cmm., there was an excess of albumin, and a - large amount of globulin. Accordingly, the diagnosis of PARETIC - NEUROSYPHILIS (“general paresis”), especially in view of the - laboratory findings, seems necessary. - - 2. What is the cause of the glycosuria? First: possibly it has no - relation with the syphilis; secondly: it may possibly be due to a - syphilitic involvement of the pancreas; thirdly: it is barely - possible that it is due to syphilitic disease of the fourth - ventricle or of the base of the brain, involving the pituitary - region. Perhaps our case is too complex for analysis. At all - events, the case brings up the possibility of a syphilitic - glycosuria. - - 3. Can the diabetes in the case of Barrie be explained as - syphilitic? Warthin of Ann Arbor has recently described somewhat - remarkable spirochete findings in his autopsy material. The order - of organic infection according to frequency is: aorta, heart, - testis, adrenal, pancreas, nervous system, liver, and spleen. - Warthin has called attention to the relation of pancreatitis and - spirochetosis to diabetes in a recent review[13] of findings in 41 - autopsied cases from the University Hospital in Michigan. Warthin - found active luetic lesions in the pancreas in 6 cases. - - - =Hemianopsia in a case of neurosyphilis.= - - -=Case 66.= Lawrence Washington, a colored cabman, 58 years of age, began -to forget addresses given him by his fares. Moreover, he could no longer -see as well as before, especially on looking toward the right side. He -himself states that the visual trouble dated back as long ago as his -39th year, at which time he had a terrific pain in both temples, leading -back from the eyes. Washington thought that his vision had been getting -slowly but steadily worse ever since. - -We got the impression that the amnesia claimed by Washington was more or -less subjective and he was found to be well informed. This association -of amnesia and impairment of vision naturally suggests syphilis. The -patient himself stated that he had had a chancre at the age of 18. - -We found the W. R. of the serum to be appropriately positive. The W. R. -of the spinal fluid was also positive though weakly so. There was an -excess of albumin; globulin appeared in large amount; the gold sol -reaction was of the syphilitic type; there were 186 cells in the spinal -fluid. - -Is this case one of paresis or of some other form of cerebrospinal -syphilis? Let us consider the data of the =physical examination=. On the -whole, the patient was well preserved. There was a slight radial -arteriosclerosis, but on the whole the cardiovascular system was almost -negative. The blood pressure was 100 systolic, 65 diastolic. -=Neurologically= the visual field of the left eye was somewhat limited, -and there was a temporal hemianopsia of the right eye. The -ophthalmoscopic examination showed a disseminated choroiditis on both -sides. The right pupil failed to react to light. The left pupil reacted -slowly. Both pupils reacted properly to accommodation. - -The knee-jerks could be obtained only on reinforcement, and when -obtained, the right was apparently more active than the left. The left -Achilles was absent; the right present. There were no other abnormal -reflexes. - -The motility of the facial muscles was somewhat impaired. -Finger-to-finger and finger-to-nose tests were rather poorly done. The -muscle sense was good; there was no swaying in Romberg position; and -there was no speech defect. - -We are unable to decide whether the case is one of the =parenchymatous= -type (paretic) or of the =meningovascular= type of =neurosyphilis=. It -is certainly rather unusual to find hemianopsia in a paretic. - -We have been unable to get definite results from the treatment of this -case, since the patient would not return for months after getting an -injection or two of salvarsan, on the ground that he was improved enough -and did not require further treatment. - - 1. What conclusion can be drawn from the 186 cells per cmm. in the - spinal fluid? Ordinarily this finding would indicate an active - process. Some writers have claimed that a cell count running above - 100 per cmm. was an indicator of diffuse non-paretic - neurosyphilis. It does not appear that this claim has been - substantiated. It is remarkable that this case shows an interval - of 40 years between infection and the occurrence of definite - clinical symptoms. With respect to the cell count, both in - untreated and in treated cases, the following conclusions from a - recent article (Solomon and Koefod)[14] are in point: - - 1. The number of cells found in the fluid of untreated cases - offers no definite information of prognostic value. - - 2. One is not justified in drawing any conclusions as to whether - the case is cerebrospinal syphilis or general paresis, nor the - time the process has been active, nor the severity of it, from the - cell count. - - 3. The cell count may vary greatly from month to month, or when - the interval is but several days, while at other times it may - remain very nearly the same after an interval of months. - - 4. Cases showing natural remissions may show no reduction in the - cell count, or other spinal fluid findings. - - 5. Cases treated with salvarsan, either intraspinously or - intravenously, tend to show a more or less rapid fall in the cell - count. This count will, as a rule, remain low during treatment, - but is likely to rise when treatment has been discontinued, but - may rise during treatment after having first fallen. - - 6. Cases may show remissions during treatment and still have a - pleocytosis. - - 7. Treated cases having the cell count fall to normal may at the - same time become very much worse and develop more marked paralytic - symptoms. - - 8. In general paresis the cell count in no way parallels the other - spinal fluid findings. - - 9. In cases in which the other tests show an improvement, for - instance cerebrospinal syphilis, the cell count also readily and - early drops to normal. At times it may drop to normal before other - spinal fluid tests become negative; again it may be last to reach - normal. - - 10. The change in cell count seen in syphilitic disease untreated - is also found in non-syphilitic diseases, as brain tumor. - - 11. The cell count offers nothing of prognostic importance in - syphilis of the nervous system unless accompanied by improvement - of the other laboratory signs. - - 12. The cell count is not an index to the predominance of - irritative or degenerative changes. - - - =Case of CEREBRAL MALARIA and SYPHILIS: simulation of PARETIC - NEUROSYPHILIS (“general paresis”).= - - -=Case 67.= Joseph Temple, 45, who had been a sea-going steamboat -steward, was brought to the hospital in a semi-stupor. He was entirely -uncoöperative, often resistive, attempting to bite the physician’s -fingers, and for the most part lying curled up. He was incontinent and -tube-fed. This phase, it seems, had begun the night before entrance to -the hospital. Twenty-four hours later, an extraordinary change was -noted. Temple became alert and attended to his wants, began to eat well, -and began to behave as normally as probably he ever behaved. - -He was now able to give a coherent history. It was now January. In the -previous September, he had left for Mexico; he was returning when he -suddenly fell to the deck, unconscious. After this fall, he had not been -well, having had chills and fever. At the Marine Hospital, he had been -diagnosed as suffering from malaria, and was given quinine. He had been -delirious a short time in the hospital, not being able to recognize his -wife, who called. He shortly improved so that his wife was able to take -him home. Nevertheless, headache, gastric distress, and intermittent -vomiting continued. A spell of confusion took place, two days before -admission. The patient tossed about, moaned, and failed to recognize -anyone. Malaria of the æstivo-autumnal type was demonstrated in the -hospital. The temperature always remained at normal. He was somewhat -emaciated and pale. The pupils were small, somewhat unequal, and reacted -though poorly to light and distance. The tendon reflexes were lively. - -The W. R. of the serum was positive, and information from the patient’s -physician runs to the effect that there was a syphilitic infection some -seven or eight years ago, followed by secondary symptoms, but the -patient had refused to take any protracted treatment. The spinal fluid -examination was practically negative. - -=Mentally=, the patient was euphoric, expansive, boastful, and showed a -marked emotional instability and considerable memory defect. - - 1. Can the diagnosis of general paresis be made in Joseph Temple? - Certainly the acute confusion and the syncope are consistent - enough with the diagnosis, yet the severe malaria makes it seem - likely that the phenomena were due to a cerebral attack of - malaria, and such occurrences are found in the æstivo-autumnal - form of malaria. Yet malaria would hardly explain the euphoria, - memory defect, and the pupillary findings, to say nothing of the - irritability and the active tendon reflexes. Even if we regard the - active tendon reflexes and the irritability as malarial, the other - phenomena remain outstanding as exceedingly suspicious of paresis. - - On the other hand, if we try to support forcibly the diagnosis of - general paresis, we are hardly able to explain the negative - findings in the spinal fluid. - - In point of fact, a study of the patient’s past life revealed a - story that the mental traits of euphoria, irritability, and memory - defect had been characteristic of the patient for many years. In - fact, there is some question whether the patient is not really to - be regarded as a moron of high grade. - - Upon this basis, if we regard the confusional phenomena as - malarial and the persistent mental phenomena as characteristic of - a moron and somewhat exaggerated by the disease, we have merely to - explain the suggestive pupils. As to these, it must be remembered - that though they reacted poorly to light, still they reacted - somewhat, so it is not a question of explaining an - Argyll-Robertson pupil, but only an impaired pupillary reaction. - Of course, some workers are of the opinion that pupillary changes, - perhaps even the Argyll-Robertson pupils, may occur in syphilitic - cases that are not neurosyphilitic, or at all events are not - victims of central neurosyphilis. Finally, we must remember that - there are cases of neurosyphilis of a vascular type which yield - negative spinal fluids. The case leaves many questions unanswered. - - - =Can paretic and non-paretic neurosyphilis be differentiated by - means of the gold sol reaction? The gold sol reaction in this case - was an extremely mild one and would not at all have warranted the - diagnosis GENERAL PARESIS, yet the discovery of a heavy meningeal - exudate including an unusually heavy deposit of plasma cells even in - the spinal pia mater will perhaps warrant us in making a final - retrospective diagnosis of paretic neurosyphilis. Autopsy.= - - -=Case 68.= We would like to give the full effect of our surprise at the -outcome of the case of Margaret O’Brien, a school-teacher, 26 years of -age. To be sure, Miss O’Brien developed symptoms at 22 or 23 which we -can now explain consistently with the outcome of the case; for at that -time, she began to complain of severe pain in the head, especially in -the forehead and temples, and also became nervous, unable to remain -quiet, and given to insomnia. She was markedly depressed at the time and -would refuse to talk at times. However, only the headache in this -prodromal period could be regarded as particularly suggestive of -syphilis, and headache in an over-worked school-teacher is not uncommon. - -In fact, the picture presented by the patient was one of catatonic -dementia praecox. The patient was admitted to the hospital after a -sudden onset of excitement. At first she was very restless, continually -looking about and getting up and walking away from the examiner, giving -the impression of understanding all questions but preserving an air of -indifference. A few days later, the patient was gotten to answer more -coöperatively. She remarked that the hospital was heaven although in -Boston; that it was summer time (correct) and that her memory was -greatly impaired. The physician was a messenger of God (delusion later -corrected). The patient had not done God’s will; her breath was leaving -her; God’s voice was heard from time to time, and Miss O’Brien had heard -it for a long time. God tells her to do His will. However, as Miss -O’Brien remarked, “I must think all this nonsense, turning against God.” - -The patient frequently attitudinized and would remain in an apparently -catatonic condition for many minutes. For the most part, she was -resistive and mute and non-coöperative as to examination. From time to -time, she made impulsive suicidal attempts. So far as a somewhat -inadequate =physical examination= was concerned, nothing abnormal could -be made out; in particular, the pupils reacted normally to light and -were otherwise normal. The routine W. R. of the blood serum, however, -returned positive, and in accordance with the policy of the Psychopathic -Hospital, the patient was subjected to a lumbar puncture. The lumbar -puncture yielded a positive W. R., 109 cells per cmm., a positive -globulin and a considerable excess of albumin, and an exceedingly mild -gold reaction—syphilitic type. - -Ten days after admission, the patient had a convulsion. She never -regained consciousness, continued to have convulsions for a few hours, -and died, apparently from paralysis of respiration. The heart continued -to beat for a short period after respiration ceased. The =autopsy= was -consistent with the diagnosis which had been rendered after the -surprising results of the W. R. in the blood and the laboratory findings -in the spinal fluid had been learned. There was a generalized -encephalitis with congestion of all the smaller cerebral vessels and -petechial areas in the meninges and upon the cortical surfaces. We -regard the case as one of syphilitic encephalitis. - -The brain weighed 1265 grams, indicating a loss of 79 grams by Tigges’ -formula (8 times the body length in centimetres). The pia mater was, in -the gross, quite normal within the cranium; nor were any cells found in -a smear from this pia mater; but the pia mater over the spinal cord was -visibly edematous, and a smear from the spinal pia mater showed great -numbers of lymphocytes and especially of plasma cells—a finding which -was confirmed in stained section, by which a remarkable display of -plasma cells was found plastered somewhat generally over the entire pia -mater of certain segments. The brain substance was softer than normal, -but displayed no differences of consistence. The stripping of the pia -mater of the temporal lobes on both sides yielded the so-called -“decortication” (that is, the adhesion of small bits of brain substance -to the pia mater). The optic nerves were somewhat thinner than normal. -No other gross lesions of the brain were found. - -The dura mater, although dense and injected, was not otherwise abnormal. -There was an early visible sclerosis of the middle meningeal arteries, -more marked on the left side. - -The cause of death, so far as the autopsy revealed it, was bronchial -pneumonia. There was a diffuse nephritis. - - 1. Are the hallucinations in the case of O’Brien characteristic? - Hallucinations are regarded as playing a minor rôle in general - paresis. In fact, earlier workers sometimes denied that - hallucinations occurred at all, and this denial has been made once - more of late by Plaut,[15] but Kraepelin quotes Obersteiner as - observing hallucinations in 10%, and regards that figure as - approximately corresponding with his own experience. Junius and - Arndt are cited as finding 17% of their cases hallucinated. - Auditory hallucinations are somewhat more frequent than those of - vision (alcoholic psychosis must be considered). The visual - hallucinations of paresis are thought by Kraepelin to be related - with atrophy of the optic nerves, and he states that they occur by - preference in patients having such atrophy. Hallucinations though - not common are more frequent in non-paretic neurosyphilis than in - paretic neurosyphilis. - - 2. What was the cause of death in Margaret O’Brien? The autopsy, as - above stated, indicated pneumonia. In point of fact, this patient - developed convulsions and ceased respiration, the heart continuing - to beat for some time after respiration had ceased. It may be that - the death should be counted as one of neurosyphilitic seizure. - - - =Tonsillar abscess associated with neurosyphilis (Lues Maligna?).= - - -=Case 69.= Frank Mason, 49 years, a rectifier of spirits, was admitted -to the Psychopathic Hospital in a tremulous, mentally confused, -depressed, and unhappy state. He was particularly concerned because he -could not give an accurate account of his past life and because he found -that he was continually contradicting himself. - -Superficial examination shortly discovered the pupils to be much -contracted, irregular, and non-reactive either to light or distance. -Although these pupils showed more than the Argyll-Robertson phenomenon, -yet the suspicion of syphilis was important. - -Throat examination showed a large area of ulceration involving the whole -of the right tonsil and extending even to the left side of the median -line so that the whole of the faucial pillar was involved. In the midst -of this ulcerative area was a mass of purulent necrotic tissue, about -which the edges of the ulcer stood out sharply. There was, however, very -little acute reaction about the margin of the area. - -The association of pupillary changes (especially stiffness to light), -what looked like tonsillar gumma, and mental disorder (including memory -disturbance) heightened the impression of syphilis. - -However, the remainder of the examination was not especially -confirmatory of the diagnosis. The man was well developed and obese, -with a slightly enlarged heart, with sounds of poor quality and the -aortic second sound accentuated. The systolic blood pressure was 130; -the diastolic, 90. There was no disorder of reflexes except that the arm -reflexes were very lively. - -After a time, a few facts concerning the patient’s life became -available. Although a rectifier of spirits, Mason could not be found to -have over-indulged in alcohol. It appears that some five months before -his admission to the hospital, a wisdom tooth had been extracted. About -four months before admission, the ulceration of the faucial pillar had -begun, and this ulceration was immediately laid to infection from the -wisdom tooth cavity. Mason then had to discontinue work and a depression -followed. But the account of this depression led us to think that he was -a victim more of natural sadness than psychopathic depression. There was -much worry and insomnia. To meet the insomnia, large amounts of -hypnotics were administered. The sequence of these hypnotics was a -tremendous disturbance and continual crying out by the patient. In fact, -Mason became so excited that he was removed to the Psychopathic Hospital -for temporary care in the condition above mentioned. - -We naturally awaited the outcome of the serum W. R. The return was -negative. However, the typical position of the ulcerative lesion and the -non-reacting pupils,—to say nothing of the mental symptoms and the -associated tremors, with incoördination (this incoördination was -non-characteristic and apparently due largely to the tremor),—led to -lumbar puncture. - -The spinal fluid yielded a weakly positive W. R. There was a slight -positive albumin, the globulin test was slightly positive, there were 14 -cells per cmm., and the gold sol reaction was of the syphilitic type. We -were, then, probably entitled to conclude that syphilis was active not -only in the body at large but also in the nervous system. Looking back -upon the case, we considered that large doses of morphine and hyoscyamus -might well have produced the marked mental confusion and possibly the -tremors that characterized Mason on his arrival at the hospital. - -Improvement followed after a few days of rest; the confusion disappeared -and the tremors diminished; the pupils returned to their normal size and -reaction; depression persisted, and the patient was very properly much -concerned about the tonsillar lesion. However, further improvement did -not take place under antisyphilitic treatment and patient died after -several weeks from what was believed to be an embolus from the tonsil. - - 1. What was the true interpretation of Frank Mason’s pupillary - changes? They were probably due to the opiates, despite the fact - that, taken in association with the gummatous lesion of the - faucial pillar, we had regarded them as possibly syphilitic. - - 2. How shall the negative serum W. R. be explained? Such a reaction - is consistent with the diagnosis _gumma_. It is, however, a little - surprising that with active neurosyphilis and a relatively active - non-nervous syphilitic lesion like that in this case, the serum W. - R. should have been negative. Possibly a repetition of the test at - various times would have shown a positive serum W. R. In any - event, the fluid reaction was positive. - - 3. Could the tonsillar ulceration be due to dental infection? The - chances are against this on account of the interval (2 months) - between extraction of the wisdom tooth and the ulceration, which - itself seems to be of a tertiary syphilitic nature. In point of - fact, the patient admitted a syphilitic infection 21 years - previously namely, at 28 years of age. At that time he took large - quantities of mercury and potassium iodid by mouth. - - 4. Relation of the case of Frank Mason to the so-called _lues - maligna_? The case closely resembled the cases reported by Bly. - Frank Mason showed great destruction of tissue, toxemia, failure - to react to antisyphilitic treatment. In both of Bly’s cases, the - tonsil was the starting point of the illness; and in both cases - there was a trauma of the tonsil or peri-tonsillar structures - (tonsillectomy and application of caustic). In our case there not - only had been extraction of a wisdom tooth, but the tonsil had - been cauterized. - - - =Neurosyphilis versus multiple sclerosis.= - - -=Case 70.= Annie Kelly is a young Irish woman, 21 years of age, who was -perfectly well until three months before her admission to the -Psychopathic Hospital, when suddenly one evening she became very dizzy. -This was followed by a chill and vomiting. The next day she had a sore -throat but was able to be about and do her work. The dizziness, however, -continued and she began to feel rather queer. Gradually it became -difficult for her to walk on account of staggering. - -A little later she noticed a weakness of the left side, involving face, -arm, and leg; then she began to find it difficult to talk. Finally the -right leg became weak, making walking practically impossible. All these -symptoms grew worse and the dizziness increased. At times her vision -would be blurred; there were somewhat frequent attacks of diplopia. -Finally she had to take to her bed, and at last she lost control of her -sphincters. - -At no time did she suffer any pain. She was taken to a hospital, and -after a time improved somewhat; but she was told she had a brain tumor -and had better be in a large city, where she could have surgical aid if -this became necessary; consequently, she was brought from Montana to -Boston. - -On admission to the hospital, the examination disclosed no important -symptoms outside of the nervous and locomotor systems. She was unable to -walk unless assisted. The pupils were large but reacted well to both -light and accommodation, were equal in size, and regular. Slight -nystagmus was present; there was no ptosis or strabismus; vision in the -left eye was poor. The other cranial nerves showed no involvement. The -tendon reflexes were all present and very lively; Babinski, Gordon, and -Oppenheim signs were present on either side. The ataxia was marked, -especially of the lower arms, and she had some difficulty in the -alignment of the fingers. The sense of position of the limbs was very -poor. There was some tremor, which was not of the intention type. The -writing showed some incoördination. The speech showed nothing abnormal. -=Mental examination= disclosed nothing of note objectively, but patient -stated she could not think so clearly as she could formerly. - -The =diagnosis= would seem to lie between brain tumor,—which had been -suggested to the patient by her physician,—multiple sclerosis, and -neurosyphilis. The numerous neurological symptoms without any definite -evidence of intracranial pressure were sufficient to rule out for the -moment the consideration of brain tumor. The syndrome of multiple -sclerosis is not complete, but the race, age, and onset, with the -increasing and decreasing intensity of symptoms are very suggestive of -this diagnosis. The symptoms, of course, are all consistent with -neurosyphilis also, and while the patient denied any knowledge of -syphilitic involvement, the examination of the blood and spinal fluid -was made. The W. R. was negative in both the blood serum and spinal -fluid. Further examination of the spinal fluid showed presence of -globulin and an increase in the albumin content, 43 cells per cmm. and a -“paretic” type of gold sol reaction. With the negative W. R. of both -blood serum and spinal fluid, and with so much in favor of MULTIPLE -SCLEROSIS, this diagnosis was made. - - 1. What is the relation of multiple sclerosis to syphilis? There is - no definite relationship between multiple sclerosis and - syphilis,—that is, multiple sclerosis is not a syphilitic disease; - but the complete syndrome of multiple sclerosis is often given by - a syphilitic involvement of the central nervous system (see case - Lauder, 71). - - 2. Is the spinal fluid finding in this case consistent with multiple - sclerosis? According to Nonne, about 19% of the cases of multiple - sclerosis show globulin and pleocytosis in the spinal fluid. As a - rule, the number of cells ranges between 10 and 20 per cmm. and - the globulin is not present in large amounts. In this case, the - amount of globulin, which was given as 2+, is only a moderate - amount,—less than is usually found in cases of general paresis. - There are not very many cases of multiple sclerosis in the - literature in which a gold sol reaction has been performed, but in - the majority of those tested, the reaction is reported as mild. - However, cases of multiple sclerosis giving a typical paretic - curve have been described by a number of observers, among whom may - be mentioned Kaplan and Solomon. - - 3. How frequently is it necessary to make a differential diagnosis - between multiple sclerosis and neurosyphilis? Before the days of - the W. R. this differentiation was much more difficult than at - present. But we, however, still have to face a not very rare - difficulty in separating the two conditions. Syphilis is prone to - cause small localized lesions in the nervous system. The changes - in the patient’s condition, with improvements and regressions are - equally characteristic of both diseases. How closely the - symptomatology of neurosyphilis may simulate that of typical - multiple sclerosis is shown in the next case (Lauder, 71). When - the sclerotic area of multiple sclerosis occurs in appropriate - parts of the cerebrum, symptoms of mental disturbances will occur. - In its histological picture multiple sclerosis is at times highly - suggestive of syphilis, even showing mononucleosis and meningitis. - - - =Optic atrophy; nystagmus; spasticity; intention tremor. Diagnosis: - ?= - - -=Case 71.= James Lauder began to lose his eyesight at 32 years, and was -shortly determined to be suffering from primary optic atrophy. In the -course of a year, he had become completely blind. No mental symptoms had -developed. - -=Physically=, Lauder was in very good condition. =Neurologically=, there -was a complete optic atrophy with paralysis of the internal rectus -muscle, marked nystagmus, and absent pupillary reactions. All the tendon -reflexes were exceedingly lively, though the right arm reflexes were -more lively than the left, and the left leg reflexes more lively than -the right. There was an ankle clonus on both sides. The abdominal and -cremasteric reflexes were lively. There was a slight intention tremor. -There was, however, no ataxia and no speech defect. - -=Diagnosis=: The nystagmus, optic atrophy, and the reflex disorder -suggested multiple sclerosis, although the liveliness of the superficial -reflexes, especially the abdominal reflexes, was a point somewhat -against any advanced degree of multiple sclerosis. It would appear that -the absence of pupillary reaction to accommodation is also rather -unusual in multiple sclerosis. - -The serum and spinal fluid W. R.’s proved positive. There were 25 cells -per cmm., albumin was in excess, and there was a positive globulin -reaction. - - 1. What is the significance of optic atrophy and other optic changes - with respect to neurosyphilis? Canavan, from our laboratory, has - reported that she found that 40 of 58 unselected cases of mental - disease exhibited obvious and undeniably important changes in the - optic nerve. She found that optic nerve changes were even more - frequent than chronic spinal cord changes as detectable by the - same method (Weigert myelin sheath method); there were only 34 of - the 58 cases which showed chronic spinal cord changes. Eighteen - cases very probably syphilitic (although the clinical evidence was - not in all cases supported by the W. R.) failed to show optic - nerve changes in but three instances. The 15 syphilitic cases that - did show optic nerve changes showed them in but one eye in three - cases, in both eyes in 12 cases. Canavan incidentally demonstrated - a spirochetosis in the pial sheath of the optic nerve in a case of - neurosyphilis, possibly paretic. - - 2. What is the frequency of eye changes in neurosyphilis? Posey and - Spiller (“The Eye and the Nervous System,” 1906) quote Kéraval as - finding 42 instances of fundus change in 51 cases of paresis. - Clifford Allbutt found 41 cases of atrophy in 53 of paresis; other - authors have found far fewer. Optic atrophy sufficiently marked to - cause blindness is relatively rare in paresis. Compare table of - eye changes from Joffroy under Case Falvey (55). - - As for optic atrophy in tabes, Posey and Spiller record statistics - as so various as to be on the whole unsatisfactory. The highest - percentages found appear to be those of Mott, 80%, and Gross, 88%. - It is evident that the standards for measuring optic atrophy must - differ very much. - - - =Atypical case of neurosyphilis. Picture of Huntington’s chorea.= - - -=Case 72.= Margaret Green, 28, was received at Danvers State Hospital in -an excited and frightened state. She was very talkative and said that -she was being bitten by snakes and serpents. She thought every one -approaching her was the devil, and sprinkled what she called “holy -water” about her for protection. It was clear that she was hallucinated. -She heard her child crying, and she saw a woman carrying it away. - -After a few weeks, Mrs. Green grew quiet and more rational except for a -few spells of violence and noise; she gave the impression of a rather -pleasant and agreeable, though somewhat demented, patient. Physically, -beyond a tremor of fingers and tongue and lively knee-jerks and some -evidence of enlargement of the heart, there was nothing to be found. - -Margaret Green is still in the Danvers Hospital, being now 48 years of -age. During the twenty years, she has presented,—besides the mental -picture of impairment of memory—occasional spells of confusion, a -variety of delusions based, at least in part, upon auditory and vivid -visual hallucinations, a certain irritability and psychomotor -excitement, and a picture of Huntington’s chorea. The diagnosis of -Huntington’s chorea has always been in doubt by reason of the lack of -any evidences of hereditary taint; it has, however, not been possible to -secure a properly intensive account of her relatives. - -It appears that the choreic movements were first observed—in the -hospital at least—about 16 years ago. The patient has always been -decidedly mixed upon dates. From internal evidence derived from her -obviously in part erroneous statements, it may be that the chorea began -at the age of 23. It appears that she had been often termed a victim of -St. Vitus’ dance, and had had to leave her work in the mill on account -of the disease. From one source of information, it would appear that the -patient began to have what was called St. Vitus’ dance when she was 14 -or 15 years of age; so far as this informant knew, no other member of -the family had had the affliction. - -The first movements observed in the hospital were irregular, jerking -movements, more marked in the left arm but also occurring in the other -extremities, as well as in the face, wherein were produced peculiar -grimaces. The twitching movements would become decidedly worse during -spells of irritability. Observation in the patient’s early thirties left -the question in doubt whether the left pupil reacted to light or not. In -1904, when the patient was 36, both pupils failed to react to light -either directly or consensually. At this time, the jerky movements -continued, especially in the left hand and forearm, the tongue was -tremulous, test phrases were poorly pronounced, the knee-jerks were -exaggerated (especially the left), and both wrist-jerks were -exaggerated. The systematic examination, however, revealed no other -neurological disorder. Within a year, slight spurious ankle clonus -developed on both sides; the eyes, especially the left, gave the -appearance of developing cataracts. A slight consensual light reaction -was demonstrable on the right side, but all light reactions were absent -in the left eye. - -At the age of 42, the patient was still disoriented for time, place, and -persons and subject to a deep amnesia; was tidy, tranquil, and of a -pleasant demeanor, but many of her muscles were in continual motion. -There were chewing movements and both hands and feet were rarely still. -There were no longer any spells of irritability or violence; and once -when found crying on the piazza, Mrs. Green, on being asked the reason, -replied that a gray cat had come and looked at her so hard it made her -cry. There were other crying spells at times for equally good reasons, -or for no reason. - -More recently, the patient has become fairly well oriented for time and -place, and has acquired a fairly good insight into her condition and a -good memory for past events. She has had occasionally auditory -hallucinations, as of water running. In 1914, it was reported that the -pupils reacted to light, and the rest of the systematic neurological -examination was negative except that the knee-jerks were exaggerated; -and a re-examination in 1916 showed the pupils still reacted to light. -At present, the patient is disoriented for time, stating that her age is -about 25; she is no longer subject to auditory hallucinations; she has a -marked difficulty in enunciation, emphasized by the lack of teeth and in -part due to continual movements of the tongue; the movements appear to -be part of a generalized chorea. - -In a systematic review of the Wassermann findings in the hospital -population, the blood of Margaret Green was examined and found to be -positive. Lumbar puncture forthwith performed showed a positive W. R. in -the fluid; there was a positive globulin and an excess of albumin; the -gold sol was characteristic of paresis; there were, however, but three -cells per cmm. - - 1. Are the choreiform movements related to the demonstrable syphilis - of the nervous system? Neither the fluid W. R. nor the gold sol - reaction should be regarded as necessarily an indicator of tissue - loss. The fluid W. R. is commonly thought to signify merely that - the fluid contains substances which are somehow due to the - presence of spirochetes in some region pretty closely related with - the fluid. The gold sol reaction, although well established to be - characteristic of neurosyphilis, is perhaps not so strong an - evidence of the existence of spirochetes in the region from which - fluid constituents are derived. There is no pleocytosis. However, - the positive globulin test and the excess of albumin do indicate a - certain amount of destructive process somewhere in the neural - tissues. Are we to suppose that these substances have been - continually found during the course of this disease? This question - cannot be answered with the data in hand, and we can only suspect - that these positive tests for albumin and globulin are an effect - of tissue destruction caused by neurosyphilis. It must be admitted - that the argument here is a little tenuous. The lesson is plain: - that in the present stage of our knowledge the W. R. should not be - omitted even in cases which present a fairly convincing picture of - some well-known entity. Thus, a disease, which looks like - Huntington’s chorea, as well as a disease suggestive of multiple - sclerosis, requires investigation by the methods of the - syphilographer. - - 2. How shall we explain the changes in pupillary reaction in this - case? They cannot yet be explained. A few observers have reported - changes in pupillary reflexes in the direction of normality. In - our experience such changes have not been noted. It cannot be too - strongly emphasized that it is very easy to make errors in judging - pupillary reaction if care is not used. For instance, if the - patient is accommodating for near vision, light will probably not - cause contraction. A frequent cause of error in testing the light - reflex arises from using a weak electric light. An electric - flash-light is much less efficient than daylight. Probably the - most satisfactory method is to take the patient to a window, ask - him to look at a distant object, shade the eye with the hand, - remove hand, and observe. - - 3. What is the chief triad of symptoms in Huntington’s chorea? (1) - Choreiform movements associated with (2) progressive mental - enfeeblement, (3) occurring in a patient whose family history - shows a similar condition in a preceding generation. - - - =Differential diagnosis between NEUROSYPHILIS and SENILE - ARTERIOSCLEROTIC PSYCHOSIS.= - - -=Case 73.= Marcus Chatterton was a retired sea captain, 75 years of age. -At the age of 71, he had had a seizure with a slight right hemiplegia -and inability to talk. He had been slightly confused for a short time -but had rapidly recovered. During the intervening four years, there had -been three similar attacks, and the last one had caused him to come to -the hospital. He was, in fact, confused upon admission but had become -perfectly clear by the next day. There was a considerable memory defect, -which the patient himself did not entirely appreciate. Possibly his -judgment had been deteriorating slightly. He had been irritable of late -and sometimes sleepless. - -=Physical examination= showed a rather well preserved man with but -slight senile changes. The pupils were equal and reacted readily to -light and accommodation. There was no sensory disorder and no -disturbance of coördination. There were no tremors. The systolic blood -pressure was 205, the diastolic 135. The arteries were sclerotic upon -palpation. A sufficient diagnosis would have seemed to be -arteriosclerosis, and the hypothesis of syphilis would hardly have been -raised off-hand by most practitioners. The W. R. of the serum was -negative. What led to lumbar puncture in this case was the fact that the -sea captain’s wife had died 15 years before of general paresis. The -lumbar puncture was rewarding since the W. R. was positive. There was an -increase of albumin and globulin, a “paretic” type of gold sol reaction, -and 56 cells per cmm. - -Accordingly, we must regard the condition as one of neurosyphilis. -Perhaps the arteriosclerosis was of syphilitic origin. If this is a case -of general paresis as we suppose, it is one of very long-standing -syphilis. - - 1. Do delusions of grandeur in the senile period suggest syphilis? - Not necessarily; it appears that there is a small group of senile - cases which might be called cases of senile pseudoparesis in which - extravagant delusions of grandeur are entertained, and in which - frontal atrophy is found although entirely without evidence of - chronic inflammation. It has not been proved that these cases are - of syphilitic origin. It is suggestive that the site of the most - extensive lesion is precisely the site of the most extensive - lesion classically found in paretic neurosyphilis, viz., in the - frontal regions. - - 2. Is neurosyphilis frequently found in both mates? It can hardly be - said that this is a usual finding. However, it is far from rare, - and it occurs frequently enough to be used in support of the - theory that there is a special strain of spirochete that has a - predilection for nervous tissue. It must be remembered, however, - that the wives of syphilitics are frequently infected without - being aware of it. In such cases they receive no treatment and - consequently have a larger chance of developing neurosyphilis. It - is a good rule to consider the mate of every syphilitic a - candidate for neurosyphilis. - - - =An atypical case of recurrent dazed states resembling HYSTERICAL - FUGUES. Probably an instance of NEUROSYPHILIS.= - - -=Case 74.= Abel Bachmann, a man of 40 years, remains doubtful and -perhaps belongs to the still unresolved group of mental cases due to -syphilis that cannot be placed in any of the well-known categories. -Bachmann had been found by the police, working in front of a cowbarn -without the consent or even the knowledge of the owner. Bachmann had, in -fact, spent the night in the cowbarn and was working with the idea of -paying for his night’s lodgings. The situation struck the police as so -peculiar, and Bachmann was so confused and irresponsive, that he was -brought to the Psychopathic Hospital. The afternoon of his admission, -however, he entirely cleared up and was able to give a good account of -himself. - -His story was that he had been worrying a good deal about a divorce -suit, and the morning of his episode he had awakened with peculiar -feelings. He walked from Boston to Cambridge, feeling that he was in a -strange city. He recognized the places he passed, yet they all seemed to -be changed. Upon reaching Harvard Square, he determined to return to -Boston and walked and walked, failing to reach Boston. All day he had -eaten nothing; when night fell he stole into a field and dug out -radishes. A postman stopped and said, “Hello, Bill,” which awakened him -as by an electric shock. A barn presented itself, in which he spent the -night. In the morning, the barn looked different. In fact, his entire -surroundings appeared mysterious. As he felt like working, he went to -work in front of the barn. - -It seems that in his life there had been two other episodes of a similar -nature; in fact, Bachmann had been in a state hospital for six weeks -after the first episode. The first episode had lasted a few days only, -and followed worry when he learned that the girl with whom he was in -love was married. The second attack followed the death of his mother, -whereupon he was taken to a state hospital although the total duration -of symptoms was only three days. Bachmann had had a chancre or some -other form of genital disease at 26, and had at that time been treated -with mercury. - -Except for irregular and absolutely rigid pupils, reacting neither to -light nor to accommodation, Bachmann showed no physical and especially -no neurological disease whatever. Moreover, the W. R. in the blood serum -was negative. - -As to diagnosis, one might consider hysteria, of which, however, there -are no visible stigmata. It would not appear that brain tumor would be -likely to have lasted so long as eight or nine years, even if we should -attempt to make the hypothesis of tumor cover both the non-reacting -pupils and the episodes. Bachmann was non-alcoholic, and there was no -sign of any other form of intoxication. The spinal fluid showed a -negative gold sol reaction, there were no cells in the fluid, there was -no globulin; albumin was normal. However, the W. R. was strongly -positive. - -The situation, then, in this case is that we have somewhat peculiar -psychopathic episodes, pupils rigid to light and accommodation, a -positive W. R. in the spinal fluid, and extremely little else to permit -a diagnosis. We are ignorant as to the course and pathology of such -cases. However, we cannot resist the temptation of the diagnosis of -neurosyphilis, although further classification is not ventured. - - 1. What is the significance of stiff pupil as an isolated symptom? - Nonne finds that in the end, after years of observation, the - Argyll-Robertson pupil turns out to be an advance courier of other - more functionally serious signs and symptoms of neurosyphilis. We - can confirm this experience and regard it as an established - clinical proposition that the Argyll-Robertson pupil cannot be - neglected. In this connection, refer to the case of alcoholic - pseudoparesis (Murphy, 60), and also to the case of pineal tumor - (Donald Falvey, 35). Enthusiastic reports have occasionally been - made upon apparent restoration of the true syphilitic - Argyll-Robertson pupil to normal light reaction. The difficulties - in rendering the symptomatic diagnosis of Argyll-Robertson pupil - in a given case are so great, and the chances of complication so - numerous, that we are inclined to attach little significance at - present to these claims. - - It may not be amiss to mention a somewhat humorous incident familiar - to some local neurologists. A case was reported by the interne for - a number of months as a victim of a pupil stiff to light and - accommodation, and the entirely adequate cause of this phenomenon - was actually only discovered at autopsy by the triumphant medical - examiner, who demonstrated that the patient in question was - possessed of a =glass eye=. - - - =TABETIC NEUROSYPHILIS (“tabes dorsalis”) versus PERNICIOUS ANEMIA - with spinal symptoms.= - - -=Case 75.= Mrs. Brown was a woman of 56, who for the past eight or ten -years had been complaining of trouble in her legs. As she described it, -at times her legs were so weak she could hardly stand; at other times -there was considerable pain and numbness. She has always been considered -“high strung”; that is, she had a very bad temper and lost control of -herself almost entirely when she became excited. Her legs had been -growing progressively worse, and for about a month prior to admission -she had been unable to stand or walk. She had also lost control of her -bladder. On account of her temper, it had been almost impossible to -nurse her; no nurse would stay with her because of her scolding and -fault-finding. Recently, she had been having fits of the blues. - -Her husband, who was seen before Mrs. Brown, was an old gentleman, over -70, who was chiefly remarkable from the fact that he had unequal, -irregular pupils, which reacted neither to light nor accommodation; -there was also a speech defect. - -The patient herself proved to be extremely irritable, as had been -stated,—so much so that at times it seemed almost impossible to do -anything for her. She was very querulous, constantly complaining, and -not satisfied with anything that was done. Aside from this, her =mental -examination= proved to be entirely negative; that is, there were no -psychotic symptoms. - -The systematic =physical examination= gave the following significant -findings: blood pressure, 160 systolic, 90 diastolic; no evidences, -however, of peripheral arteriosclerosis. Patient was unable to walk or -stand, and had no control over her bladder. The knee-jerks and -ankle-jerks absent on both sides; ataxia in the leg movements; loss of -sense of localization, with no tenderness over the nerve trunks; no -atrophy, paralyses, or muscular asymmetry of the parts. The vibratory -sense was maintained. Subjectively, the patient thought that the -vibratory sense differed in the legs from that in the arms. -Localization, touch, pain, heat, and cold responded to correctly. The -arms showed nothing abnormal; there was no incoördination, dysmetria, or -dysdiadochokinesis. Her pupils were equal, regular, and both reacted -normally to light and accommodation. - -=Diagnosis=: The first consideration in the case is naturally tabes -dorsalis, especially when one considers that the husband had signs which -suggested syphilis of the nervous system. The rapid onset of the acute -symptoms in this case, and the absence of the characteristic signs of -pain were at least atypical for this diagnosis, as was the absence of -any pupillary signs. Further, the W. R. was negative in the blood and -spinal fluid; there were no definite signs of inflammatory reaction as -shown by the other spinal fluid tests. These findings made a diagnosis -of tabes entirely improbable. After tabes, the most frequent cause of -the symptoms above enumerated is perhaps to be found in pernicious -anemia. Examination of the blood showed that the patient had 2,500,000 -erythrocytes per cmm. The hemoglobin by Tallquist scale was 80%. The -smear was practically negative; no blasts could be seen. Although this -picture is not a typical one for pernicious anemia, at least it is -significant in the low number of red cells to be found, and as no causes -for anemia were to be found, it seemed probable that we were dealing -with a primary anemia. The diagnosis in the case, therefore, is spinal -sclerosis of primary anemia. The mental picture was not uncharacteristic -of PERNICIOUS ANEMIA. - - 1. Could the diagnosis be rendered in this case without a lumbar - puncture? In the first place, the emaciation is not entirely - characteristic. The pupils react normally to light. Probably such - a case might well have been regarded as one of tabes dorsalis in - former days, or even at the present time, if a lumbar puncture had - not been resorted to. - - 2. Could this case possibly have been one of tabes dorsalis with - negative spinal findings? Such cases have been reported - frequently, but, unlike the present case, are apt to be of - long-standing and non-progressive, in which the active - inflammation is no longer present. The negative findings would not - be consistent with tabes, in which the symptoms are of short - duration and of sudden onset. - - 3. If the serum W. R. had been positive would the diagnosis have - been neurosyphilis? We are loath to make the diagnosis of spinal - syphilis when the spinal fluid is normal. Syphilis may produce a - marked anemia, however, and thus produce symptoms such as shown by - Mrs. Brown. It is even possible that such is the explanation of - this case, taking into consideration the suggestive findings in - the husband. However, there is insufficient evidence to make such - an hypothesis rock firm, and we do not more than suggest it. - - - =Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.= - - -=Case 76.= James Seabrook, 20 years of age, obviously showed a number of -signs of congenital syphilis. The =physical examination= disclosed an -old scar and indentation in the right mastoid region, another on the -right side of the neck, another on the posterior surface of the right -forearm, and two on the outer surface of the right upper arm. The -lesions were about the size of half a dollar. There was a saddle-shaped -nose and a perforation of the palate and uvula; there were palpable -cervical and axillary glands, small but numerous. There was a dulness in -the region of the right scapula, and slight dulness on both sides -behind. There were loud whispering and piping râles and bronchial -breathing throughout the chest, more marked on the left; there was much -coughing, and the sputum was at times blood-stained. The pupils were -irregular but reacted perfectly. The left knee-jerk was slightly more -active than the right. The W. R. in blood and fluid was negative; the -gold sol, globulin and albumin tests were negative. There were, however, -56 cells per cmm. in the fluid. - -We learned that the patient had had several spells of great excitement, -with pounding on the door and a desire to fight bystanders. There were -spells of headache and vertigo. =Mentally= the tests showed him to be -subnormal. - -The =diagnosis= Of CONGENITAL SYPHILIS seems established; possibly the -pulmonary condition is syphilitic. The mental subnormality as well as -the abnormal traits and episodes are probably to be accounted for on the -basis of syphilitic involvement of the brain. - - 1. Are the headache and vertigo connected with syphilis? This is - perhaps suggested by the pleocytosis in the spinal fluid. - - 2. How shall we explain the negative W. R.? This patient had - received, shortly before his entrance to the hospital, salvarsan - and mercury. Possibly the drug treatment has little or nothing to - do with the negative W. R.’s since they not infrequently grow - weaker as congenital syphilitics grow older. - - 3. What is the explanation of the spells of excitement? Compare the - spells of excitement in a form of neurosyphilis described by - Kraepelin, namely: syphilitic paranoia, discussed in the case of - Bridget Collins (59). - - 4. Is treatment indicated considering the W. R.’s to be negative in - blood and fluid? Despite the negative W. R.’s in this case - treatment is strongly indicated on account of the pleocytosis. - This would seem to indicate that there is an active inflammatory - process in the cerebrospinal axis, and it is more than probable - that this process is syphilitic. How much real improvement of the - symptoms would result from antisyphilitic treatment it is - impossible to prophesy. Every case is a special problem, and this - case is very unusual in showing a pleocytosis in the absence of - other indications of syphilitic nervous disease, _viz._, globulin, - albumin and W. R.’s. - - - =CONGENITAL NEUROSYPHILIS resembling an undifferentiated case of - FEEBLEMINDEDNESS—actually PARETIC.= - - -=Case 77.= John Friedreich, a 7–year old boy, was brought to the -Psychopathic Hospital by agents of a charitable society, who found him a -neglected child and quite evidently a subnormal one. - -The dominance of syphilis in the situation was clear. The boy’s father -had died but a few months before of syphilitic heart disease, from which -he is said to have suffered for five years. The boy’s mother (the -parents were first cousins) had also been treated for syphilis and was -excessively alcoholic. The first child of this union—a girl—had died at -6 years, of a disease diagnosticated spinal meningitis. The history -indicates that syphilis was acquired after the birth of this first -child; but in any event it is possible that the meningitic condition of -which the first child had died was syphilitic. The second pregnancy -terminated in a stillbirth; the third issued in a girl, who died two -weeks after birth of what was termed “inward convulsions.” The fourth -pregnancy resulted in a miscarriage; the fifth in our patient, John -Friedreich. The sixth pregnancy resulted in a girl, now 5 years of age, -who is apparently normal. (Her W. R. was negative and she shows no -stigmata of syphilis.) - -The patient, John Friedreich, at some very early age had a rash on his -body diagnosticated as syphilis. He also had many seizures called -fainting spells. Ever since birth he had been taking mercury pills. He -had not learned to talk until his third year, and was able then to say -only a few disconnected words. In fact, John has never been able to talk -in complete sentences, mumbling much that is quite unintelligible. -However, he walked at 15 months in a normal fashion and nothing peculiar -in his gait was noted until he was 5 years old, when he began walking on -his toes, particularly those of his left foot. Shortly thereafter, the -seemingly inevitable trauma appeared; John fell out of a window and -severely injured his left leg, whereupon the peculiarity of toe-walking -became more pronounced and associated with a limp. - -[Illustration: - - Juvenile paresis. 7 years. -] - -The patient strikes one =physically= as having the development of a -child of about five years (actual age, 7). There are a few lymph nodes -palpable in the anterior triangles of the neck. The dilated and slightly -unequal pupils react neither to light nor accommodation. There is -practically complete deafness; loud sounds are not at all noticed. - -Withal, the child in a general way presents a somewhat attractive -appearance, being very playful and mischievous, lying about on the floor -and playing with whatever comes to hand, talking to himself or making a -few indistinct remarks to the bystanders. He walks awkwardly, on the -toes of the left foot. He pays little or no attention to his toilet and -needs to be dressed and cared for in all ways. He is quick-tempered and -at times very difficult to manage. - -There was, of course, little doubt of the =diagnosis= of CONGENITAL -SYPHILIS and of FEEBLEMINDEDNESS. The W. R. was positive both in the -blood and in the spinal fluid. The gold sol reaction of the fluid was of -the “paretic” type; there were 44 cells per cmm. and there was a large -excess of albumin and much globulin. - -As to prognosis, there is doubt. - - 1. Is, or is not, this a case of juvenile paresis? - - 2. Is it, perhaps, a relatively permanent case of feeblemindedness - due to congenital syphilis? On the whole, on account of the spinal - fluid symptoms, we should be inclined to give the case a - relatively poor prognosis, namely, of death in a few years. - However, we may perchance be later surprised to learn that the - patient has lived on, at least into early adult age. - - Note: Mercury tablets in some cases of congenital syphilis do not - seem effective. John Friedreich was treated most intensively by - syphilographers from birth. - - Dr. W. E. Fernald in a personal communication stated that - syphilitic cases of feeblemindedness are rather those of the - imbecile and idiot groups than of the higher levels. This - statement emphasizes again that the true hereditary cases of - feeblemindedness are rather those of the higher group, whereas the - cases in which special causes have operated in the uterus or in - early life eventuate in idiocy and imbecility. However, such a - case as that of Friedreich shows that now and then a case of - feeblemindedness without evidence of neurological disorder and - looking in almost all respects like an hereditary case may be at - times produced by syphilis. - - 3. How often is the central nervous system involved in hereditary - syphilis? An interesting table bearing on this point is presented - by Veeder.[16] The table concerns the lesions in various parts and - systems of the body in 100 cases of late syphilis. It appears that - in 47, or approximately one-half of Veeder’s series of 100 late - cases, the infection developed some form of lesion of the nervous - system. As Veeder remarks, this result runs counter to the common - statements of pediatricians, notably of Holt. - - Bones: - Periostitis tibia 4 - Periostitis skull 1 - Osteomyelitis 1 - - Joints: - Acute arthritis knee 8 - Acute arthritis ankle 1 - - Skin: - Macular eruption 1 - Condyloma anus 3 - Gummata 3 - Alopecia 3 - - Eye: - Interstitial keratitis 24 - Choroiditis 1 - - Ulcerations: - Nasal 2 - Laryngeal 1 - Pharyngeal 1 - - Central Nervous System: - Mental deficiency 23 - Cerebrospinal syphilis 14 - Hemiplegia 6 - Epilepsy 5 - Spastic paraplegia 4 - Chorea 2 - Hydrocephalus 2 - - Miscellaneous Conditions: - Ozena 1 - Enlarged spleen (only symptom) 1 - Torticollis 1 - Aortitis 1 - Obscure abdominal pain 1 - Obscure pain in legs 2 - Endarteritis obliterans 1 - Paroxysmal hemoglobinuria 1 - Raynaud’s disease 1 - Hutchinson’s teeth 4 - - - =Juvenile paretic neurosyphilis. Quadriplegia.= - - -=Case 78.= Gridley Ringer, 15 years of age, had the facies of a -congenital syphilitic, including Hutchinsonian teeth, rhagades of the -face, and the so-called Olympic brow. No secondary sexual -characteristics had developed. There was a marked speech defect. -=Mentally=, Ringer was a low-grade imbecile. He had been born at full -term, and delivery had been normal. There had never been other -pregnancies. He had never developed normally. - -The father admitted syphilis 23 years before, namely, 8 years before the -birth of his son, but the father had been treated for several years and -had been declared cured. - - 1. What would be expected in the spinal fluid of this case? Without - the history, it would perhaps be impossible to say whether the - case was one of a quiescent imbecility or one of juvenile paresis. - The spinal fluid of the juvenile paretic gives a picture identical - with that in the adult. The spinal fluid in this case showed a - positive W. R. (as did also the serum), a marked increase of - albumin and globulin, 115 cells per cmm., and a “paretic” gold sol - reaction. Accordingly, the diagnosis of GENERAL PARESIS was made. - - 2. What is the prognosis? The prognosis of juvenile paresis is - currently regarded as entirely grave. There is probably less hope - for improvement in juvenile paresis than in the acquired paresis - of adult life, since it seems to be a general principle that - congenital syphilis is always more difficult to cure than acquired - syphilis. - - This case had seizures a few months after initial observation, and - the seizures were followed by a transient right hemiplegia. This - right hemiplegia was shortly followed by a left hemiplegia, which - remained permanently. Moreover, a few weeks later, a right - hemiplegia again developed, leaving the patient with complete - paralysis and aphasia. Death followed in six weeks. - - 3. What effects were shown in the parents? Following up the parents - was rewarded by the discovery that the mother was suffering from - nerve deafness, probably of syphilitic origin, and that the father - had recently begun to suffer from what he considered rheumatism, - but which on examination was shown to be tabetic neurosyphilis - (“tabes dorsalis”). This family again supports the hypothesis that - there is a strain of spirochetes especially prone to attack the - nervous system. Here it would seem that the syphilis acquired by - the father had infected the mother and been transmitted to the - son. In all three infected by the same strain or strains of - organisms the nervous system was involved. It is difficult, - nevertheless, to explain on this hypothesis why in one case the - disease took the form of tabes dorsalis, in the second, eighth - nerve involvement and in the third, paresis. This question of - strains is really more than academic because it enters deeply into - the question of treatment, as well as that of the suggested - increased viability of the neural strain. - - - =Is there a relation between epilepsy and juvenile neurosyphilis?= - - -=Case 79.= John Doran fell off the rear of an ice-wagon, at six years of -age, and shortly afterward developed fits. It appears that John was not -unconscious at the time of his fall, but that he complained of headache. -Although the convulsions were fairly frequent at first, it appears that -they later became rare and occurred only when the patient got into a -temper. At the stage of exhaustion after violent excitement, John would -fall. - -=Physically=, at 9 years a fair development and nutrition were evident. -There was a great exaggeration of the frontal bosses; the nose could not -be said to be typically saddlebacked, yet there was a suggestion of a -sinking of the bridge. The teeth slightly suggested the Hutchinsonian -type, but only slightly. There was a slight roughening of the tibia, and -there was a slight scar over either knee. The patient graded according -to the Binet scale at 9 years, and he was regarded as definitely -feebleminded. - -The family physician states that, according to his information, the -father contracted syphilis when the child was between three and four -months of age, and that the mother also was infected at this time. -However, the child had not been suckled except immediately after birth, -and there had been no evidences, according to the family physician, that -John had acquired syphilis. - -Ordinarily, one might content himself regarding the case of John Doran -as one of idiopathic epilepsy with mental defect or deterioration. -However, the frontal bosses, suggestive teeth, the flattened bridge of -the nose, the roughened tibiae, and the old scars, though singly not of -great significance, collectively make one suspicious. Despite the family -physician’s belief that John could not have acquired syphilis from the -parents, the infection seems entirely possible despite the fact that no -symptoms developed early thereafter. - -The W. R. in this case proved positive in both blood serum and spinal -fluid. - - 1. What is the relation of trauma to this case of JUVENILE - NEUROSYPHILIS? Probably none. - - 2. What would be the effect of treatment? For a number of years John - Doran was lost sight of. He was, however, treated, according to - our information, with intraspinous injections of salvarsanized - serum, whereupon his convulsions shortly ceased. He has been - recently examined mentally once more, and still grades as - feebleminded. He still has violent outbreaks of temper. - - 3. Is such a case as Doran typical? Shanahan has investigated - conditions at Craig Colony. There were 22 out of 886 epileptics - (at Craig Colony) or 2½%, who showed a positive W. R. Nine of - these cases were regarded by Shanahan as cases of epilepsy - actually caused by syphilis. Viet had found 7%, and Bratz and Lüth - 5% of constitutional epileptics to be syphilitic, but the data of - these German authors were obtained before the era of Wassermann - tests. - - - =Adrenal tuberculosis complicating juvenile paretic neurosyphilis - (“juvenile paresis”). Autopsy.= - - -=Case 80.= When James Arnold appeared at the Danvers Hospital in his 22d -year, he looked as if he were but 12 or 14 years of age. He was -excessively fat but of fair muscular development. The left eye diverged -outward, and the left pupil was smaller than the right. An odd feature -was a high degree of pigmentation of the skin of the genitalia and the -groins (the axilla, the mammillary areas, and the oral mucosæ were free -from pigmentation). =Physically= speaking, the patient was practically -normal. =Neurologically=, however, there was much of interest, in the -light of which the clinical history was of value. - -It seems that after an apparently normal early childhood, the boy had -begun, at the age of 11, to experience difficulty in carrying out -every-day school tasks; and after this his mental capacity had slowly -but progressively deteriorated. The deterioration was not merely -intellectual, but the boy became dishonest and untrustworthy and -developed a number of untidy and uncleanly habits, behaving at the age -of 16, as the parents stated, like a child of six. - -In his seventeenth year, the boy had been taken with a severe attack of -what was regarded as an “attack of indigestion.” This attack ushered in -a gradually developing muscular weakness, especially involving the -limbs. By the age of 21 he had become irritable and the paresis was so -extreme that the patient was unable to get in or out of a carriage. - -This generalized muscular weakness was plain upon admission to the -hospital though there seemed to be no actual paralysis. The patient was -unable to walk in a straight line and Romberg’s position could not be -maintained. Marked tremor was present in the hands and lips. There was -bilateral impairment of vision and nystagmus. Reflexes and sensations -normal. Speech was markedly affected, all syllables being very much -slurred. School knowledge and memory for both recent and remote events -very poor. The patient’s habits were very untidy. He was very emotional, -easily made to laugh or cry; and in behavior, extremely childish. - -Two months after his admission to the hospital, the weakness suddenly -became extreme. He was constantly nauseated, refusing food. The face and -hands were cyanosed and the heart’s action rapid, weak, and irregular. -This attack lasted for a week and was followed by a period of -improvement, during which, however, he still remained very weak and -apathetic. - -One month later he again became so feeble that he was unable to raise -himself in bed. He complained persistently of feeling very “sick.” His -temperature was elevated and there occurred the same train of -circulatory symptoms observed previously, _viz._, rapid and tumultuous -action of the heart, with cyanosis of face and extremities. He soon -became unconscious, remaining so until his death, which occurred on the -seventh day of the acute attack. - -This case was under observation before the days of the W. R., yet -clinically the case had been diagnosticated JUVENILE PARESIS. There was -no history of the acquisition of syphilis or any likelihood of its -acquisition. Considered clinically, many of the classical features -described by Addison were present, _viz._, marked asthenia and apathy; -severe and frequent gastro-intestinal symptoms (the disease probably -commencing with the attack of so-called “acute indigestion” six years -prior to patient’s death); attacks of extreme cardiac weakness with the -characteristic small, low-pressure pulse. On the other hand, -pigmentation of the skin (usually the most striking clinical feature) -was limited to the external genitalia, these being colored a deep brown. - -The most striking feature found at autopsy was a bilateral adrenal -tuberculosis (caseation, giant cells, lymphocytosis, tubercle bacilli). -The thymus gland was persistent (7×5×.5 cm.), whereas the thyroid gland -was smaller than usual. The brain showed macroscopic and microscopic -features entirely consistent with the diagnosis of general paresis, -including lymphocytosis, plasmocytosis, irregular degrees of nerve cell -destruction, and gliosis, with an especially characteristic microscopic -picture in the frontal regions. - -It may be of note to consider the degree of change undergone by a brain -in 11 years or more of deterioration, and the following description of -the head findings is therefore included: - - =Head=: Hair abundant, dark. Scalp normal. Calvarium, weight 435 - gm., transparent in bregmatic region only, elsewhere thick and - dense. The average thickness of the vertical plate of the frontal - bone is 7 mm. The frontal bone shows a moderate thickening and - hardening of the inner table with obliteration of diploë. Dura mater - moderately adherent to the bregmatic region of calvarium. - Arachnoidal villi moderately developed. Sinuses not remarkable. Pia - mater shows a moderate focal thickening with opacity, especially - along sulci. Vessels well injected. =Brain=: Weight, 1200 gm. The - brain shows marked focal variations in sulcation and consistence. - Spread on a board, the right hemisphere is obviously somewhat bigger - than the left. There is a difference of only 0.5 to 0.75 cm. on - measurement of the greatest circumference of the cerebrum, taken - from the median line superiorly to the median line inferiorly, but - the right hemisphere is throughout slightly more convex than the - left. Both postcentral gyri are much narrowed in their superior - portions, and the sulci posterior thereto are deeper than the other - sulci of the hemispheres. The sulci of the orbital surfaces are - asymmetrical and, on the left side, show a tendency to microgyria. - The cerebral hemispheres as a whole show a remarkable tendency to - slight protrusion of the border gyri; especially those of the two - poles, of the free edges along the great fissure, and most - strikingly the gyri at the boundary line between the inferior and - lateral surfaces. This _marginal prominence_ is slight but obvious - and is emphasized by a slightly paler color in some regions. The - cerebrum shows a general induration which is greatest in the frontal - tips and along the inferior borders of the lateral surfaces of the - hemispheres, especially right. The orbital surfaces are firm, - especially anteriorly and externally (prefrontal); the tips of the - temporal lobes are firm, and the superior temporal gyri are firmer - than adjacent gyri. The postcentral gyri are indurated more than the - other gyri of the superior surface. The hippocampal gyri are - likewise firmer than adjacent gyri. - - =Cerebellum and pons=: Weight, 145 gm. The inequality of the two - hemispheres is more marked than in the case of the cerebrum. - - Greatest lateral diameter; left, 4.5 cm., right, 5.5 cm. - - Anteroposterior diameter adjacent to notch: Left, 5.8 cm., right, - 5.5 cm. - - There is no appreciable difference in depth. The diminution in - volume appears to be chiefly at the expense of the right clivus. The - inferior surface is firmer than the superior. The laminæ adjacent to - the horizontal fissure are firmer than the remainder of the - cerebellum. The pons is small. - -There was also a lateral curvature of the spinal column, as well as -characteristic adhesions between spinal dura and pia mater which are -always suggestive of syphilis. For the rest, there were few findings of -note: some adhesions of the left pleura, hypostatic congestion of the -lungs, tracheitis, and chronic gastritis. There were four lobes of the -right lung but it is doubtful whether this should be regarded as a -stigma. - - 1. Can we separate the symptoms of Addison’s disease from those of - paresis in this case? The extreme cardiac weakness with a - characteristic, small low-pressure pulse is in point. The asthenia - and apathy are consistent enough with Addison’s disease as well as - with paresis itself. It would also be possible to ascribe the - gastro-intestinal symptoms to either disease. - - 2. Of what significance is the persistent thymus? Persistent thymus - has been observed in a few cases of Addison’s disease, but that it - plays any part in the symptomatology thereof is a matter of doubt. - - 3. How can the obesity be explained? It is of course of note that - the thyroid gland was small, but microscopically there were no - peculiar features in this gland. - - 4. Was the adrenal tuberculosis actually primary? Minute search - failed to reveal evidences of tuberculosis elsewhere unless we - regard the few adhesions binding the lower half of the lung to the - chest wall as indicative of an old tuberculosis. In particular, - the mesenteric lymph nodes were normal. - - - =Neurosyphilis? Secondary stage of syphilis.= - - -=Case 81.= Florence Fitzgerald, a woman 25 years of age, applied at the -police station to be taken care of. She said she had been a prostitute -for the last few months, was now ill, and wanted to reform. She appeared -physically ill and was sent to the Psychopathic Hospital, where she -remained at first almost mute, making answers chiefly by nodding the -head. She gave the impression of daze or stupor, and in fact her -condition was at first regarded as catatonic. This reaction, after a few -days, changed and Florence became quite normal, giving a full account of -her condition. - -It seems that four months before going to the police station, she -developed a chancre, which was locally treated. A careful physical -examination showed a fine red macular eruption which was without much -question a syphilitic roseola. The spinal fluid yielded a positive W. R. -although other tests of the fluid were negative. Curiously enough, no -physical sign of involvement of the nervous system could be discovered. -We were inclined to regard the mental symptoms as partly due to the -syphilitic intoxication, and partly due to a psychic reaction of the -nature of defense. As for the positive W. R. in the spinal fluid, in -early secondaries various observers differ as to the frequency both of -the W. R. and of other changes, percentages being given that range from -25 to 90%. See case Caperson (45). It is of note that clinically there -were symptoms referable to a syphilitic involvement of the nervous -system; namely, marked headache and malaise. The headaches of the -secondary period are frequently the result of meningeal involvement. - - - =TABOPARETIC NEUROSYPHILIS (“taboparesis”); death from TYPHOID - MENINGITIS. Autopsy.= - - -=Case 82.= Frederick Estabrook was a salesman, who, be it noted, had -never had typhoid fever or any disease remotely resembling typhoid -fever. He had acquired syphilis at 19; had married at 22; was the father -of two healthy children (no miscarriages); had had a certain disturbance -of bladder and rectum, but remained a successful salesman to the age of -28, when advancing tabes confined him to bed for a time. At 30, mental -signs of PARETIC NEUROSYPHILIS developed, and death followed at 32, -after an acute illness of a week. - -The details of the history after the first symptoms at 28 are as -follows: - -At twenty-eight patient lost control of limbs and was confined to the -house about two months, under medical care. Three months later he had -regained partial control of his limbs but had lost all control of his -sphincters. After another month he had returned to work, but did not -work steadily and seemed to have lost ambition. In the summer of 1905, -his mind became obviously altered. He grew indolent and extravagant and -given to buying expensive and useless articles. Loss of interest in -things followed, together with loss of memory for recent events, lack of -insight into illness, delusions of persecution by wife, irascibility -followed quickly by crying. Before admission to hospital, he was -euphoric, drawling and tremulous in speech, sprawling in penmanship, -alternately depressed and exalted in manner. Knee-jerks were absent, -gait ataxic, pupils stiff to light. - -The family history was negative with respect to insanity. All the family -were reported as nervous. A brother died of peritonitis at twenty-eight, -a sister of pneumonia under twenty. Another brother and sister are -living. Father and mother died of heart trouble at about sixty-seven and -sixty respectively. - -The patient was at high school one year and was a fair student. -Considerable tobacco was used, and some alcohol. Intoxication denied. -There was no history of typhoid fever or other acute disease. - -The patient on admission was sallow, poorly nourished, and flat-chested, -with a slight lateral curvature. There was slight dulness over right -apex in front and in right upper back. Voice sounds were increased over -right apex in front and over whole right back. The right chest showed -bronchial respiration throughout. The respiration in front of right -chest was of an interrupted character. The liver seemed moderately -enlarged. The urine showed a very faint trace of albumin. There were a -few small nodes in right groin and a scar on dorsum of penis. - -=Neurological Examination.= Slight swaying in Romberg position. Slight -tremor of protruded tongue and extended fingers. Pupils irregular, left -slightly larger than right. Left pupil reacted to light consensually, -but not directly. Right pupil reacted very slightly to direct light, not -consensually. Knee-jerks and Achilles jerks absent. Ankle clonus absent, -abdominal and cremasteric reflexes brisk. Sharp and dull points were -recognized in the legs with numerous mistakes. Vocal and facial tremor. -Speech slow and drawling. Test phrases repeated well if care was taken. -Consciousness clear. Orientation perfect. Calculating ability preserved. -Many words omitted in writing. Penmanship clear but shaky. - -Hallucinations absent. Memory of recent events poor. Associations of a -logical or defining type. Patient denied various statements in -commitment papers and had little or no insight into the mental side of -his disease—slight euphoria. - -After a month’s observation the patient was removed to a quiet ward and -set to work a few days in the scullery. One night he began to yell as if -assaulted and said later that he had an idea that he was going to die. -Before three months had passed he had become untidy, disorderly, and -imperfectly oriented. - -The general degeneration continued rapidly. One week before death the -temperature rose to 103 degrees F., and the patient succumbed to what -seemed clinically like a bronchopneumonia. Unconsciousness two days -before death. - -Note with respect to history of typhoid.—Inquiries of his physicians, -wife, employer, and brother tend to show conclusively that the patient -never had a disease even remotely resembling typhoid fever. - -The =autopsy= findings were as follows: - -Acute conditions: - -Hypostatic pneumonia, with early serofibrinous pleuritis and without -lymph node swelling; =enlargement of mesenteric lymph nodes=; =acute -cerebrospinal leptomeningitis=; multiple small hemorrhages of spleen. - -Other findings: - -=Scar of penis=; =sclerosis of aortic arch= (Heller’s type?) and slight -coronary arteriosclerosis; =calvarium= thin and =dense=; =dura mater -thickened= and adherent to calvarium; calcified arachnoidal villi; -=chronic= cerebral and cerebellar =leptomeningitis=; =atrophy of frontal -lobes=; =granular ependymitis=; =sclerosis of posterior columns= of -spinal cord; emaciation; unequal pupils; slight parietal fibrous -endocarditis, slight mitral sclerosis; gastro-intestinal atrophy; -chronic cystitis; chronic abscess of prostate. - - The description of the head findings is as follows: - - Skin exceedingly loose, and the whole skull cap thinned. The diploë - are absent. Adhesion with dura easily separated. The dura somewhat - thickened, but not distended. Along the longitudinal sinus extensive - calcareous granulations adhere to it. The longitudinal sinus does - not contain blood, and the inner surface is normal in color. The pia - is extensively thickened and opaque and a general subpial exudate - exists which is more marked over the vertex where it lifts the pia - from the brain surface to the extent of three centimeters in - Rolandic, superior frontal, intraparietal, and mesial precentral - sulci on each side. The arteries at base are free from atheroma. The - temporal lobes are much bound down by adhesions, as is the - cerebellum. Post mortem softening is evident. The hemispheres show - no asymmetry, but the frontal convolutions are markedly atrophic. - The corpus callosum is united to the cortex by old adhesions and has - to be dissected away from it. Lateral ventricles contain some slight - amount of cloudy fluid, and the pia along the vessels is opaque. - Some granulations in ependyma. Brain weight, 1305 grams. Pons and - cerebellum, 195 grams. - - Cord.—Dura much thickened, and the pia corresponds to its appearance - in brain with a like exudate. Cross sections of cord show sclerosis - of posterior columns. - - Bacteriologically the _typhoid bacillus_ was cultivated _from the - meninges and from the swollen mesenteric lymph nodes_. The blood was - negative; the intestines were negative so far as lesions were - concerned. - -The microscopic examination confirmed the clinical diagnosis of GENERAL -PARESIS and of TABES, since there was not only an extensive chronic -encephalitis, with the usual lymphocytic and plasma cell deposit and -irregular gliosis, but also a well marked posterior column sclerosis, -not unusual save in its extreme degree. - -It might be surmised that some difficulty would arise in distinguishing -the effects of paretic meningoencephalitis from those of the more recent -typhoidal process. The well-known tendency of typhoidal processes to -escape polynuclear exudation, at least until frank necrosis has set in, -gave rise to the idea that the two mononuclear pictures—that of general -paresis and that of typhoidal processes—might be confusing. - -The picture presented by the meninges was scarcely what might be -expected. Although numerous mononuclear phagocytic cells are everywhere -found, yet the predominant picture is that of a polynuclear exudation. - -The polynuclear leucocytes occur in greatest numbers in the tissue -spaces, especially in the meshes of the lumbar arachnoid and in the -spaces of the frontal and paracentral pia mater. In the lumbar region of -the spinal arachnoid wide fields occur in which the cells are almost one -hundred per cent polynuclear leucocytes. In places phagocytic cells -occur, and in a few fields, even in the open tissue spaces, the number -of phagocytic cells may arise to fifty per cent. Edema is a considerable -feature in the meninges. Fibrin is found chiefly in the cerebral -meninges and appears in numerous delicate strands in the tissue spaces. - - - Moloch, horrid king, besmeared with blood - Of human sacrifice, and parents’ tears; - Though, for the noise of drums and timbrels loud, - Their children’s cries unheard that passed through fire - To his grim idol. - - Paradise Lost, Book I, lines 392–396 - - - - - IV. MEDICOLEGAL AND SOCIAL - - - =Neurosyphilis in a public character: eloquence, reformatory - efforts, notoriety.= - - -=Case 83.= Major Isaac Thompson, M.D., was a character. He had been -regarded as eccentric for many years prior to his death at 63. In fact, -it seems that there had been more or less definite symptoms and signs -about his fortieth year. The doctor himself had a ready explanation for -his Argyll-Robertson pupils; he explained that he had had a peculiarly -heavy smallpox at about the age of 27 (which would be about 1872). - -The doctor had a good secondary education, he had gone through the Civil -War as a hospital steward, went into business after the war, married, -and then went to the medical school, graduating at the age of 34. He -continued in practice for a dozen years, and then gave it up. For years -he had been especially interested in certain literary lines and he had -published any number of pamphlets, all of a somewhat striking -description, often with a political color and intended to stir up reform -measures. The doctor never bore a very good reputation, and years later -it was recalled that certain books disappeared from libraries and their -loss was almost certainly traced to Dr. Thompson. In general, however, -he was considered to be a rather worthy local figure. - -It is possible that a fall on the ice in his 61st year actually started -the fatal process, since after that time the patient had difficulty in -walking, and a few months later developed periods of excitement with -peremptory insistence on obedience to his wishes. Whereas formerly the -doctor had finished up one literary piece of work after another, he now -began to do very scattering work. He appeared in public to denounce -certain financial schemes with great force and unusual eloquence. His -eloquence was greatly complimented, and these compliments induced the -doctor to a remarkable crusade against a certain corporation; there was -so much truth mixed with the fiction of his eloquence that he obtained a -considerable following in his campaign. He wanted to start a bureau of -information for the instruction of the public on these matters, and he -planned to put up a building adjoining his own home for the -accommodation of the various clerks and writers in this bureau. However, -before the building had been actually started, an outbreak occurred. - -One morning the doctor was very excitable and noisy over the telephone, -ordering typewriters and giving directions to mechanics. He repaired to -Boston in connection with certain resources that he supposed (and gave -others reason to believe) had been supplied by the Government and by a -large newspaper. One evening he returned very late. It appeared that he -had had a fracas at a hotel and had knocked down one or two colored -porters, acting as though drunk. Upon being put to bed, the doctor -talked incessantly of religious matters, proposing to undertake a Sunday -School class. His interlocutor did not exhibit a particular interest in -this scheme, whereupon Dr. Thompson threatened him with violence. Police -and doctors were called in and a constant stream of conversation lasted -for hours. The patient was finally brought to Danvers Hospital upon -representation by physicians, to whom he told that his luck had turned, -that he was about to be made senator from the district, and that he and -Roosevelt were going to break up the trusts, and that, as a matter of -fact, he was a relative of Mr. Roosevelt. - -Upon admission, the patient was a well preserved and well groomed man -with gray hair and beard. He was somewhat pallid but his teeth were well -preserved and well cared for, and there was little or no physical change -except a slight hypertension. He claimed that he had suffered from -kidney disease for some years, and there was in fact a trace of albumin -in the urine. - -=Neurologically=, the plantar and Achilles reactions could not be -obtained, but there were no other reflex disorders except the bilateral -Argyll-Robertson pupil. The doctor’s explanation for these stiff pupils, -which he described as existing for many years, was frank and -circumstantial, so that the unlikelihood of Argyll-Robertson pupils due -to smallpox was rather frowned upon by him. Without entering upon a -detailed description of the clinical symptoms and course of the disease -which led to death a little over a year after admission, it may be said -that the differential diagnosis lay between the expansive form of -general paresis and a maniacal condition, presumably the maniacal phase -of manic-depressive psychosis. From the data of a special staff meeting -held upon the case, we learn that the diagnosis of manic-depressive -psychosis was entertained more strongly than that of general paresis. -Thus, for general paresis alone was the somewhat gradual onset with -increasing excitement, accompanied by expansive delusions concerning -unlimited finance, personal over-importance, and Argyll-Robertson -pupils. Dismissing the Argyll-Robertson pupils from consideration, the -diagnosticians were led to see in the constant motor activity displayed -in conveying an enormous number of thoughts on paper, inconsistent -talking with digressions, a manic-depressive psychosis. There was no -amnesia and no other sign of mental deterioration. There was a certain -improvement early in the hospital stay of the patient. Consciousness was -clear and orientation perfect. The delusions themselves, though -extravagant, were not inconsistent or fantastic. The hallucinatory -disorder was hardly characteristic either of manic-depressive psychosis -or of paresis. - -The patient might be described as “interesting.” A good preliminary -training with years of travel and variety of occupation, furnished him -with a fund of knowledge. An excellent memory, prompt replies and -repartee, endless digressions with voluntary return to the original -topic, caused him to be an amusing and even instructive interlocutor. -However, his commitment and confinement in the institution seemed always -entirely wrong, and he expressed mixed feelings about the family, now -being bitter against them, and again condoning their mistakes. The -patient’s conduct was good and he was tidy in habits, and tried as far -as possible to conform to the requirements of the hospital. The doctor -showed a marked antipathy toward a certain male attendant, who had -removed articles from his clothing upon admission and had reclaimed a -book on rules and regulations. The doctor prepared a list of 327 -different acts of abuse, lack of care, and insubordination which he said -he had observed in the hospital. - -In the last weeks of the patient’s illness, his ideas became more -expansive and extravagant, dealing with a grapevine system of wireless -communication and delusions of unlimited wealth. He would at times keep -his room flooded with urine and water for the purpose of keeping down -the plague which he said was infecting the hospital. Later he mixed food -with urine and other ingredients, claiming that he was constructing an -elixir of life. - -The =autopsy= showed few changes of the calvarium or of the dura mater, -nor was the pia mater more than slightly thickened and milky over the -frontal poles, along the longitudinal fissure and over the sulci. There -were fairly firm adhesions of the pia mater to the dura mater along the -longitudinal fissure and over the frontal poles and at the temporal -tips. The hemispheres were firmly interadherent, and the -cerebello-pontine tissues were covered with a firm leptomeningitis. The -floors of the ventricles were smooth and the basal vessels showed little -beyond a few spots of sclerosis. There was a generalized increase of -consistence. The frontal gyri were rather prominent with wide sulci, but -upon section no very marked atrophy of the gray matter could be shown. -The rest of the brain failed to show any flaring of sulci or any special -evidence of cortical atrophy. The brain weighed 1250 grams; a possible -diminution of 100 grams, considering the patient’s body length. However, -it must be remembered that he was at this time 63 years of age. - -=Microscopically=, the diagnosis of GENERAL PARESIS was confirmed on the -basis of plasmocytosis, lymphocytosis, gliotic changes and nerve cell -destruction. There was an unusual variation in the degree of the -destructive process, which picked out, for example, certain regions of -the right side for maximal lesion (cornu ammonis, gyrus rectus, and -superior frontal gyrus). - -If the patient’s own estimate of 35 years’ duration for his -Argyll-Robertson pupils can be trusted (and in general his memory was -extremely good), we may well conceive an unusual duration for the -process in his case. There was, however, in the body at large no very -marked degree of changes. There was a slight old tuberculosis. There was -a slight interstitial nephritis, with cardiac hypertrophy and fibrous -myocarditis. There was also a sclerosis of the mitral and aortic valves; -there were chronic changes in the spleen, liver, and bladder; there was -generalized arteriosclerosis of mild degree; there were two round -gastric ulcers near the pylorus. The liver weighed but 800 grams, and -its left lobe was somewhat rough. - -This case is placed among the medicolegal and social cases because the -phenomena that ushered in his last illness were mistaken by the local -public for meritorious social reform measures. They were regarded as not -markedly different from the variety of steps taken by the very active -doctor in previous years; indeed the public eloquence that he displayed -a year before his death was quite in line with previous habits, despite -the suspicious over-brilliance of language. It is an important question, -how far the eccentricity and literary overactivity of the latter half of -the doctor’s total life can be explained on the basis of a mild -syphilitic irritation of the nervous system. In this connection we are -tempted to recall the suggestions of Mœbius concerning a portion of the -literary products of Nietzsche. Our doctor was by no means so brilliant -an exemplar of syphilitic literature as was Nietzsche, if we grant the -hypothesis of Mœbius to cover our doctor’s case as well as that of -Nietzsche. In the future, important studies of character change under -the influence of syphilis will doubtless be made. With modern diagnostic -methods, of course, the diagnosis would have been rendered almost at -once in the case of Major Isaac Thompson, M.D., and much of his past -life would have been brought under special review in connection with the -syphilis which doubtless the blood serum or at any rate the -cerebrospinal fluid would have shown. - -This case illustrates but one of the many social complications arising -as the result of paresis. When one recalls that the onset is often -insidious and not correctly understood for a period of time, it is -readily seen that many unfortunate acts may be committed by a patient. -As hypersexual desire is not an infrequent early symptom and as judgment -is early disturbed, loose morals may ruin the patient’s reputation. The -poor judgment and expansive delusions often lead to foolish business -deals wherein the patient’s family is left destitute. At other times the -onset is sudden and then the danger of false commands or acts by a -person in a responsible position, as a steamship captain, an engineer or -chauffeur, may lead to loss of life and property. - - - =Sudden grandiosity: debts. PARETIC NEUROSYPHILIS (“general - paresis”): Question of liability.= - - -=Case 84.= Lester Smith was a salesman, 31 years of age, who, while on a -business trip, accompanied by his wife, suddenly developed grandiose -ideas. He originated a scheme of cornering the phonograph market. His -prospects seemed so certain to him, that he hired an expensive suite of -rooms in a hotel at something over $35 a day. As at the first -presentation of his bill it was found that he had no money to meet these -charges, he was taken into custody and at once transferred to a hospital -for the insane, where it was discovered that he was suffering from -GENERAL PARESIS. - - 1. What is the patient’s responsibility for these debts? Legally the - patient or his estate is responsible for debts accruing from - services rendered or goods received. As he is adjudged _non compos - mentis_ contracts entered into would not hold, and he would not be - considered liable for criminal acts. - -Note: This case shows how dangerous paresis may be not only to the life -and usefulness of a patient, but further how it may ruin a family -financially. Mr. Smith’s little escapade used up all the money that he -had been able to save in his life and when he was taken to a hospital -his wife was left destitute. - - - =Suicidal attempt (?) by a neurosyphilitic.= - - -=Case 85.= At first Mrs. Annie Monks, a widowed seamstress, 50 years of -age, did not particularly suggest syphilis. Mrs. Monks was sent to us -from a general hospital. She had been found unconscious in her room, -with gas turned on, and a diagnosis of gas poisoning was made. Mrs. -Monks remained unconscious for 24 hours, and her apparent suicidal -attempt seemed to warrant her being sent to the Psychopathic Hospital. -Mrs. M., however, scoffed at the idea of any attempt at suicide, and -claimed to have had no recollection of any such affair. On the contrary, -she had gone to mass the morning of the day on which she was taken to -the hospital, remembered well enough returning to her room but nothing -of what followed until she woke up. - -Mrs. Monks was not coöperative and would reveal few facts about her -history. For years, she had had edema of the feet and palpitation of the -heart (the heart was somewhat enlarged, with a double murmur in the -aortic area, systolic louder, and a blood pressure of 160 systolic and -85 diastolic; clubbed fingers; palpable liver). She had been treated in -the out-patient department of a general hospital for a number of months. -We could obtain no evidence of mental impairment, particularly none of -memory. - -Aside from the heart lesions above indicated, the patient was fairly -well nourished, with a slight enlargement of superficial glands, and was -otherwise normal. - -=Neurologically=, the slightly irregular pupils reacted poorly to light; -the right knee-jerk could not be obtained, whereas the left knee-jerk -was very active. Systematic examination revealed no other disorder -except that the abdominal reflexes could not be obtained. - -Here we have, in a cardiac patient, a possibly or probably accidental -gas poisoning, and little to go upon for a profounder diagnosis than the -sluggish irregular pupils and unilateral absence of knee-jerk. - -The routine serum W. R. came through as positive. Following custom, we -examined the spinal fluid, finding the W. R. here again to be moderately -positive (strongly positive to 1 cc., moderately to 0.7 cc., and -negative to 0.5, 0.3, and 0.1 cc.). The gold sol index was 1 2 2 1 0 0 0 -0 0 0, which must be interpreted as syphilitic. There were 16 cells to -the cmm., the albumin was 1+, and the globulin stood at 2+. - -Here, then, we seem to have evidence of an inflammatory process of the -central nervous system, and it is natural forthwith to be sceptical as -to the accidental nature of the gas poisoning. Perhaps there was an -attempt at suicide based upon a passing impulse, or perhaps there was a -period of confusion in which the cock was not turned off. - -In any event, we feel justified in making the diagnosis of cerebrospinal -syphilis on the basis of the neurological and laboratory findings. On -the whole, we are inclined to make a diagnosis of VASCULAR NEUROSYPHILIS -with a moderate involvement of the MENINGES. - - 1. What is the outcome in such cases as that of Annie Monks? The - case somewhat resembled that of Martha Bartlett, who still - survives. The case of Annie Monks illustrates another outcome. A - few days after her admission, she became unconscious once more, - and upon recovery remained very much confused and aphasic, - moaning, and unable to handle herself well, although without - definite paralysis. Three weeks later the patient died, although - in the meantime strenuous antisyphilitic therapy was practised. - Death was sudden. We thought death due to cerebral embolism. - - - =Early delinquency and neurosyphilis in a juvenile.= - - -=Case 86.= Frank Johnson was 21 years of age when he was taken up by the -police for threatening his sister with a revolver. The police thought he -deserved an examination at the Psychopathic Hospital. The patient -protested that he had threatened his sister only to frighten her -because, he said, she nagged him and made him nervous. In fact, they had -always had trouble as she had always nagged him and they had always -fought together. Moreover, their mother always took the sister’s part. -They had been troubling him for days, and at last Frank could stand it -no longer. His sister had complained of the way he treated her dog. -Moreover, Frank said he had not been feeling well; there had been some -trouble with his stomach; and after one of the nagging attacks, he had -taken out an old empty pistol to scare his mother and sister. - -In these cases, it is good practice to consult the sister also. She said -that Frank had always been very difficult to manage, unwilling to work, -preferring to loaf about, spending every obtainable cent; he was once in -a reformatory for several years, but not reformed thereby; recently -given to drinking; at times acting somewhat peculiarly (sitting at the -window with his hat on, refusing to move). - -Further =mental examination= of Frank showed that he was properly -oriented and in possession of a good memory, although he was quite -obviously a liar. He lay about in bed at the hospital, saying that he -was too weak to be up. He was a bit dull, at times not readily grasping -ordinary questions. - -=Physically=, Johnson was rather thin; the teeth were somewhat -peg-shaped although far from typically Hutchinsonian. The pupils were -unequal and irregular, and failed to react to light or even to -accommodation when tested. The deep reflexes of arms and legs could not -be obtained, though the superficial reflexes were present. For the rest -systematic examination proved negative. Serum W. R. negative. - -The first thought in such a case would be that the criminological -diagnosis of delinquency would be sufficient. However, the pupillary -disorder and the areflexia are suggestive despite the negative serum W. -R. Resort was naturally had to lumbar puncture, whereupon a positive W. -R. was found, a characteristically “paretic” gold sol reaction, -globulin, excess albumin, and 134 cells per cmm. In short, it would -appear that we must consider a diagnosis of JUVENILE PARESIS, and, in -point of fact, the patient deteriorated rapidly from this time, becoming -demented at the end of a few months. - - 1. How far are the early difficulties of management (leading to a - reformatory) due to syphilis? We should not dogmatically say that - there is a relation between the early delinquency and syphilis. - Still, it is not unusual to find emotional disorder and - instability as well as delinquency in congenital syphilitics. - - 2. What suggestion, if any, should be made to the patient’s - intelligent and seemingly normal sister, two years older? We - prevailed upon Miss Johnson to submit to the W. R. of the serum, - which was found, as in the case of Frank, to be negative. Frank’s - sister should undoubtedly submit to a lumbar puncture; but in the - present phase of mental hygiene, she would be difficult to - persuade. - - 3. How is it possible to find such a marked evidence of congenital - syphilis in a younger sibling with no evidence of syphilis in the - elder? In the first place, there may be a history of entrance of - syphilis into the lives of the parents between the pregnancies. - However, in other instances, there is no evidence of such - intercurrent syphilis, and contrary to the prevailing opinion it - is not so infrequent to find congenital syphilis in the younger - brother or sister of a normal person. - - 4. What can be said of treatment in such cases? In the first place - it is clear that delinquent cases should be tested far earlier for - the possibility of syphilis. Had this case been examined by a - neurologist or alienist many years earlier, it is probable that - the same pupillary signs and the peg-shaped teeth would have been - found, and that the hypothesis of syphilis might have been raised. - There is no good evidence as yet that these cases can be markedly - benefited by treatment. - - - =Neurosyphilis in a “defective delinquent.”= - - -=Case 87.= Vivian Walker, 22 years of age, was arrested on the streets -of Boston for drunkenness. Upon arrival at the jail, she developed a -series of convulsions, each lasting a very brief time, with loss of -consciousness, frothing at the mouth, and jerky movements of the arms -and legs. - -The Walker family was known to the police, since there were police -records in two generations on the maternal side. The father was regarded -as of rather low-grade mentality; a sister had committed suicide. Vivian -herself had been irregular at school, was regarded as vicious, and had -been hysterical. She had been committed to a reformatory at the age of -15 years. In the reformatory she had a number of excited outbreaks, with -resentment of discipline, and these outbreaks presented hysterical -traits. After each outbreak Vivian was depressed. It was during her stay -at the reformatory that her sister committed suicide. Vivian attended -the funeral, and the idea of suicide appears to have taken hold of her -mind, as she constantly spoke of suicide, threatened suicide, and made -several attempts. She claimed at this time to see visions and to hear -her sister’s voice. On that ground she had been committed to a hospital -for the insane at 16. - -At the hospital there were many fluctuations in mental condition. Vivian -professed discouragement on account of poor home influences, telling how -her mother had often been in prison, allowing Vivian to come under the -influence of bad girls. Now and then Vivian had outbreaks of profanity -and glass-breaking, and she also made at the hospital for the insane -several half-hearted attempts at suicide. At the age of 19 she was -returned to the reformatory, whence she was placed out on probation and -allowed to return home. - -However, she was shortly re-committed to the insane hospital in a phase -of excitement, talking continuously of men and sex relations, and also -of imaginary illicit sex relations with any man whom she happened to -see. Again from time to time she made attempts at suicide. However, she -was allowed to go out on visit, returned to her habits, and at the time -of her arrest was living as a prostitute. - -After her convulsions in jail, she was admitted to the Psychopathic -Hospital. At first obstinate and stubborn, later she became tractable. -Special mental tests left her in the subnormal class, but we could -hardly class her as feebleminded. We were able to observe her in a -number of seizures, during which she would drop to the floor, apparently -lose consciousness, writhe about, and assume the position of -opisthotonos, the whole attack lasting but a minute or two. - -There was pelvic tenderness, with gonococci in the urethral smear. -Salpingectomy had to be performed, but after the operation Vivian -insisted upon getting up and running about on the second day, tearing -the bandages from her abdomen, and infecting the wound. Outbreaks of -excitement also followed the operation. - -In the diagnosis of this case, we must probably separate the convulsive -phase from the remainder of the phenomena. The conduct disturbance, -emotional outbreaks, and suicidal attempts date from early youth, and no -doubt the diagnosis defective delinquent would fit Vivian from the -beginning. The hereditary taint is characteristic enough. The sundry -phenomena in the insane hospital, and particularly the hallucinations, -lead one to wonder whether Vivian is not possibly even suffering from -dementia praecox. - -As to the convulsions, it would hardly appear that they are typically -epileptic, although certainly epileptoid. Their onset at 22 is somewhat -unusual. Several features of the seizures together with the opisthotonos -and the previous history of hysteria, lead one to think of making the -diagnosis hysteria. - - 1. Can cerebrospinal syphilis cause the symptoms? We found the serum - W. R. to be positive though Vivian denied syphilitic infection. - (She also denied gonorrhœal infection despite the clinical and - laboratory findings.) We found that the spinal fluid yielded a - gold reaction of a typical syphilitic nature, showed an excess of - albumin, a slight amount of globulin, and 130 cells per cmm. Even - these findings, however, would perhaps not justify stating that - the convulsive seizures are of syphilitic nature. The seizures - disappeared under the administration of antisyphilitic remedies. - It would seem, therefore, that the seizures should be regarded as - of syphilitic nature. In any event, the diagnosis of cerebrospinal - syphilis is justifiable. This syphilis, however, is of an active - nature and probably of recent production. We should be at a loss - to explain the earlier mental features in Vivian as syphilitic and - are therefore fain to associate the two psychoses, PSYCHOPATHIC - PERSONALITY and DIFFUSE CEREBROSPINAL SYPHILIS. - - - =NEUROSYPHILIS (“paresis sine paresi”) in an habitual criminal, a - forger.= - - -=Case 88.=[17] —— was brought to the hospital by the police. He was -charged with having forged a check, and on account of the crudeness of -the work his mental condition was suspected. - -=Family History.= The paternal grandfather was considered fast, drank a -great deal and was said to be a thief. The father is said to have been -forced to leave the State when a young man in order to avoid the -reformatory. Paternal cousin murdered a man; the sisters of this cousin -said to have been wild and one brother married a prostitute. Nothing -known of maternal relatives. - -=Past History.= Medical history is unimportant. He denies syphilis. His -early childhood is of little significance. He was somewhat dull in -school. At about the age of twelve he began to lie and steal, and has -continued this ever since. His attempts have all been very crude, it is -said, and when confronted he would strenuously deny his deeds, even when -the evidence was overwhelming. He forged checks, borrowed money from all -his friends, and charged things at stores to the family. The family paid -the bills for a time, and then later had him sent to a reform school. He -was married at nineteen, but wife has left him and obtained a divorce. -He has been excessively alcoholic for years, and is suspected also of -taking drugs. He was discharged from the navy dishonorably. He later -joined the army and was discharged therefrom on account of “rheumatism,” -according to his account, but in reality deserted. He had finished a -jail sentence of thirteen months for forgery a little over a year before -entrance. - -=Physical examination= shows a well developed and nourished man. The -general physical examination is negative. The lungs show nothing -abnormal. The heart is not enlarged, there are no murmurs or -irregularities; blood pressure, 145 systolic. The alimentary system is -negative. No palpable lymph glands. =Neurological examination=: pupils -equal and react to light and accommodation. Extraocular movements well -performed. Tongue projects in the median line, with no tremor. There is -no evidence of facial paresis or weakness of the muscles. The biceps, -triceps, knee-jerks and ankle-jerks are present and equal on the two -sides. There is no Gordon, Babinski or Oppenheim; no ankle clonus. There -is no tremor of the extended hands. No Romberg sign. There is a little -difficulty in the finger-to-finger test. There is no sensory disturbance -either subjective or objective. No tenderness over nerve trunks. - -=Mental examination= shows nothing of a psychotic nature. Patient is -well oriented; memory for remote and recent events is well preserved, -school knowledge well retained, grasp on current events good; no -delusions or hallucinations elicited. Patient is not feebleminded, -according to the intelligence tests of Binet and Simon and -Yerkes-Bridges, but shows poor attention and gives evidence of weakness -in volitional spheres; is very suggestible. - -To summarize the case, then, we have a man of thirty years of age who -has shown criminalistic and anti-social tendencies since childhood, -whose general physical and neurological examination is negative -(excepting the laboratory tests), whose mental examination shows no -psychotic symptoms, and who seems not feebleminded. In other words, with -the exception of the serological and chemical findings in the blood and -cerebrospinal fluid, there is nothing to suggest that he is more than a -“criminal type.” - -Wassermann reaction in blood serum positive. - -Wassermann reaction in cerebrospinal fluid positive. Examination of -cerebrospinal fluid: globulin ++, albumin ++, cells 55 per cubic -millimeter; large lymphocytes, 9.1 per cent; small lymphocytes, 90 per -cent; plasma, 90 per cent. Gold sol reaction, 3321000000. - - 1. Can the criminalistic tendencies be condoned in this case on the - ground of neurosyphilis? As a matter of fact the delinquencies in - this patient reach back to early childhood and as there is no - evidence of congenital syphilis it cannot be held that syphilis - had any bearing in the causation of symptoms. Even were the - delinquencies only of recent date it is doubtful if the court - would take cognizance of the laboratory findings in the absence of - definite mental symptoms. In this connection it may be stated that - the court takes cognizance only of the acts of a patient at time - of examination, and not of the history or laboratory findings, in - committing a person. We have had several patients who from - history, physical signs and laboratory tests made the diagnosis of - paretic neurosyphilis easy and yet who could not be committed - because they were mentally clear at the time. Such patients may be - of grave potential danger to themselves and families, and present - numerous social problems. See case of Joseph Wilson (95). - - - =JUVENILE PARETIC NEUROSYPHILIS (“juvenile paresis”) with initial - trauma.= - - -=Case 89.= Margaret Tennyson was a small girl of six years, described as -having been normal until run down by a double-runner sled about 13 -months before her arrival at the hospital. The change was stated to be -remarkable. “She was as unlike her own self as darkness and daylight.” -Once fat and sunny, talkative and demonstrative with her toys, now -Margaret had become silent, sullen, worried, and of a violent temper, -stubborn and unmanageable. It does not appear that the patient was -seriously injured by the double-runner, as she was able to walk a short -distance home. Shortly, however, she began to have trouble with her feet -(diagnosed at the time as flat-foot), and thereafter her whole character -and disposition changed. Upon arrival at the hospital, the patient -walked with a typical scissors gait of spastic paraplegia. - -=Physical examination= was very difficult through lack of coöperation -and a screaming and kicking resistance upon every attempt. There was a -suggestion of hydrocephalus in the protrusion of the forehead. The -pupils reacted readily to light and accommodation. The knee-jerks were -active, but there was otherwise no disorder of reflexes. The patient had -great difficulty in getting up from the floor, and for the most part -insisted upon lying in ventral decubitus on the floor, crying when -attempt was made to raise her. An attempt was made to test her by the -Binet scale, by which she was found to rate at 2⅘ years although a -portion of this low-rating was thought to be due to a failure of -coöperation. - -The =family history= threw little or no light upon the case. The parents -were living and well; a brother of 16 years was at work in the market -district; two of the other siblings are in the first and second grades -at school and regarded as exceptionally bright by their teachers. The -fourth was the patient, Margaret; a fifth had died at 9 weeks of heart -trouble; the sixth, seventh, and eighth, of 3, 1½ years and 3 months -respectively, appeared entirely well. There were no miscarriages or -stillbirths. - -[Illustration: - - Juvenile paresis—spastic paraplegia. 5 years. -] - -The scissors gait and spasticity seem to point undoubtedly to organic -disease of the nervous system, along with which the mental deterioration -seemed to suggest an active progressive involvement of the cerebrum. The -history seemed to be convincing that the child was not an instance of -congenital feeblemindedness. - -A neurologist’s clinical diagnosis would naturally be syphilis. In point -of fact, this diagnosis was borne out by the laboratory tests, which -showed a positive W. R. in the serum and spinal fluid, positive -globulin, a slight excess of albumin, and a syphilitic gold sol -reaction. - - 1. What is the significance of the trauma in the case of Margaret - Tennyson? The trauma seemed to the family the precipitating cause. - We find cases of general paresis in adults very definitely - following trauma, yet neurosyphilis, both in adults and in younger - patients, mainly occurs without trauma. On the whole, in this - case, it is perhaps safer to regard the trauma as mere - coincidence. A sister older than Margaret was found upon - examination to have a positive W. R. The other children could not - be examined. - - - =Traumatic form of PARETIC NEUROSYPHILIS (“general paresis”).= - - -=Case 90.= The point about Joseph O’Hearn was his entire mental -soundness up to the time of an injury at work, when he was blown through -a double window in an explosion, badly bruising his head. Shortly after -the accident, although not immediately, the patient began to show signs -of mental disorder, doing very foolish things, losing his memory, and -becoming unable to work. - -It was eight months after the explosion when O’Hearn, at the age of 36, -was admitted to the hospital with general mental impairment. O’Hearn was -confused and disoriented for time and place, although he seemed to -understand that he was in a hospital. He was given to foolish laughter -and a silly manner. There was considerable emotional disorder; judgment -was clearly impaired, and memory was poor. - -=Physically=, there was little to be found except upon =neurological -examination=. The right knee-jerk was greater than the left; the tongue -and fingers showed marked tremor, there was a speech defect and writing -disorder. - -On the whole, it seemed impossible not to make the diagnosis GENERAL -PARESIS, especially in view of the laboratory tests, with positive W. R. -in both serum and fluid, a “paretic” type of gold reaction, 59 cells per -cmm., excess albumin, and a large amount of globulin. - - 1. What is the relation of the trauma to the paresis? Trauma is - regarded as a precipitating cause, and Industrial Accident - Commissions have been known to allow damages in such cases. Mott - believes that the symptoms of a post-traumatic paresis must not - develop until after a week’s interval of freedom from symptoms, - since he believes that time is required to destroy or irritate the - brain to the point of producing the paretic picture. Our data are - in agreement with those of Mott. Mott also points out that gumma - sometimes occurs at the site of the trauma. - - - =False claim for compensation in neurosyphilis.= - - -=Case 91.= The facts in the case of Levi Sussman can be brought out by -the following extracts from a report to the Industrial Board: A claim -was made to the Board that the symptoms had developed after a fall from -a building, some _nine months before hospital observation_. No -connection could be found between this accident and the PARETIC -NEUROSYPHILIS found. We introduce the case to emphasize the possibility -that irrelevant accidents may be regarded by ignorant or unscrupulous -persons as setting up a mental disorder for which damages are claimed. -If symptoms are already in existence before the accident and are not -especially increased thereafter, naturally no damages should be -recovered. Unscrupulous persons may falsify about the pre-traumatic -history and claim the development of symptoms immediately after the -accident. Such claims are beyond question to be viewed with the greatest -suspicion. Some days or weeks should elapse before definite symptoms in -post-traumatic paresis appear. Just how long an interval may elapse -between trauma and paretic symptoms and shall entitle the case to be -regarded as one of traumatic paresis, is perhaps a matter of doubt. It -would seem, however, on general grounds that three months is the longest -period in which the post-traumatic effects are likely to be delayed. - -The question of traumatic paresis is of great interest on account of the -war. The great strain under which the men at the front live and the -physical injury due to being “buried” is probably responsible for an -increasing number of cases of neurosyphilis. Such at least is the -impression of Canadian medical officers with whom we have spoken. See -Section VI, Neurosyphilis and the War. - - - =Traumatic exacerbation(?) in PARETIC NEUROSYPHILIS (“general - paresis”).= - - -=Case 92.= The case of Joseph Larkin was of note from the point of view -of the Industrial Accident Board. This Irish teamster was said to have -been injured in his head two or three months before coming up for -examination at the age of 45. For a week Larkin had had frontal -headaches, had been sleeping poorly, and had been somewhat worried. In -fact, he had stopped work. The W. R. of the serum was positive and a -diagnosis of PARESIS could be made. The case did not come up for -consideration by the Industrial Board until two years after his initial -appearance. - -The =physical examination= showed irregular pupils, sluggish pupillary -reactions, Achilles absent, swaying in the Romberg position, enlargement -of the heart to the left, positive W. R. of the blood and of the spinal -fluid. - -=Mentally=, the patient’s orientation for place was poor and his memory -defective. Emotionally he was depressed or apathetic and was -apprehensive. His flow of thought was slow, and his insight into his -condition poor. - -It is interesting that a variety of causes have been assigned in this -case for the condition: such as, his work, anemia, unhygienic -surroundings, and arteriosclerosis. - -This case is not a sharply-defined case of post-traumatic general -paresis, since there had undoubtedly been a variety of mental changes -before the accident. Accordingly, recovery of damages to a full amount -could hardly be expected as in certain cases in which the phenomena of -paresis appear only after the trauma. - -[Illustration: - - Post-traumatic cranial gumma—developing 13 months after local injury - of skull. -] - - - =Trauma: syphilitic lesion of skull at site of injury.= - - -=Case 93.= The medicolegal interest of Richard Marshall is extreme, as -may be seen from the following brief report by the Psychopathic Hospital -to the Industrial Board. - - “As to the case of Richard Marshall, a patient under the provisions - of the temporary care act from December 1 to December 10, inclusive, - this case has proved unusually interesting in that the patient has - proved to be syphilitic by the Wassermann reaction of the blood. - There is no evidence of syphilis in the examination of the - cerebrospinal fluid. The X-ray examination of the skull, taken in - connection with the Wassermann reaction of the blood, warrants the - diagnosis of syphilitic osteitis of the skull at the site of the old - injury. We regard his present condition as shown by the X-ray as a - syphilitic bone condition predisposed to by the injury. We do not - find that the patient has any features of traumatic neurosis. - - “Mentally, having an actual age of 30, patient grades at 11.2 years. - It may be that patient has always been a moron. He has earned about - $8.30 a week. - - “We regard the patient as deserving treatment and feel that - responsible parties in the case would do well to have such treatment - instituted.” - -The principal symptom of which Marshall complained was headache chiefly -felt in the region of the osteitis. There was marked sensitiveness to -percussion in this area. It is of course difficult to decide whether the -headache was entirely due to the gummatous lesions or whether the trauma -had caused contusions of the brain as well. It is also possible that the -dura underlying this area was involved. - - - =OCCUPATION-NEUROSIS in a granite-cutter: SYPHILITIC NEURITIS?= - - -=Case 94.= David Fitzpatrick was a case referred to the Psychopathic -Hospital by the Industrial Accident Board. He was a granite-cutter of 52 -years of age, and had begun to complain of pain in the forearm, -extending back from the elbow, about six months before admission. It -seems that the patient had been growing progressively worse and had -thought he would have to quit work because of difficulty in grasping the -hammer. A physician had told him that he must stop his work at -granite-cutting or else he would entirely lose the use of his arm. He -was in point of fact laid off because of slackness of work and had been -unable to get work again. The pain in the arm, however, had continued -and at times was very severe. Sometimes the pain and the worry led to -insomnia. Fitzpatrick wanted the insurance company to pay certain -accumulated bills, and maintained that he would be able to do work at -$15 a week if work could be found for him. The general situation in this -case can be gathered from the following abstract from the report to the -Industrial Accident Board. - - “Secretary Industrial Accident Board, - “Dear Sir: - “_In re_ David Fitzpatrick - - referred to us with a copy of an impartial report filed by the - Massachusetts General Hospital,—we concur with said impartial report - that there is now no evidence of paralysis of the arm. We do not - find that the positive Wassermann reaction, although it indicates a - history of syphilis, has affected the patient other than possibly to - have reduced his general mental capacity. Our special tests yielded - a percentage of 62% of what a patient of his age and station should - possess. There seems, however, to be no connection between this - reduction of mental capacity and the difficulty with the arm. We - cannot connect the history of alcoholism with the arm trouble. - - “There is some evidence that other stone workers have at times shown - such effects. - - “The patient’s fairly circumstantial account of his difficulty seems - to point to a degree of myalgia or muscular pain in the region of - the forearm when held in a certain position and a feeling of - numbness in the third and fourth fingers. Whether these phenomena - are due to local pressure upon nerves in the upper part of the - forearm due to neuritis, or whether we are dealing with a functional - neuralgic phenomenon is a question. - - “We have applied some special tests for faradic sensibility to all - the fingers of both hands and have found that the fingers of the - right hand are still less sensitive than those of the left, - particularly the thumb and the little finger. This test has not yet - been applied in a sufficiently large number of cases to prove any - difficult point, nevertheless the findings are in line with the - patient’s own circumstantial account of former feelings of numbness - in the third and fourth fingers of the right hand. - - “Obviously, then, our opinion is that there is still to be found - some effect of the disease, whatever it was, which caused the - patient to knock off work. If we had more experience with such cases - and more data with the new test which we have applied, we should - perhaps be inclined to admit the diagnosis of _occupation neuritis_ - and to suppose structural alterations in the nerve trunks - corresponding with the location of the muscular pain and the - anesthesia of fingers and the dulling of electric sense, but in the - present stage of our experience, it is probably wiser to call the - case one of _occupation neurosis_.” - -It is clear that the W. R. in this case was of peculiar value in at -least partially clearing up the findings, yet it must be remembered that -it is a principle of the modern administration of industrial accident -boards and similar organizations that it is the employer’s lookout -whether the employee has syphilis. Recovery can be made as if the injury -were due wholly to an accident. It was not possible however definitely -to prove or disprove a relation of syphilis in the form of a syphilitic -neuritis to the condition in this case. - -The special tests above referred to are the electric sensory threshold -tests of E. G. Martin. - - - =Character change: neurosyphilis.= - - -=Case 95.= Joseph Wilson offered a very serious social problem. He was -the father of two children, and his wife was pregnant at the time of his -admission to the Psychopathic Hospital. He was a husky-looking man of 33 -years of age, but for the past four years he had been deteriorating in -his work; he had been drinking heavily, and finally had stolen to obtain -money for liquor. It was on account of his alcoholism and delinquency, -which were taken as an indication of change of character, that he was -sent to the hospital. - -Examination on his arrival disclosed at once that there was more to the -case than alcoholism, for the =neurological examination= showed that the -pupils were irregular, the right being larger than the left, both -reacting sluggishly to light, and there was an inequality in the -reaction of the two eyes, the left being better than the right. The -tendon reflexes were exaggerated, with ankle clonus on both sides, more -marked on the right. There was also a marked speech defect. Otherwise -the =physical examination= showed nothing of importance. - -The W. R. of the blood and spinal fluid was strongly positive. The -globulin test was strongly positive, the albumin was markedly increased, -there were 74 cells per cmm., and a gold sol reaction of the “paretic” -type. - -A formal =mental examination= did not show very much of consequence; his -memory showed no marked impairment, he was not deluded or hallucinated, -and he had a pretty good insight into his failings. However, he was -somewhat childish, and his irritability was quite marked. Were one to -rely upon the mental signs alone, it is probable that a diagnosis of -chronic alcoholism with deterioration would be made; but in the presence -of the physical findings and the laboratory tests, the diagnosis of -neurosyphilis had to be given. It is obvious that, while the patient was -suffering from a progressive brain disease, and while he did show mental -symptoms, there was not sufficient ground on which to commit him, and -therefore he had to be turned out into the community. As a matter of -fact, he was not prosecuted on account of his theft, because, although -legally responsible, it was felt that his disease was at the basis of -the character change which had led him into difficulties. Further -developments of his relations with society had to be considered, -however. It was possible to get him to discontinue the use of alcohol -altogether, and for nearly a year he has taken no alcoholic liquor and -has been self-supporting. However, his irritability has been very great, -making it very difficult for his wife to live with him, and causing his -sister to break off all relations with him. - -Here, then, is a man with a marked CHARACTER CHANGE as the result of -neurosyphilis, so that it is difficult for him to maintain the usual -social relations. It does not seem possible to remove him from the -community. - - 1. May one speak of general paresis without mental symptoms? If one - considers general paresis a mental disease, of course it cannot - exist without mental symptoms. However, if one considers the - disease as a chronic syphilitic meningoencephalitis characterized - by its pathological anatomy, then one may readily speak of general - paresis although no real evidence of mental symptoms can be - discovered. It would seem that we must take this attitude with our - present conception of brain localization, for it is easy to - conceive of a general paretic process affecting areas which do not - definitely relate to psychic function. And further, such a process - may exist but not be of such a grade as to cause mental symptoms. - - - =The neurosyphilitic’s family should not be forgotten in diagnosis - and treatment.= - - -=Case 96.= The Bornstein family is remarkable. Let us hang the story on -Becky, the mother, an Austrian woman of 43 years, who appears to have -been perfectly well up to within a year. About a year ago, Mrs. -Bornstein began to suffer from severe headaches, which were treated with -apparent success by an osteopath: at all events, Mrs. Bornstein -recovered therefrom in about six months. However, two months later, she -had a convulsion, with foaming at the mouth, blueness of face, and -general muscular stiffening. The convulsion lasted for several minutes. -Again, a fortnight before admission, the patient had five convulsions of -an identical nature in a single night. - -Moreover, since the first convulsion, Mrs. Bornstein’s =mental -condition= has altered and become variable, so that at times she is -excited, at times depressed. She would assert inaccurately that there -was some one in the house, and that she had at different times committed -crimes of a heinous nature. Now and then she would seem to see moving -pictures. Her memory was poor and she seemed to believe that events of -five or six years ago had just happened. - -The pupils were sluggish, the knee-jerks and ankle-jerks were absent, -there was slight ataxia, and there was speech defect. The suspicion of -neurosyphilis was so strong that it seemed surprising that the W. R. of -the blood serum, even after repeated tests and after the provocative -injection of salvarsan, proved negative. However, the spinal fluid -yielded a positive W. R., and a gold sol reaction of the “paretic” type, -together with 12 cells per cmm., and a marked increase of albumin, with -positive globulin. It would seem warrantable to make a diagnosis at -least of syphilis of the nervous system in this case, but it is a -question whether we should be warranted in making the diagnosis general -paresis. - -That the diagnosis is doubtful may perhaps be seen from the variety of -diagnoses in the rest of the family. In the first place, Mrs. -Bornstein’s husband admits syphilitic infection many years before. He -states also that his wife after marriage showed signs of syphilis and -received some treatment, although limited. It is stated also that the -husband himself at this time has a positive W. R. and has stiff pupils -and petit mal attacks. The oldest son, 22 years of age, is confined in -an institution with juvenile paresis. The second son has recently died -at the age of 20 years, receiving a diagnosis of rupture of the aorta. A -third son, 19 years of age, has the appearance of having achondroplasia, -although the proportions of his limbs do not quite correspond with those -of an achondroplast. The fourth son, 17 years of age, is suffering from -caries of the spine. A fifth son, 14 years old, is neurotic and has the -so-called Olympic forehead. The sixth and last son died shortly after -birth of unknown cause. - - - =Neurosyphilitic’s normal-looking family proved syphilitic.= - - -=Case 97.= Walter Heinmas was a draughtsman 33 years of age when he was -brought to the Psychopathic Hospital suffering from mental disease. This -was diagnosed as general paresis, both on account of the clinical -symptomatology and on account of the laboratory findings. In fact, it -was a case of the classical type with marked euphoria and grandiosity. - -As is the routine procedure at the Psychopathic Hospital, in the case of -all syphilitic patients, the family was sent for. This consisted of the -wife and two daughters, aged 9 and 7 respectively. The patient denied -any knowledge of a syphilitic infection. The wife, also, gave no history -of any primary, secondary, or tertiary symptoms; there had been no -abortions, miscarriages, or stillbirths; both children had been born at -term and had been entirely healthy. Examination showed that the mother -had no signs referable to syphilis, and that both the children were -mentally well endowed, with good physique and showing no stigmata of -congenital syphilis. Still the W. R. of all three (the mother and the -two children) was positive in the blood serum. These tests were repeated -several times on the children, with and without injections of salvarsan, -and they remained consistently positive. - - 1. Are these children to be considered congenital syphilitics - despite the absence of stigmata or symptoms? We must consider - these children as congenital syphilitics and candidates for the - group frequently spoken of as _syphilitis hereditaria tarda_. - - 2. What is the frequency of syphilitic involvement in the mate and - children of paretics? In our series, we have found that about 15% - of the marriages where one member develops paresis, result in - sterility; that in 18% abortions, miscarriages and stillbirths - occur; and that in 15% positive W. R. is obtained. We have adopted - the motto: “=The families of paretics are the families of - syphilitics.=” - - - =Neurosyphilis: question of marriage.= - - -=Case 98.= Mr. Jacobs’ wife was admitted to the hospital with a -diagnosis of general paresis. A few weeks after her admission, she died -as a result of her disease. According to our routine, her husband and -the children were examined for evidences of syphilis. - -Mr. Jacobs’ blood serum was found on repeated tests to be positive. He -resolutely denied any knowledge of a syphilitic involvement, but it was -later learned from his brother that about two years before his -marriage—that is, more than 25 years before we saw him—he had acquired -syphilis and had had a very small amount of treatment. - -Mr. Jacobs was put upon antisyphilitic treatment in the form of -injections of .3 gram of salvarsan every two weeks with occasional -intramuscular injections of mercury salicylate. After seven months of -treatment, the blood serum still remained positive. At about this time, -the patient came to us to ask about getting married again. He said that -he was living with his sister, who kept telling him that he was the -cause of his wife’s death, and this was so unpleasant that he desired to -start a home for himself again! - - 1. What advice should be given? It is a general opinion that the - longer the period after the initial infection, the less the - chances of infecting a partner. This chance is further reduced - under antisyphilitic treatment, of which a considerable amount had - been given in the case of Mr. Jacobs. However, when one considers - the trickiness of syphilis and the fact that there is some chance - of infection, which we would apparently overlook if we gave him - permission to marry at this time, the only possible course was to - tell the patient that he should not consider marriage until his - Wassermann had become negative and remained so for some time. The - children in this case were negative. - - 2. What is the physician’s duty to the family of a syphilitic - patient? It is our firm conviction that it is the duty of every - physician to his syphilitic patient, to the patient’s family, and - to the community, to examine the mate and the children for - evidence of syphilis acquired or congenital and to offer treatment - if it is found to be needed. This is one of the chief means at our - disposal today to prevent the late disasters of syphilis, acquired - or congenital, for by such examinations the syphilitic condition - is discovered before lesions have occurred which are irreparable. - We know that the mate and children of a syphilitic patient have - been exposed to syphilitic involvement, and it is our duty as - physicians in possession of such knowledge, and as guardians of - the public health, to investigate such cases, so that if they be - found to have syphilis, steps may be taken to treat them early. - - 3. How much danger is there of causing unhappiness and breaking up - families by this procedure? This question offers a chance for many - theoretical answers. The facts are, however, that in doing this as - a routine for nearly three years and examining several hundred - families, there has been no instance to our knowledge in which a - family has been broken up or grave difficulties have been - encountered by this procedure. - - 4. In what percentage are the mates or children of neurosyphilitics - found to show definite symptoms of syphilis? It is our opinion - that the situation in regard to neurosyphilitics is the same as - for syphilitics in general: That the same laws of attenuation of - virus, and of chance occur here as elsewhere. - -Just as this book is going to press, we have learned that the distraught -Mr. Jacobs, still desirous of starting a home for himself and feeling -entirely well, consulted a physician. This physician took a sample of -blood and had it tested at a competent laboratory, which reported the -blood negative. - -On the strength of this test, the physician felt himself warranted in -recommending, or at least not advising against, Mr. Jacobs’ marriage, -which has probably now taken place. - -Although there is some doubt what ethical relation a state institution -shall maintain with physicians in private practice, we took occasion to -call the attention of our patient’s new counsellor to the fact of the -patient’s neurosyphilis. We noted that the man’s serum had been -constantly positive (Massachusetts State Board of Health findings) in a -score or more of observations. We called attention to the fact that -lumbar puncture had shown positive signs of neurosyphilis, including a -positive W. R., globulin, excess albumin, pleocytosis, and positive gold -sol. These facts, according to a letter received from the private -practitioner in question, have not altered his opinion in the slightest -to the effect that our patient is completely normal and entirely -suitable for marriage. It is clear that he regards the patient as not a -victim of General Paresis. - - 5. What is the significance o the negative observation eventually - obtained in Jacobs’ serum? One’s first thought is to impugn the - accuracy of the laboratory work, but against this suspicion is the - excellent reputation of the laboratory in question, and the - agreement in the majority of its findings with those of the State - Board of Health. It is more likely, as we assured the private - practitioner at whose request the observation was made, that this - negative test was an exceptional and isolated observation such as - is not infrequent in long series of observations, particularly - those made under therapeutic conditions. In so important a matter, - we are inclined to feel that the physician in question should have - resorted to two more observations at intervals before running - counter to the position taken by the hospital. - - - ——many a hard assay - Of dangers, and adversities, and pains. - - Paradise Regained, Book IV, lines 478–479. - - - - - V. SOME RESULTS OF TREATMENT - - -Cases 99–103 show the Variety of Structural Lesions that Treatment has -to face. - - - =SPASTIC HEMIPLEGIA in PARETIC NEUROSYPHILIS (“general paresis”), - showing marked degenerative changes, a condition in which therapy - could be theoretically of very little avail. Autopsy.= - - -=Case 99.= James McDevitt arrived at the Danvers Hospital, July 20, 1906 -(saying that he came to be “thawed out”), and died less than six months -later: January 12, 1907. He was 34 years of age. He had been a -shoe-worker after leaving school, had worked eight years with the -General Electric Co., and had then become a bartender. He had, however, -stopped work in September, 1905, and we may safely say that mental -symptoms had begun insidiously at about that time. His symptoms, if -there were any, had been masked by a heavy alcoholism, but an obvious -change had appeared in November, 1905. The patient lost ambition, smoked -and loafed about his room, and developed speech disorder. He denied -venereal disease, nor was there any superficial evidence of such. - -=Physically=, the patient showed little or no disorder except acne of -the trunk, patches of eczema on the left lower chest, and numerous -brownish scars along both tibiae. - -=Neurologically=, the Romberg position was maintained, but the gait was -very unsteady on attempts to walk a straight line; fingers, tongue, and -face were tremulous, and finer movements were performed with marked -incoördination. No direct or consensual light reactions could be -obtained in the pupils, which were dilated and irregular. - -The condition of the reflexes is important on account of the autopsy -findings. The abdominal and cremasteric reflexes were prompt, and the -knee-jerks equal and very lively. Achilles and normal plantar reactions -were present; there was no clonus; the arm reflexes were very brisk. - - - =COMMON THERAPEUTIC CONCEPTION= - - [M]VP = TYPICAL PARESIS - - MV[P] = TYPICAL CEREBROSPINAL SYPHILIS - - [M]V[P] = TYPICAL SYPHILITIC ARTERIOSCLEROSIS - - (M = Membranes, V = Vessels, - P = Parenchyma, [] = not involved) - - CHART 21 - - -The =mental symptoms= need not detain us. Consciousness was clear; -orientation for time, place, and to some extent for persons, was -imperfect. Arithmetic had been largely forgotten. Handwriting was -irregular and scrawling, and in places unintelligible. Although the -patient claimed that his memory was intact, it was decidedly imperfect. -He remarked that John D. Rockefeller, a Chicago king, was President; the -General Electric Works had almost 50,000 people at work; and in fact -Lynn was one of the largest cities in the state, having over 12,000 -people. The height of patient’s room was estimated at 25 feet. There was -a slight euphoria. There was never any doubt of the =diagnosis= of -PARETIC NEUROSYPHILIS (“general paresis”). - -Five months after admission, slight convulsions developed, after which -the patient was more dull and demented; he became bedridden. More -convulsions followed, leaving the right arm and hand useless. There were -clonic spasms of the muscles of both lower legs. Decubitus developed and -death occurred. - -We may set the total duration of symptoms in the case of James McDevitt -at a little over a year; nor is there any evidence of previous or -prodromal symptoms beyond a total period of about 15 months, unless we -may regard his leaving the General Electric Works to become a bartender -some nine years before death, as a symptomatic change of character. In -any event, it is of note that the =autopsy= showed singularly few -lesions. Death was due doubtless to complications following decubitus, -and there was a slight acute splenitis. The kidneys showed some -parenchymal change. The aorta showed many patches of sclerosis, with -calcification or ulceration throughout its length. These changes were -not characteristic of syphilitic disease. There was considerable -coronary arteriosclerosis and a slight mitral valvular sclerosis. There -was a brown atrophy of the heart muscle, somewhat surprising in a man of -34 years. The =brain= was practically normal, weighed 1200 grams, and -showed convolutions normal in size, relation, and arrangement. There was -no sclerosis grossly evident in the blood vessels. The pia mater -appeared to contain a considerable excess of clear fluid. The calvarium -was of normal thickness and showed diploë and the dura mater failed to -show adhesions. There were no macroscopic signs of lesion in the spinal -cord. - -=Microscopically=, the lymphocytosis, plasmocytosis, and phagocytosis of -the perivascular spaces, (relative?) increase in blood vessels, the -gliosis, and evidence of nerve cell destruction, taken together -warranted the diagnosis of PARETIC NEUROSYPHILIS. It was plain that the -nerve cell destruction was best marked in the _inner layers of the -cortex_. The microscopic study of the spinal cord showed that there was -very possibly a slight sclerosis of the posterior columns in the lumbar -region, but this was so slight that it could hardly be noted in the -myelin sheath stains (Weigert). Very sharply marked, on the other hand, -were the _bilateral pyramidal tract lesions_ in the lumbar and thoracic -regions, less marked at the cervical levels. - -Without attempting to analyze carefully all these findings, it is -interesting to note in this case a foil to the usual spinal cord picture -of paretic neurosyphilis. The spinal cord, ordinarily normal, or perhaps -more usually affected by a degree of posterior column sclerosis, in this -case showed such well marked pyramidal tract sclerosis that we may -perhaps place the case in a subordinate group of SPASTIC PARETIC cases -of NEUROSYPHILIS. The source of the pyramidal tract disease lodges, -however, in the cortex cerebri itself, being part and parcel of the -lesions mentioned above as affecting more directly the inner layers of -the cortex. Many of the so-called giant, or Betz, cells had undergone a -complete destruction. It will be remembered that clonic spasms of the -muscles of the legs appeared in the fortnight preceding death, and that -there had been convulsions for about six weeks before death. There was -no evidence at the autopsy why the right arm and hand should have become -useless, whereas the left upper extremity remained normal. This case, -then, forms an exception to the ordinary paretic neurosyphilis group in -that the brunt of the microscopic process was borne by the inner layers -of the cortex. The cells of origin of the pyramidal tract fibres had -been cut in this lesion, and had become subject to partial or complete -destruction. Note, however, that the lesion remained a microscopic one -and that the marked convulsions were not related to gross lesions, -thereby following the rule for paretic seizures. - -[Illustration: - - Bilateral pyramidal tract sclerosis, secondary to destruction of large - motor (Betz) cells of motor (precentral) cerebral cortex—paretic - neurosyphilis. -] - -From the standpoint of possible treatment, it is of course true that few -organs of the body showed grave lesions save in the calcified and -ulcerated aorta, which conceivably might have become quiescent under -appropriate treatment. But, although the brain was almost if not quite -normal in the gross, and although its membranes showed practically no -lesion, treatment would not have been very promising. To be sure, the -exudate might have been cleared away if the spirochetes responsible -therefor had been destroyed by treatment. Yet the destruction of the -giant cells of origin of the pyramidal tract fibres to such an extent as -in this case could hardly have been compensated for by any known -process. So far as we are aware, the destruction of considerable numbers -of the smaller association elements of the brain is subject to the -compensation of other elements of the nervous system, which conceivably -might be re-educated or newly educated to perform certain processes. The -histological picture in a case like that of McDevitt accordingly leads -to the hypothesis that so well marked a spastic paresis, even in the -presence of otherwise favorable signs, would be of especially baneful -portent therapeutically. - - - =NEUROSYPHILIS with total duration of symptoms twenty-two days. The - comparatively MILD BRAIN LESIONS, INFLAMMATORY AND NOT DEGENERATIVE - in type, suggest the possibility that therapy might have been - successful. Autopsy.= - - -=Case 100.= Jacob Methuen, 35, was a carpenter from Newfoundland. He was -working upon a certain Thursday with his brother, who noticed that Jacob -was lifting the tools about in an unusual manner and talking strangely -to his fellow workmen. He fell asleep, going home in the street car, and -said afterward that he felt dazed and peculiar. He talked all kinds of -nonsense to his wife upon arrival. Methuen remained in bed next day, -fancying he was going to die, calling his family together, and saying -good-bye to them. He remained in bed all through the next day, but on -Sunday appeared better,—more active, and in fact quite natural. He slept -only an hour Sunday night, calling to his wife that it was time to get -up. On Monday he began to be irritable to his wife, and accused her of -flirting with his brother and intending to elope with him. He struck his -wife several times, and when two brothers came to watch him, accused -them both of trying to steal his wife, and struck them. Tuesday he -remained in bed until late at night, when he arose and tried to assault -the family. - -It seems that another brother of the patient had died but eleven days -before his admission to the hospital and five days before the onset of -Jacob’s symptoms. Since his brother’s death he had been dwelling upon -religious matters, and in fact the day after his brother’s death, he -waked up during the night, saying that he was too happy to sleep, that -he heard the Master’s voice, and at times the devil’s voice; that there -was to be a modern miracle and his spiritual life from now on would be -different. - -Eleven days after admission to the hospital, Methuen died, making a -total duration of symptoms, beginning at his brother’s death, of 22 -days. - - - =NEUROSYPHILITIC LESIONS= - - =LESIONS OF THE SECONDARY PERIOD= - - (1) INTERSTITIAL ENCEPHALITIS OR MYELITIS - (“meningitis”) - (2) PARENCHYMATOUS ENCEPHALITIS OR MYELITIS - (“encephalitis,” “myelitis”) - - =LESIONS OF THE TERTIARY PERIOD= - - (1) CHRONIC INTERSTITIAL ENCEPHALITIS OR MYELITIS - (“gummatous meningitis”) - (2) CHRONIC PARENCHYMATOUS ENCEPHALITIS - (“dementia paralytica”) - (3) CHRONIC PARENCHYMATOUS MYELITIS - (“tabes dorsalis”) - - “We have shown that the central nervous system is affected by - syphilis at the same periods and in the same manner as are other - internal organs. In addition the ‘parasyphilitic’ lesions are also - of a typically syphilitic nature, being directly comparable to the - parenchymatous affections found elsewhere in the body. They are - ‘tertiary’ lesions differing only from the so-called ‘gummatous’ - processes in the central nervous system in that their localization - is in the parenchyma while that of the latter is in the - interstitial tissues.” - - McIntosh and Fildes, 1914 - - CHART 22 - - -=Physical examination= showed a man 5′ 9″ tall, weighing 149 pounds, -rather pale and poorly nourished, with a somewhat enlarged heart and no -evidence of venereal disease. - -=Neurologically= there was a slight facial and digital tremor, but -otherwise no symptom or reflex disorder except that the tendon reflexes -were generally increased; the knee-jerks especially were very vigorous. -There was no speech defect. His handwriting was fairly legible. - -The patient was very noisy and uncontrollable, tearing clothing and -biting, striking the attendants, refusing food, talking rapidly, loudly, -and incoherently. His manner suggested auditory hallucinations but no -positive evidence of these was obtained. His clothes could not be kept -on him. The following is a sample of his reactions: As the examiner -entered, the patient stood stark naked and glaring. He started to talk -as follows: “Methuen,—I, Saviour, come to life and ought to die—— Now I -lay me—— Now I die—— The heart beats—— No, I ain’t going to die—— I am -going out soon. I want my clothes—— You can’t hold me; I am strong.” -(Struggles violently with the attendants.) “I am God. God. I know you, -you can’t fool me.——I am here——I can do you all. How many doctors are -there here?” (Struggles violently. Looks at examiner.) “He is writing -something. Sir, you can’t fool me in a million years. Do you understand -that, doctor? You can’t fool me. Write all the prescriptions you want -to. Ten thousand years; you hear that, doctor? Ten thousand years. You -can’t fool me; ten thousand years. Ten thousand years are but a day for -the spirit of the Lord,” etc., etc. - -The excitement continued unabated. The patient became entirely -disoriented, and finally almost unable to move. He lay in bed trying to -talk and muttering broken gibberish, still attempting to struggle to the -extent of his limited strength. - -The =autopsy= showed no sign of lesion (brain weight 1380 grams), -unless, perhaps, the occipital regions were slightly firmer than the -rest of the brain. Death was apparently due to a bilateral pneumonia, -bronchial type. There was an acute splenitis. The only chronic lesions -of the body were a bilateral chronic adhesive pleuritis and a slight -sclerosis of the arch of the aorta. - -[Illustration: - - Paretic neurosyphilis (“general paresis”) macroscopically normal, - microscopically characteristic. Treatment does not have to face - massive destructive processes already complete. -] - -=Microscopically= there was a distinct though mild degree of -lymphocytosis of the perivascular spaces in many regions. Somewhat -extended _search failed to reveal plasma cells_, and it is certain that -if plasma cells existed, they must have occurred in very small numbers. - -Here, then, was a case of DIFFUSE NEUROSYPHILIS (with brain picture -consistent) with symptoms lasting but 22 days and with an appearance of -acute mania. It is to be noted that this case arrived at the hospital on -the eleventh day of his symptoms. The case occurred long before the -development of the temporary care system in Massachusetts. It is -probable, or at any rate possible, that he would have been brought to -the hospital far earlier, say, upon the sixth day, had the modern -temporary care system been installed at that time. The routine W. R. -examination would then have been made. With more effective hydrotherapy, -it is possible that the patient’s life might have been prolonged and -that treatment might have been effective. So far as we can see, the case -would have been a singularly good one for treatment despite the -practical unmanageability of the case under ordinary home treatment, and -even under hospital conditions where modern hydrotherapeutic appliances -are not available. - - - =PARETIC NEUROSYPHILIS showing very MARKED MENINGITIS, suggesting - that therapy might have produced improvement. Autopsy.= - - -=Case 101.= We report the case of John Baxter, a boat tender of 48 -years, because this particular victim of PARETIC NEUROSYPHILIS seems to -have had the most markedly thickened and altered meninges in our whole -series. Of course, the therapeutic theory upon which we now proceed in -the treatment of non-paretic and possibly even of paretic neurosyphilis -is that, other things being equal, the meningitis can be removed by -treatment, or in the course of treatment, so that the degree of ultimate -recovery rather depends upon the condition of the brain substance itself -than upon the condition of the meninges. Here, at all events, is an -example of the most highly meningitic neurosyphilis that we have seen. - -Curiously enough, two of Baxter’s brothers were also patients at the -hospital at which Baxter died, and a number of the other members of the -family are reported as “nervous.” It seems that at 35 Baxter began to -drink heavily and had never given over the habit of alcoholism. - -Upon admission to the hospital, in fact, he showed a sufficiently -typical picture of delirium tremens. His consciousness was clouded, he -had vivid visual hallucinations and was very apprehensive. - -His heart was enlarged to the left; the pulse, 120, was of increased -tension and irregular; there was peripheral arteriosclerosis; the teeth -were poor; the tongue coated; and the mouth foul. The urine showed a -trace of albumin and rare hyalin casts. - -=Neurologically=, the gait was somewhat unsteady, there was an extreme -tremor of the whole body, including the tongue and fingers. The Romberg -sign was negative although there was marked swaying. The pupils were -equal and reacted normally; the knee-jerks were markedly exaggerated, -the arm reflexes somewhat exaggerated. The remainder of the reflexes -upon systematic examination were negative. - -[Illustration: - - A high degree of chronic leptomeningitis. Pia mater thick, opaque, - concealing brain. In paretic neurosyphilis (“general paresis”). -] - -Upon arrival, Baxter was put to bed, but he barricaded his door and -fought with the attendants. The tremor increased, the hallucinations -were both visual and auditory. After a few days, Baxter became so weak -that he could not move. He refused to eat for a period of two days, -explaining in whispers that he did not wish to be poisoned; a voice had -told him the food was to be poisoned. The voice was of agreeable tones, -probably belonging to a lady; it did not speak, but sang to him. The -clouding of consciousness failed to clear up, as in delirium tremens, so -that, though patient was admitted March 3d, it was hardly possible to -speak freely with him until more than a month later, April 9th. A -good-natured conversation would run as follows: - - “What is your name?” “Baxter.” - “First name?” After long pause, “Don’t know.” - “John?” Pause of 7 seconds, “Yes, I think it is.” - “How old are you?” “There are legs——there is a body——up to here——” - “Say the alphabet.” Term not understood. - “Say the _a_, _b_, “Oh yes; a, b, c, d (long pause), e, f; I cannot - _c_.” say it, I did not have much education; I am not - intelligent.” (In point of fact, the patient had - a good grammar-school education, and had long - worked as a clerk in a grocery store, with good - wages.) - There was some - speech defect. - -Soon the hallucinatory phase passed, and the patient remained in a -cloudy and disoriented state, inaccessible, rarely speaking, and -gradually failing physically. Death occurred about three months after -admission (pulmonary symptoms). - -In estimating the duration of the process in John Baxter, we must take -into account that he left the grocery business and became a hard-working -but poorly-paid boat tender at about 35 years, at the same time that the -alcoholic habit began. - -The =autopsy= showed that death was due to bronchopneumonia with -pleurisy. There were in the body a variety of chronic lesions, such as -gastritis, colitis, epididymitis, splenitis, parietal and valvular -endocarditis, prostatitis, chronic appendicitis, and some mesenteric -lymphnoditis. The heart was somewhat hypertrophied. There was a slight -diffuse nephritis with cysts, emaciation, and decubitus. The calvarium -was thick and somewhat dense. The dura was thickened and adherent, and -the pia mater,—as above stated, the most thickened and altered pia mater -in our series,—is described as everywhere thickened, of a brownish gray -and white color, especially over the vascular lines, and as showing -small white areas of deeper thickening scattered over the surface, but -most markedly over the sulci, and not as a rule over the crowns of the -gyri. There were also yellowish brown spots with a suggestion of fibrin -over the lateral aspects of both hemispheres. The vessels at the base -were not remarkable in the gross. The brain weighed 1220 grams, and -appeared to be of darker color than usual. - - - =Some cases of PARETIC NEUROSYPHILIS (“general paresis”) have so - much BRAIN ATROPHY that it is not possible to expect much - improvement through antisyphilitic therapy.= - - -=Case 102.= Theodosia Jewett, dead at 58 years, showed the most -remarkably wasted brain in a long series of victims of paretic -neurosyphilis. We present her case to emphasize what therapy must face -in certain instances, but would recall the fact that exceedingly few -such wasted brains have come to our attention in cases dying in the -institutions of Massachusetts. - -Mrs. Jewett, a housewife, whose parents died of shock, and one of whose -two brothers also died of shock, was a normal child and schoolgirl, and -worked as dressmaker until she was married, at 24, to a grocer, by whom -she had two children. At the age of 46, Mrs. Jewett began to suffer from -so-called “nervous prostration.” The attack lasted some two years, but -there were no psychotic symptoms beyond worry and insomnia. The -menopause occurred at 52, at which time the first signs of psychosis -appeared, namely, a forgetfulness concerning familiar matters, such as -sewing, cooking, and the like. At 55, this amnesia had become so marked -that Mrs. Jewett could neither write nor tell time. She, however, was a -perfectly quiet and easily manageable patient, often subject to -drowsiness in the day. - -Six months before her admission to the hospital, she began to suffer -from insomnia, failed to recognize her surroundings, and had a number of -crying spells. Restlessness had begun a month before admission; auditory -hallucinations developed in the form of imaginary conversations with -dead persons. A certain loquacity set in, and for a week before -admission, Mrs. Jewett became somewhat resistive. - -=Physically=, the patient was sallow, poorly nourished, with pale mucous -membranes, peripheral arteriosclerosis, no teeth, muscular feebleness, -tremor of hands and tongue, and active knee-jerks. =Mentally=, the -patient was depressed, talked to herself, assumed a supplicating -position, suddenly altered her attitude, and was very tremulous. Her -talk was low, mumbling, and incoherent, for the most part composed of -answers to her own questions. Sometimes there was a curious difficulty -in speaking, such that the lips moved but no sound emerged; but for the -most part there was no difficulty in uttering words. The patient either -could or would not write. Only when the attention was secured by -speaking to her sharply was she apparently able to understand questions, -and the answers to these sharp questions came spasmodically and as if -interrupting her own thoughts. Nor was it ever possible to obtain a -repetition of the same answer. - -The patient died in exhaustion, with pulmonary symptoms three weeks -after admission. - -The =autopsy= which was performed 3½ hours after death showed the -following points of interest: - -The heart weighed 210 grams. There was marked thickening of the aortic -valve. The coronaries were slightly thickened. - -The lungs were slightly adherent to the chest wall at the apices and -posteriorly. The right lung was consolidated in the lower two lobes -posteriorly and the bronchi exuded pus; the left lung was not -remarkable. There was a chronic splenitis. - -The liver showed fibrous changes, was a brownish-red in color, mottled -with yellow. - -Combined weight of the kidneys 195 grams. The capsules were adherent, -tearing the cortex when stripped. - -The diploë were well marked. The dura was not adherent. The pia was -slightly thickened and raised from the cortex by a large amount of -subpial fluid (showing atrophy of the cortex). The pial vessels were -injected, more markedly so on the left side. The arachnoid villi were -reported as moderately developed, especially along the longitudinal -fissure. - -The brain was rather soft in all regions. The weight was 1045 grams. -According to Tigges’ formula the weight of the brain should be -approximately 8 times the body length in centimeters. The length in this -case was 158 cm., therefore, according to this formula the weight of the -brain should have been 1464 grams. The difference of more than 400 grams -is evidently a loss to be accounted for by atrophy, a very heavy loss. - -[Illustration: - - Perivascular exudate (low power) in atrophic cortex from case of - general paresis. -] - -[Illustration: - - Markedly atrophic cortex, but without local perivascular exudate. -] - - 1. Was the “nervous prostration” at 46 of syphilitic origin? One - cannot give a categorical answer to this question. The high - incidence of shock in the family suggests poor stock in which a - psychoneurosis is not an unusual phenomenon. The presence of - syphilis might act as a debilitating factor or _agent - provocateur_, if it were not to cause any demonstrable brain - lesion. As pointed out in the case of Harrison (9), however, it is - not unusual in neurosyphilis to find a history of symptoms - occurring years before the final breakdown and symptoms frequently - not recognized as of neurosyphilitic nature. - - 2. Does the fairly long duration of the psychosis (at least 3 years) - explain the marked atrophy? Cases having symptoms even much longer - than three years at times show relatively very little atrophy, so - that this factor in itself cannot be said to explain the - tremendous destruction of tissue. - - - =The THERAPY OF NEUROSYPHILIS has to face not merely variations in - the degree of brain wasting and in the degree of meningitis, but - also variations in the topographical distribution of lesions. - Autopsy.= - - -=Case 103.= To bring out this point we may instance the case of Alfred -Weed, a victim of PARETIC NEUROSYPHILIS, dying at the age of 48 years -after a course of about seven years. The following is an abstract of the -clinical history: - -A. W. suffered from lues some 24 years before his death at Danvers -Insane Hospital in 1907. There is no account of insanity in his family. -The patient had been undergoing mental changes for six years before -death. At the age of 42 began to take interest in socialism and -spiritualism. Would become excited at times and was observed to talk to -himself. At times it seemed that he was reacting to visual -hallucinations. After eight months he became depressed and apprehensive -and developed delusions of poisoning. - -On admission to the Danvers Insane Hospital in June, 1902, the subject -was found to be ataxic, falling in the Romberg position. Pupils were -equal but of pin-point size. There was tremor of the facial muscles. The -knee-jerks were absent. Speech was ataxic. Memory defective. Depressed. -Thought he was to be punished. Refused to eat. - -Later in the year of admission, patient became more negativistic. He -refused to have his clothes brushed. His answers were slow. Mental -arithmetic was correctly but slowly done. During January, 1903, the -patient was apt to be active and talkative for a time, and then his -attitude would suddenly change to one of silence, resistivement and -untidiness. From time to time he would be querulous and sulky. In -August, 1903, the patient became weaker and could walk with assistance -only. Paralysis developed in the left facialis region and in the left -external rectus. Pupils were still small, but the left had become -smaller than the right. Light reaction tests unsatisfactory. Knee-jerks -could not be obtained. - -In December, 1903, the patient was untidy and helpless, lying with his -thighs and legs flexed. The limbs were spastic on passive motion. In -1905, the pain sense of the legs was found lost and the pupils were -small and stiff. The protruded tongue was deflected to the right. The -right labial fold was more prominent than the left. Knee-jerks remained -absent. Ataxia was extreme. - -The =Neurological Findings= may be summed up as follows: - - 1. Ataxia of the legs. - - 2. (Probable) Diminished sensibility in the legs. - - 3. Pupils small and stiff. Left smaller than the right. - - 4. Paralysis of left facialis. - - 5. Paralysis of left external rectus. - - 6. Tongue protruded to right. - - 7. Right elbow jerk greater than left. - - 8. Knee-jerks absent. - -The cause of death was bronchopneumonia. The walls and valves of the -heart showed a few chronic changes. There was a marked splenitis and an -atrophy of the liver. The kidneys showed numerous depressed scars. The -arch of the aorta was somewhat sclerotic. The following is a full -description of the head findings which we present by way of comparison -with other cases. Note especially the cerebellar, dentate, and olivary -changes. Note also the fact that palpable sclerosis is demonstrable over -a far larger area than atrophy, so that we may almost safely conclude -that the process of induration sometimes precedes that of atrophy. One -gets the impression from the extent of visible atrophy and tangible -induration in this case, that a possible therapy would have not merely -to clear the perivascular spaces of cells and spirochetes, but would -also need to arrest the indurating and wasting process. Nor could any -therapy deal effectively with the superior frontal and upper central -atrophy of the cerebrum of this case, or with the olivary and cerebellar -lesions. - -=Head=: Hair thin at vertex. Scalp normal. Calvarium thin and dense. -Dura mater slightly adherent to calvarium at vertex. Sinuses normal. -Arachnoidal villi well developed. Pia mater of anterior and central -regions contains an excess of fluid. The pial veins well injected. - -The =pia mater= exhibits one unusual lesion: Faintly yellowish brown -spots of miliary and slightly larger size are scattered irregularly in -clusters over the vertex. These miliary pial macules are observed -especially over the posterior third of the left superior frontal gyrus -(a group of twelve or more). Two are seen in the pia mater of the right -superior frontal gyrus. One is seen in the upper part of the left post -central gyrus. The upper end of the right postcentral gyrus contains -three macules. - -Besides these brownish macules, the pia mater also shows focal white -thickenings which resemble the more frequent appearances of chronic -fibrous leptomeningitis. The white thickenings are of irregular size but -are, as a rule, larger than the macules above mentioned. They occur, as -a rule, over the sulcal veins and are most frequent in the anterior -region. - -The vessels at the base are normal. There is no evidence of pial -thickening at the base of the brain. =Brain= weight, 1265 grams. There -is visible atrophy of both superior frontal gyri and of the upper -two-thirds of both central gyri. The extent of palpable sclerosis -surpasses that of visible atrophy. Palpable increase of consistence is -shown by the prefrontal, orbital (more marked on left side), frontal, -central, hippocampal and occipital regions. The temporal cortex is of -normal or slightly reduced consistence. - -Section of the cerebral cortex shows everywhere preservation of the -cortical markings. The sclerosed areas show a diminution in depth of the -cortex, which is more marked in the left prefrontal region. The white -matter of the centrum semiovale of the prefrontal and occipital regions -on both sides shows an increase of consistence. The cerebellar cortex -also shows variations in consistence. The clivus and lobus cacuminis and -the posterior half of the inferior surfaces of both cerebellar -hemispheres are firmer than normal. The laminæ of the left clivus are a -trifle narrower than those of the right. There is visible extensive -atrophy of the laminæ on both sides of a fissure in the middle of the -left lobus cacuminis. In the coördinate portion of the right cacumen -there is a similar process which is less marked. The dentate nuclei are -firm. The olives show an increase of consistence, equal on both sides. -The left olive shows on section a crowding together of its folds in the -middle part of the upper limb. - -Spinal cord was not remarkable. - -=Summary=: - - Adhesive pachymeningitis - Chronic fibrous leptomeningitis - Miliary pial macules - Cerebral atrophy - Cerebral sclerosis - Cerebellar atrophy and sclerosis - Bronchopneumonia - Chronic splenitis - Nephritis - Aortitis - - - =It is generally recognized that DIFFUSE NEUROSYPHILIS - (“cerebrospinal syphilis”) frequently is cured through - antisyphilitic therapy. Example. Mental improvement, in one month; - recovery from paralysis, ten months.= - - -=Case 104.= John Edwards, a man of 28 years, well developed and -nourished, with general enlargement of glands and skin lesions, came to -the hospital in a stuporous condition, with evidences of a complete -hemiplegia. - -According to the wife, Edwards had had a chancre of the lip about a year -before, for which he had been treated with an intravenous injection, -presumably of salvarsan, and also presumably with mercury. The lip -lesion had then disappeared. For a month before admission, Edwards had -had headache and dizziness, for which he was given pills and drugs. -There had also been difficulty with speech and numbness of the left arm -as far up as the elbow, but this paresthesia had quickly disappeared. -The hemiplegia was of only a few days’ duration. After a feeling of -nausea and vomiting, the patient had fallen with left-sided paralysis. -Afterwards, he had shown mental peculiarities, eventually becoming -noisy, hard to manage, and appropriate for hospital care. - -The =physical examination= showed a variety of increased reflexes, -including ankle clonus on the left side. - -The question might arise whether this case was one of hemorrhage or -thrombosis, and the facts about the onset of the hemiplegia are -inadequate for a decision. However, at so early an age, the probability -of syphilis is large and the history of labial chancre was quite -suggestive. If we may conclude neurosyphilis, the diagnosis of -thrombosis rather than rupture of blood vessel is likely. The laboratory -tests bore out the diagnosis since the W. R. of serum and fluid both -proved positive; the gold sol reaction was syphilitic; there were 176 -cells per cmm.; there was excess albumin, and a positive globulin -reaction. - - - =NON-PARETIC NEUROSYPHILIS= - - =DIFFUSE NEUROSYPHILIS, - MENINGOVASCULAR PARENCHYMATOUS, - CEREBROSPINAL SYPHILIS= - - CASES SYSTEMATICALLY TREATED 13 - CLINICAL RECOVERY, C.S.F. NEGATIVE 11 - UNIMPROVED 1 - UNIMPROVED, BUT C.S.F. NEGATIVE 1 - - MASSACHUSETTS COMMISSION ON MENTAL - DISEASES, - _November, 1916_ - - CHART 23 - - -The outcome in such a case is dubious. If death does not occur soon, -recovery is not impossible under treatment. At all events, a -considerable improvement is likely. - -Edwards was given bi-weekly injections of salvarsan, intramuscular -injections of mercury salicylate, and doses of potassium iodid, -averaging 100 grains, three times a day. Under this treatment, he slowly -recovered and became mentally clear after a few weeks. The paralysis -seemed complete and permanent. Even after three or four months, there -was absolutely no change in the condition, and Edwards was quite unable -to move either arm or leg. Meanwhile, the spinal fluid had become -practically negative to all tests. - -_Treatment_ was somewhat optimistically continued and was _rewarded at -the end of ten months_ with marked improvement such that the patient was -able to stand on the paralyzed leg and move the arm to a certain degree. -This improvement is still continuing. The spinal fluid and the serum -have remained negative to laboratory tests. - -Note: A period of six months is commonly regarded as that period in -which improvement in paralysis is to occur if there is to be any -improvement. There was certainly not the slightest improvement in the -paralysis of this case before eight or nine months of treatment had -elapsed, and it took ten months to secure the marked improvement -mentioned. - - 1. What is the significance of the prodromal symptoms? The headache - and dizziness should have been viewed with great gravity. They are - characteristic in MENINGOVASCULAR NEUROSYPHILIS. - - Moreover in this case there had also been difficulties with speech - and other transient symptoms which should have called attention - far earlier to the possibility of neurosyphilis. - - 2. What is the significance of the high cell count: 176 per cubic - millimeter? Such high cell counts are frequent enough in diffuse - neurosyphilis, but low cell counts are frequent also. But although - the high cell count taken alone is of lesser significance, the - fact that the high cell count in this case is associated with a - “syphilitic” gold sol reaction is of far greater significance for - diagnosis. These associated findings are characteristic of - meningovascular neurosyphilis. - - 3. What kind of recovery may be expected in successful examples of - treatment in meningovascular cases? Recovery with defect. It will - be noted that ten months elapsed before any marked improvement - occurred on the paralyzed side. We could not expect a complete - recovery from this paralysis. - - 4. Was inadequacy of treatment following the chancre responsible for - the early cerebrospinal involvement? In this connection one must - remember that such neural involvements occur occasionally even - during active treatment (neurorecidives). The discontinuance of - treatment after a short period, in this case less than a year, is - always a risk to say the least. And this is true even though the - W. R. becomes negative, for trouble of a neurosyphilitic nature - may occur later; this when both blood and spinal fluid have - previously been found negative. The old rule of following and - treating a syphilitic for several years despite the disappearance - of symptoms is still a good rule. - - - =The results of systematic, intensive, intravenous salvarsan therapy - in atypical neurosyphilis (cases not certainly paretic, tabetic or - the common types of meningovascular neurosyphilis) may be in our - experience as good as the results of treatment in common - meningovascular cases: example.= - - -=Case 105.= Henri Lepère, a machinist, 48 years of age, came voluntarily -to the Psychopathic Hospital for a gradually failing memory and -inability to work. He had had indigestion for four years (epigastric -distress, nausea, no vomiting). He was still suffering from epigastric -distress and from headaches. At times he had had difficulty in walking. - -=Physically=, Lepère looked older than he was; he was very poorly -developed and nourished, and seemed very weak. There was a slight -visceroptosis. - -=Neurologically=, there was considerable speech defect, particularly -well marked in test phrases. The pupils were contracted and gave the -Argyll-Robertson reaction. Neurologically there were no other signs. - -=Mentally=, there was a depression with worry; but it was a question -whether these phenomena were not entirely natural. The special complaint -was of failing memory. - -The Argyll-Robertson pupil also _prima facie_ signifies neurosyphilis. -Lepère, in fact, admitted syphilitic infection at 23. The gastric -symptoms at once suggested tabes. The knee-jerks and ankle-jerks were, -to be sure, preserved; however, this is not very unusual in tabes. The -amnesia and aphasia naturally suggested paresis. Without resort to -laboratory findings, accordingly, the diagnosis of taboparetic -neurosyphilis (“taboparesis”) was suggested. - - - =EFFECT OF EARLY TREATMENT ON THE DEVELOPMENT OF NEUROSYPHILIS= - - TOTAL CASES 4134 - DEVELOPED GENERAL PARESIS 198 = 4.8% - DEVELOPED TABES DORSALIS 113 = 2.7% - DEVELOPED CEREBROSPINAL SYPHILIS 132 = 3.2% - ——————————— - 443 = 10.5% - - =EFFECT OF TREATMENT= - Repeated - None 1 course energetic - NUMBER OF CASES 100 134 924 - DEVELOPED G.P. 25 = 25% 31 = 23.1% 30 = 3.2% - DEVELOPED TABES 11 = 11% 16 = 11.9% 25 = 2.7% - DEVELOPED C.S.S. 3 = 3% 21 = 15.6% 71 = 7.6% - - Poorly Better - treated treated - 1880–84 1895–99 - NUMBER OF CASES 617 1139 - DEVELOPED G.P. 60 = 9.7% 37 = 3.2% - DEVELOPED TABES 22 = 3.5% 16 = 1.4% - DEVELOPED C.S.S. 15 = 2.4% 28 = 2.4% - - MATTAUSCHEK AND PILCZ - - CHART 24 - - -The serum W. R. proved positive, but the spinal fluid W. R. very -slightly so (yielding only moderate reaction with 1 cc., 0.7 and 0.5 -cc., and a negative reaction with 0.3 and 0.1 cc.). Globulin was -moderate, and albumin was found in only moderate excess. There were 21 -cells per cmm. in the spinal fluid. The gold sol reaction was that which -we regard as typical of syphilis or tabes. If we were to rely upon the -weakness of the fluid W. R. and the nature of the gold sol reaction, we -should be inclined to favor the diagnosis of DIFFUSE NEUROSYPHILIS -(“cerebrospinal syphilis”) rather than resort to the diagnosis of -paretic neurosyphilis. - -Salvarsan treatment was attended by the rapid disappearance of headaches -and gastric symptoms and by a rapid gain in weight and feeling of -well-being. Salvarsan was continued twice a week for two months, -whereupon Lepère returned to work. He has been successfully at work now -for seven months without return of symptoms. Four months after beginning -of treatment, the spinal fluid was examined and found entirely negative. -Nevertheless, the serum W. R. has remained positive despite eight months -of salvarsan treatment. - - 1. What is the meaning of the titrations in the spinal fluid - Wassermann reaction? When Plaut originally applied the Wassermann - reaction to spinal fluids, he used 0.2 of a cc. of spinal fluid. - With this amount of fluid he found that cases of general paresis - gave a positive reaction in about 100% of the cases while this - positive reaction was only given by 40 to 60% of the cases of - cerebrospinal syphilis and tabes dorsalis, hence he promulgated a - differential point that a negative reaction in spinal fluid - indicated that the case was not general paresis. Hauptmann later - showed that if 1 cc. of spinal fluid were used, a positive - reaction would occur in practically 100% of the cases of general - paresis, cerebrospinal syphilis and tabes. Therefore, at present, - we use the different titers of spinal fluid from which we draw the - following conclusions: If the reaction in the untreated case is - negative with 0.1 and 0.3 of a cc. and positive with the 0.5, 0.7 - and 1 cc. dilutions as in the case of Lepère, we are probably - dealing with non-paretic neurosyphilis. With this method of - titration we are also better able to watch the progress of - treatment as the dilutions of 0.1 and 0.3 cc. become negative - first. - - 2. How soon can one expect improvement after commencement of - salvarsan therapy in cases of diffuse neurosyphilis? The time - relation of results in treatment varies with each individual case. - In the case of Lepère gastric symptoms that had been present for a - number of months disappeared as if by magic after the first - injection of salvarsan. As a rule, it is true that the more acute - the symptoms the quicker their disappearance but this does not - hold for all cases, as in this particular instance the - long-standing symptoms disappeared very rapidly. The symptoms - often disappear very much more rapidly than the laboratory, tests - change. - - 3. How can the mental symptoms (depression and failing memory) of - which patient complained be explained? In the first place, as has - been stated, it is doubtful if these are more than subjective and - the result of the patient’s feeling of discomfort and pain. - However, it is also possible that there may be intracranial - involvement of the meninges or of the brain itself. And, if such - were the case, the improvement might be the result of the - treatment. - - - =The Argyll-Robertson pupil should not be used as a basis for a - necessarily bad prognosis if treatment can be given.= - - -=Case 106.= Frederick Stone was a business man of large interests. He -had been in the hands of physicians for several years for a variety of -disorders such as renal, respiratory, cardiovascular, and so on. No -suspicion of syphilis had apparently been uttered by the physicians -despite the fact that Mr. Stone readily stated that he had had a chancre -thirty years before, and that he had received several years’ treatment -of mercury and potassium iodid by mouth. - -It appeared that a few years ago he had begun to have trouble with his -nose, which was cauterized and operatively interfered with without -satisfactory results. This nasal condition had later been diagnosticated -as gummatous, and had improved considerably under a mild antisyphilitic -treatment. However, this nasal condition had been considered and treated -quite separately from the remainder of Mr. Stone’s troubles. - -What brought him to attention was a sudden diplopia with ptosis. There -was a paralysis of the external rectus of the left eye, as well as a -drooping of the lid on this side. The left eye was much inflamed. The -diplopia greatly bothered the patient, and there was also considerable -pain in the left frontal region, confined chiefly to the distribution of -the first division of the trigeminal nerve. According to the patient -this headache was periodic. There was considerable tenderness to -pinprick over the area and a diminution of sensory discrimination of -fine touch. Both the pupils failed to react to light. - -The remainder of the neurological symptomatic examination was -surprisingly clear of disorder, nor was there anything in the history -suggestive of tabes. There was ozena as well as evidence of the -operative work upon nares and throat. Possibly the arteries were -slightly hardened; blood pressure was 165 systolic. There was a large -trace of albumin, and there were numerous hyalin casts in the urine. - - - =PARETIC NEUROSYPHILIS= - - =(GENERAL PARESIS)= - - Cases systematically treated 50 - - CLINICAL REMISSIONS 34 68% - C.S.F. ALTERED TO NEGATIVE 4 8% - C.S.F. ALTERED TO WEAKER 16 32% - C.S.F. UNALTERED 14 28% - - CLINICALLY UNIMPROVED 16 32% - C.S.F. WEAKER 7 14% - C.S.F. UNALTERED 9 18% - - MASSACHUSETTS COMMISSION ON MENTAL DISEASES - NOVEMBER, 1916 - - CHART 25 - - -=Mentally=, there was a degree of depression and worry hardly out of -keeping with the general situation. Despite the preservation of memory, -Mr. Stone failed to do rather simple arithmetical calculations; this was -the more remarkable as in his business he had to handle figures a great -deal and had been doing so until recently. There was a slight tremor in -his writing, as well as a certain difficulty in enunciating test -phrases. Insomnia, irritability, and a feeling of nervousness and of -being tired out, completed the picture. - -A suggestion for diagnosis would be classically offered by the -Argyll-Robertson pupils. Should not a patient with the Argyll-Robertson -pupils have either tabes or paresis? However, in favor of tabes, besides -the pupil, are to be counted merely the troubles with the eyes. In the -direction of paresis we have to consider speech defect, to say nothing -of less definite symptoms such as insomnia and increased irritability. - -We are inclined to think, however, that the disease in this case is -meningovascular. This diagnosis is suggested by the cranial nerve -palsies and by the headache. Headache is much more rarely a phenomenon -in the paretic type of neurosyphilis than in the meningovascular type. - -In point of fact, the spinal fluid phenomena bore out the diagnosis of -MENINGOVASCULAR NEUROSYPHILIS inasmuch as the globulin, albumin, -cellular content, gold sol, and W. R.’s were all weakly positive. - - 1. How far can we regard the cardiorenal defects as syphilitic? - Perhaps we may do so on the general principle of parsimony in - scientific interpretation. - -The diagnostic lumbar puncture led to an extremely severe exacerbation -of the pains on the left side of the head. In fact, these pains could -not be held in check by the exhibition of pyramidon. Mr. Stone regarded -the pain as due to the lumbar puncture. However, there was no -improvement in the pain in the prone position,—a feature characteristic -of lumbar puncture pains. Upon administration of salvarsan, this local -pain rapidly disappeared. In fact, there was a startling improvement; -the ocular palsies disappeared in a few weeks, although these palsies -had been present for several months before the administration of -salvarsan. The blood pressure was reduced; the urine became negative. -Perhaps the most startling feature of all (although of this we are not -sure) was that the patient states he was accepted by a life insurance -company although he had been twice refused previously. - -=Note= in this case the 30–year interval between infection and -generalized neurosyphilitic involvement. Note also the amenability of -the process despite this duration. We are perhaps entitled also to note -that a neurological examination careful enough to detect an -Argyll-Robertson pupil should have been made by a number of examiners -long before the particular crisis which we have sketched. It is also -permissible to note that the rhinological work should not have been -carried out independently of all other medical work. - - 2. What are the untoward results of lumbar puncture? It is true that - there is always a possibility of setting up a septic meningitis by - lumbar puncture, but this is a very remote possibility and with - any reasonable care it is not to be considered. Lumbar puncture - also has a considerable danger in cases of increased intracranial - pressure. In cases of brain tumor where the tumor is located in - the posterior fossa, sudden death may occur from withdrawal of - spinal fluid. This is supposed to be due to the medulla being - pressed down into the foramen magnum and causing paralysis of - respiration. Therefore lumbar puncture should never be performed - except with the greatest caution in a case in which brain tumor is - suspected. - - However, aside from these remote serious consequences which play - very little rôle in the ordinary procedure of lumbar puncture, - certain unpleasant symptoms do frequently arise. These symptoms - are chiefly headache and nausea, but, however, may go as far as - vomiting. These symptoms occur almost entirely in the cases in - which there is no abnormal condition producing increased spinal - fluid pressure. Such unpleasant symptoms may last as long as four - or five days; as a rule, however, last only for a period of a day - or two. - - 3. What is the treatment of discomfort following lumbar puncture? It - is a rule well worth observing that the patient after lumbar - puncture should remain flat on his back without a pillow for 24 - hours in order to avoid any unpleasant symptoms. If any symptoms - do occur, it will be almost certainly when the patient arises, and - in nearly every instance they will be overcome if the patient - again assumes the prone position. Raising the foot of the bed so - as to lower the head also helps. Veronal or bromides may be given - but as a rule are not very satisfactory. - - 4. How permanent is the improvement obtained in the case of Mr. - Stone likely to be? As a matter of fact, the patient discontinued - treatment as soon as he felt well again, but after two months the - pain returned to be again quickly dispelled by salvarsan. This - improvement must be considered as only temporary. Under continued - treatment there may be no further relapse. There is, however, - evidence that much damage has been done to the body by the - spirochetes, much of which is irreparable. It is even possible - that further disintegration might occur even while undergoing - treatment. Still treatment offers much in such a case and is to be - highly recommended. - - - =In DIFFUSE NEUROSYPHILIS, rendering the spinal fluid negative by - treatment may mean neither cure nor disappearance of symptoms.= - - -=Case 107.= Greta Meyer, a widow, 51 years of age, came voluntarily to -the hospital, seeking medical aid for a marked depression. She was also -suffering from a right hemiplegia. It appeared, according to Mrs. Meyer, -that she was married at 16, and lived with her husband until 29, -whereupon she left him on account of his alcoholism, his abuse of her, -and the discovery through his physician that he was suffering from -venereal disease. She had had two healthy children and there never had -been miscarriages or stillbirths. Six years after the separation, namely -at 35 years of age, and 16 years before resort to the Psychopathic -Hospital, Mrs. Meyer developed certain red areas on her hand, and -learned at a hospital that these were due to syphilis. She kept up -treatment for these lesions for a year, until she seemed perfectly well. - -She had, in fact, remained perfectly well for some 14 years, until at -49, a small tumor had appeared on the right side of the forehead, near -the hair line. This tumor was firm and not sore. Medical treatment -reduced it, leaving, however, a depression in the bone. One day, about a -month after the appearance of the tumor, the patient lay down for a nap, -and upon awaking found she could only with difficulty move her right arm -and leg. Her face was not affected; she was not in pain; and there was -no disorder of speech. In a few days she got much better and she had -been improving for some time past through the administration of further -medicine. - -However, since the onset of the hemiplegia Mrs. Meyer had been very -despondent. There had been ups and downs but she had rarely felt well. -The depression was a mild one and in point of fact may perhaps be -regarded as non-psychopathic, since at her age with her disability, -there might well be a degree of sadness and unhappiness concerning the -future. =Mentally=, there was no other disorder of note, and in -particular no disorder of memory. - - - =METHODS OF TREATMENT= - - I. BY MOUTH. - 1. MERCURY - 2. IODIDES - 3. ARSENIC - - II. INTRAMUSCULAR INJECTIONS - 1. MERCURY - 2. SALVARSAN, NEOSALVARSAN, OTHER ARSENIC PREPARATIONS - 3. SODIUM NUCLEINATE - 4. ANTIMONY - - III. INTRAVENOUS - 1. MERCURY - 2. MERCURIALIZED SERUM - 3. SALVARSAN, NEOSALVARSAN, ARSENIC - 4. IODIDES - - IV. SPINAL INTRADURAL - 1. SALVARSANIZED SERUM (IN VIVO—SWIFT-ELLIS) - 2. SALVARSANIZED SERUM (IN VITRO—MARINESCO-OGILVIE) - 3. MERCURIALIZED SERUM (BYRNES) - - V. CEREBRAL SUBDURAL AND INTRAVENTRICULAR - 1. SALVARSANIZED SERUM (IN VIVO) - 2. SALVARSANIZED SERUM (IN VITRO) - 3. MERCURIALIZED SERUM - - CHART 26 - - -=Physically=, the patient showed a right-sided hemiplegia with excessive -right knee-jerk, but without Babinski or other abnormal reflex -phenomena. The extraocular movements were somewhat restricted in range -but there was neither strabismus nor nystagmus. - -The question arose whether the hemiplegia was of hemorrhagic or -thrombotic origin. After all, at 51 years, hemiplegia is rather unlikely -to be of a non-syphilitic arteriosclerotic origin; moreover, we had a -clear history of syphilis. The serum W. R. proved positive as well as -the spinal fluid W. R. The finding of 77 cells per cmm., excess albumin, -and positive globulin test, taken in connection with the entire picture -seems to warrant a diagnosis of CEREBROSPINAL SYPHILIS. If we proceed on -statistical grounds, it might be regarded as more probable that the -hemiplegia is THROMBOTIC in origin rather than hemorrhagic. It appears -that syphilitic cerebral thrombosis rather characteristically occurs -without preliminary symptoms, despite the fact that many cases do show -headache, dizziness, and restlessness as prodromal symptoms. - - 1. What is the treatment indicated in the case of Mrs. Meyer? - - It would appear that little or nothing can be done for the - hemiplegia unless the claims of Franz with respect to - reëstablishment of a degree of function in certain hemiplegics are - substantiated. However, the indication of meningitic process as - shown by the spinal fluid, suggests that the case is not a purely - vascular one but may be regarded as meningovascular. (Possibly, - also, we should regard the left frontal depression and scar as - indicative of a non-parenchymatous and non-vascular process.) - Accordingly, antisyphilitic treatment should be theoretically of - some value. - - In point of fact, the patient was given injections of mercury - salicylate, mercury by mouth, and potassium iodid. Her - psychopathic depression under this treatment, supported by proper - hygiene and rest, diminished. However, six months later, the - patient slipped on a wet floor and fell. Though the impact seemed - hardly sufficient to cause a fracture, the pelvis was somewhat - severely fractured. Very probably there was a syphilitic - rarefaction of the bone. Six months later the patient’s depression - was still in evidence, though somewhat less than upon admission. - The blood serum remained positive but the spinal fluid had become - entirely negative, both in respect to the W. R. and in respect to - the other findings. - - 2. How may one explain the continuance of the depression after the - spinal fluid had become entirely negative under treatment? It may - be that while the active process had been stopped, as seems - probable from the negative spinal fluid, that a permanent - destruction of brain tissue may account for the depression. We - recognize this readily in instances of vascular disturbance where - (as also in this case) the active process being stopped, a - residual defect remains. - - 3. Should treatment have been discontinued on reduction of the - gumma? It cannot be too often emphasized that the disappearance of - symptoms in cases of syphilis can not be considered as evidence of - cure. The neurologist and psychiatrist see only too often cases of - neurosyphilis occurring in patients who have been declared cured - at some time previous because the symptoms then present had - cleared up and remain in abeyance for years. - - - =Contrary to various warnings, arteriosclerosis by no means - absolutely contraindicates intensive salvarsan therapy.= - - -=Case 108.= Victor Friedberg, 42 years of age, gave the following -history. He acquired syphilis at 22 years. He had “adequate” medical -treatment for two years with inunctions of mercury and mercury by mouth -and potassium iodid. The only secondary symptoms were skin lesions of -the legs; these disappeared upon treatment. Married, Friedberg has one -child, apparently normal. There had been no miscarriages or stillbirths. - -At about 34 years, there began to be shooting pains in the legs, -occurring at first about once in three months, but later much more -frequently. These pains were severe, lightning in character, lasting -several days at a time, at which period his head would feel heavy; but -there were no disturbances, crises, or difficulty in locomotion. - -At 36 years of age, Friedberg waked up with pain one night, and found he -was unable to move his left leg or hand, and he felt his mouth drawn to -the left. Upon trying to get out of bed, he fell to the floor. In five -hours, however, he was entirely recovered, able to get up and walk -about, and to use his left arm quite normally. He went to sleep, but -upon waking up after an hour, discovered that his left side was again -paralyzed. After two weeks in a hospital, he was able to walk with a -crutch. The arm remained helpless for about a year. Both arm and leg -improved slowly for two years, after which time his condition had -remained stationary. For four years past, there had been no more pain, -but at 42—about two years before admission—the pains returned in his -legs, back, and side. At that time he received four injections of -salvarsan, mercury tablets, and potassium iodid. Three weeks before -admission to the hospital, Friedberg again began having headaches, very -much worse than formerly. At first these headaches were frontal, then -occipital, and there was a feeling as if something were growling inside -of the head. There was a feeling of pressure in front on the head and at -the base of the nose. - -=Physically=, Friedberg appeared somewhat older than his assigned age. -There was a degree of general peripheral arteriosclerosis, but in -general the physical examination was negative. _Neurologically_, there -was a left hemiplegia with appropriate increase of the reflexes on that -side, spasticity, Babinski reflex, and an Oppenheim; the pupils reacted -properly; there was no Romberg reaction. - -_Mentally_, Friedberg was entirely negative. - -The W. R. of the blood serum was doubtful, as was that of the spinal -fluid. There were but two cells per cmm. and there was neither globulin -nor excess albumin in the spinal fluid. - -The =differential diagnosis= might lie between cerebral hemorrhage and -syphilitic thrombosis. Thrombosis is much more common as a result of -syphilis than is hemorrhage. The occurrence of the thrombosis during -sleep without premonitory symptoms is also characteristic in syphilis. -Possibly there was a low-grade spinal meningitis at the bottom of the -lancinating pains. Whether the headache is an arteriosclerotic effect or -due to a meningitis not shown in the cerebrospinal fluid is doubtful. -However, the absence of inflammatory products in the cerebrospinal fluid -rather indicates that the headache is of arteriosclerotic origin. -Autopsies, however, warn us that we may have a localized meningitis in -various parts of the cranial cavity without the determination of any -inflammatory products in the spinal fluid. - - 1. How shall we explain the doubtful (slightly positive) W. R. in - the spinal fluid if the case is one of VASCULAR BRAIN SYPHILIS? - The finding is not unusual in these cases. The W. R. producing - body is recognized to be of a separate nature from the globulin - and albumin bodies, and is probably also separate from the gold - sol reaction producing bodies. - -Treatment: The theory of treatment is that any spirochetes that may be -still active in the body should be destroyed. Accordingly, although -salvarsan can certainly have no effect in reproducing nerve tissue, it -nevertheless seems indicated. It is frequently stated, however, that -salvarsan is dangerous in cases of this group. We have not found this -statement correct. In this case, there was a symptomatic improvement, as -far as pain and discomfort went, under salvarsan and iodids. - - 2. What precautions should be taken in intensive salvarsan treatment - of syphilitic arteriosclerosis? Treatment should be begun with - very small doses of salvarsan, that is, about 0.1 of a gram and - then the amount slowly increased. The injection should be given - slowly so as not to put too great a load upon the cardiovascular - system. - - 3. What rôle does the mental attitude of the patient play in a case - like that of Friedberg? It was quite evident that Friedberg was - neurotic and that he had a syphilophobia. Consequently some of the - symptomatic improvement may have been more results of assurances - offered by the physician and knowledge that he was being treated, - than results of salvarsan. In some cases mental anguish suffered - by the patient is of more importance than the actual symptoms of - the disease and this point must be always borne in mind in - handling syphilitic patients. - - - =Symptoms of intracranial pressure cured by antisyphilitic - treatment.= - - -=Case 109.= Mrs. Annie Rivers, a housewife 36 years of age, sought -advice and treatment for severe convulsions which she had had during a -period of several weeks. She left the hospital before being properly -examined, and had several more convulsions, after which she was brought -back in a state of marked confusion. The confusion shortly disappeared -almost completely, and a good history was obtained. - -It appears that the patient led a normal life and had had six children, -the last of whom was born about four months before her coming to the -hospital. The first symptoms appeared about a month after the birth of -the child, when, one afternoon, Mrs. Rivers suddenly fell unconscious -while ironing. She remained unconscious for nearly three hours. During -this attack there were no convulsive movements or tongue-biting; and -after the spell, she felt neither lame nor sore, but merely tired. This -was Mrs. Rivers’ statement; but her daughter stated that the patient -really did have convulsive movements. A week later came a second -convulsion, followed by daze and stupor. This second attack lasted two -hours. - -About a week before entrance, the patient had remained in bed on account -of dull grinding pain in the left side of the head, below the ear, and -upon this day the patient vomited twice. In addition to the dull -grinding pain, there were pains referred to the ear itself and to the -left side of the head, especially over the left eye; there were no pains -on the right side of the head. The next day the patient was better, but -the day thereafter again remained in bed. The only other symptoms were -cold feelings at times and bright spots in the field of vision. - -No =mental symptoms= were observed in Mrs. Rivers except a bit of -depression after her hasty retreat from the hospital the first time. -Upon her second admission, however, after a week or ten days’ residence, -apathy developed together with considerable amnesia for the same facts -she had quite readily remembered a few days previously. Along with the -apathy and amnesia developed considerable headache; and there were -attacks of vomiting. - - - =UNTOWARD SYMPTOMS OF THERAPEUTIC AGENTS= - - =A. SALVARSAN= - - CYANOSIS MALAISE - RAPID PULSE - PERSPIRATION - RESPIRATORY DIFFICULTIES - FEVER - NAUSEA, VOMITING, DIARRHOEA - DERMATOSES - EDEMA - KIDNEY IRRITATION - LIVER IRRITATION - INTENSIFICATION OF SYMPTOMS - COLLAPSE - - =B. MERCURY= - - SALIVATION - FETID BREATH - EXCESS FLOW OF SALIVA - TENDERNESS OF TEETH—LOOSENING AND FALLING OUT - SPONGY GUMS—EROSION - METALLIC TASTE - NECROSIS OF BONES OF JAW - SORENESS OF PARETIC AND MAXILLARY GLANDS - SWELLING AND EROSION OF TONGUE AND MUCOUS MEMBRANES - GASTRO-INTESTINAL SYMPTOMS - ANEMIA - PAIN IN JOINTS - NEPHRITIS - - =C. IODINE= - - SKIN LESIONS - METALLIC TASTE - SALIVATION - CORYZA - URTICARIA (EVEN TO GRADE OF ANGIONEUROTIC EDEMA) - PAINS - CONSTIPATION - INVOLVEMENT OF JOINTS - FEVER - SOFTENING AND BLEEDING OF GUMS - EROSION OF MUCOUS MEMBRANES - GASTRO-INTESTINAL SYMPTOMS - ANOREXIA - WEAKNESS - - CHART 27 - - -On the =physical= side, it is interesting to note that the -ophthalmoscopic examination upon Mrs. Rivers’ first admission to the -hospital was entirely negative, whereas a week later, pronounced -difficulty with vision appeared so that in a few days she was able to -make out only very large type. The fundi now showed hazy and indistinct -disc outlines, with small yellowish areas of fatty degeneration above -the disc, reduction of arterial calibre, and dilated and somewhat -tortuous veins (no projection of papillæ), so that the ophthalmological -diagnosis was chronic neuritis. - -The physical examination otherwise was mostly negative. The skin -presented irregular areas covered with silvery scales over the arms and -chest, back, abdomen, and legs (the patient had had psoriasis several -years before). Both pupils reacted to light and distance, though the -right was slightly larger than the left and somewhat irregular. There -was a slight tremor of the tongue and extended fingers. The reflexes -were active, especially the knee-jerks; no abdominal reflexes could be -obtained. The serum W. R. was positive, but the spinal fluid W. R. was -negative. The spinal fluid showed but 3 cells per cmm., but there was a -positive globulin test and an excess of albumin. - -=Diagnosis=: After the symptoms had fully developed, it became clear -from the optic neuritis, headaches, and vomiting that a condition of -intracranial pressure existed. In view of the positive serum W. R., it -is natural to conceive that the agent producing the intracranial -pressure was a gumma. - -It is, of course, possible that a marked degree of meningitis might be -so localized as to produce the same symptoms. The diagnostician would -crave a pleocytosis of the spinal fluid if a diagnosis of meningitis is -to be made; and there was no such pleocytosis. On the whole, we do not -feel that it is possible to make a diagnosis either of MENINGITIS or of -GUMMA. - -=Treatment=: Treatment, however, caused a disappearance of all symptoms. -The treatment consisted of but one injection of 0.3 gram of salvarsan, -followed by a few injections of mercury; whereupon Mrs. Rivers became -much brighter, recovered her vision, lost her headaches, ceased to have -convulsions or vomiting spells. - - 1. Is salvarsan contraindicated in cases with involvement of the - optic or auditory nerves? Such a contraindication exists according - to prevailing opinion. In this particular case, a hemorrhagic - retinitis occurred after the injection of salvarsan, but this - retinitis disappeared along with the other symptoms. On the whole - we believe that in many cases of optic or auditory nerve - involvement salvarsan should be used. However, one should never - lose sight of the possibility of untoward results and should - advise such treatment only when other treatment seems inefficient. - - - =TABETIC NEUROSYPHILIS (“tabes dorsalis”) may show very marked - improvement as a result of intraspinous therapy.= - - -=Case 110.= Mr. McKenzie[18] was a retired merchant of 42 years whose -complaint was that he tired very easily, could not make his legs go -where he wished, was unsteady and felt a numbness in his legs. These -symptoms had been in progress for a few months only when the examination -was made. This disclosed Argyll-Robertson pupils, absent knee-jerks and -ankle-jerks, Romberg sign, unsteady gait, moderate ataxia and dysmetria. -The W. R. was negative in the blood serum but positive in the spinal -fluid with 0.2 cc., and there were 107 cells per cmm. With the symptoms -and signs it was therefore easy to make the diagnosis of TABETIC -NEUROSYPHILIS (“tabes dorsalis”). - -The patient was given five intraspinous injections of mercuric chloride -in blood serum (mercurialized serum) according to the method of Byrnes. -The dose was 0.001 gm. of mercury. Two weeks after the first injection -the cell count was 58 cells per cmm., the Wassermann was positive only -with 0.4 cc. After the fourth injection there were but 18 cells and the -Wassermann reaction was negative even with 1½ cc. of spinal fluids. The -symptoms had improved to such a degree that the patient had no complaint -whatsoever and considered himself cured. - - 1. What are the unpleasant results of intraspinous therapy? - Frequently there is an exacerbation of symptoms and pain may be - quite severe after intraspinous injections. This, however, lasts - only a short period, that is, as a rule less than 24 hours. There - may be other symptoms of cord irritation as retention of urine or - lack of sphincter control. A rise of temperature is not unusual. - - - =Treatment may alter the W. R. to negative in blood and spinal fluid - in TABES DORSALIS.= - - -=Case 111.= Ivan Rokicki was a baker, 43 years of age, who came -complaining of exceedingly severe attacks of abdominal pain with -vomiting. He described these attacks as having occurred periodically for -a number of years, lasting sometimes as long as a week, during which -time Rokicki could not eat or get relief short of large doses of -morphine. - -Upon his arrival, Rokicki was seen in one of his attacks; he was curled -up with excruciating pain, and the abdomen was rigid, though it was -impossible to produce additional pain by external pressure. There was -spasmodic vomiting, frequently followed by slight relief from the pain, -which however shortly recurred and caused the patient to cry out in his -suffering. The condition was controlled by opiates but lasted a full -week. The leucocytes remained normal and there was no rise of -temperature. The attack ceased spontaneously. - -Save for the pain, Rokicki’s =mental examination= proved entirely -negative. =Physically=, Rokicki was fairly well developed and nourished. -His pupils were slightly irregular: the left markedly larger than the -right; both pupils failed to react to light, and the left pupil also -failed to react in accommodation. There were no other reflex disorders -evident to systematic examination, nor was there sensory disturbance or -speech defect. The heart seemed somewhat enlarged but there were no -murmurs; blood pressure: systolic 150; diastolic 110. - -The correct symptomatic diagnosis in Rokicki’s case proved to be gastric -crises, and this diagnosis must perforce be the first to entertain in -view of the chronicity, the periodicity, the non-relation to diet, and -the spontaneous cessation of the seizures. The observation of -Argyll-Robertson pupils was naturally held to substantiate the diagnosis -of TABES DORSALIS. - -The possibility of abdominal inflammation could be shortly dismissed on -account of the absence of tenderness (the rigidity in this case was not -accompanied by tenderness), fever, and other characteristic signs. There -was no diarrhoea, such as is found in lead colic, and there was no other -sign of plumbism. Jaundice was absent and there was no special radiation -of pain from the abdomen. One had to think of gastric ulcer and -hyperchlorhydria, and possibly malaria or gastroenteritis. - -The pupillary reactions pointed to a syphilitic condition despite the -fact that the lack of reaction to accommodation (over and above the -Argyll-Robertson phenomenon) in the right pupil is not entirely typical. -Accordingly, although there was no areflexia, Romberg sign, or ataxia, -resort was had to the W. R. This however proved negative, in blood and -spinal fluid; nor was there any globulin or excess albumin; there were 5 -cells to the cmm., in the spinal fluid. - -We are left, accordingly, with characteristic gastric crises; -Argyll-Robertson pupils, slightly irregular; and a somewhat enlarged -heart. - -Upon investigation, it appeared, however, that a year before the attack -above described, the patient had been examined and both blood and spinal -fluid found positive to the W. R. At that time, treatment, consisting of -intravenous injections of salvarsan and intraspinous injections of -salvarsanized serum (Swift-Ellis), had been instituted. Whereupon the -laboratory tests had become negative, as above stated, and there had -been no alleviation of the symptoms. - - 1. How can Rokicki’s normal deep leg reflexes be explained? The - abolition of the deep reflexes is of course due to lesions - properly localized. It is probable that this particular case of - tabes dorsalis is more truly “dorsal” than most cases; for most - cases exhibit lesions involving regions lower than the dorsal. - Both in these dorsal cases and in certain rare cases of cervical - tabes, the deep leg reflexes are preserved. (See cases Green (30) - and Halleck (31).) - - 2. What is the mechanism by which a characteristic gastric crisis is - produced? The mechanism is unknown. Some endeavors have been made - to meet gastric crises by surgery of the posterior roots, on the - assumption that the irritation causing the pain was located either - in the posterior ganglion or in the passage of the nerve through - the meninges. In only a few instances, however, has the result - been what was desired. In many instances the gastric crises and - pain continued uninterrupted and in addition came discomfort due - to the lack of sensation in the part supplied by the severed - nerve. At present this treatment is seldom carried out. - - 3. Should antisyphilitic treatment be continued in such a case? As - far as our present knowledge of syphilis goes one would hesitate - to suggest further antisyphilitic treatment, feeling that the - active process had been entirely stopped as suggested by the - absence of any positive findings either in the blood serum or in - the spinal fluid. We should perhaps conclude that there was no - more activity in this case and that the crises were due to the - changes that had already taken place in the nerve tissue and which - could no longer be changed. - - - =The literature is in doubt concerning (in fact is preponderantly - against) the success of treatment in PARETIC NEUROSYPHILIS (“general - paresis”). Our experience has yielded a number of apparently - successful results through systematic intensive intravenous - salvarsan therapy. Example.= - - -=Case 112.= Albert Forest had always been a successful salesman, but in -the middle of March, in his 46th year, he was arrested for grabbing a -purse from a woman in front of a theatre and running down the street -with it. In court, Forest acted strangely and he was sent to the -Psychopathic Hospital for observation. Upon investigation, it appeared -that his wife thought he had been showing mental changes for about a -year. For example, he would embrace his wife on a street car, or refuse -to pay her fare. He once attempted to hit his son on the head with a -red-hot poker. Now and then he would become sleepy and stupid. He looked -rather older than his age and had a coarse tremor of the hands. -Otherwise, no change could be detected in the physical examination, -either neurologically or otherwise. As for the manual tremor, Forest’s -wife gave a history of considerable alcoholic indulgence on his part. - -For several days, nothing abnormal could be detected in the man; and in -particular, his memory for both remote and recent events was very good -and his knowledge of current events was good. Simple arithmetic was easy -to him. - -One evening his temperature was found to be 104° F. and no cause could -be discerned for this. The next morning, Forest was discovered in a -stupor, with a complete right hemiplegia. The Babinski reflex, the -Oppenheim reflex, and ankle clonus had appeared on the right side, and -the right arm was spastic. - -However, all symptoms of this paralysis had disappeared by four o’clock -in the afternoon, and the paralytic phenomena were replaced with -violence. The patient fought with the attendants and for some time -remained extremely difficult to manage, being confused and subject to -outbreaks of violence with destruction of furniture and other property -about the ward. - -=Diagnosis.= At first we were naturally inclined to dismiss the case -with a diagnosis of alcoholism. The transient hemiplegia at once raised -a considerable question of brain syphilis or of brain tumor. - -The W. R. of the serum was doubtful. The spinal fluid yielded, besides -marked excess of albumin and much globulin, also a “paretic” gold sol -reaction and 75 cells per cmm. The W. R. was positive. - -=Treatment.= The patient was given injections of salvarsan, 0.6 gram, -twice a week, with potassium iodid. After a few weeks improvement -followed, and after several months all the laboratory tests became -negative, the patient was apparently perfectly normal mentally and was -discharged from the hospital, and has remained well for 18 months -without further treatment. The serum W. R. has continued to be negative. - - 1. What is the significance of the so-called “doubtful” W. R.? Where - there is not a complete uniformity the results of the strong and - weak antigens (see appendix on technique of Wassermann reaction) - the result is reported as doubtful. In the majority of instances - repetitions will give a strong positive reaction. - - 2. Is the case of Forest to be regarded as one of general paresis? - Sometimes such cases are termed in the literature _syphilitic - pseudoparesis_ (see case Burkhardt (58)). The differential - diagnosis of this group is entirely therapeutic. There are, - unhappily, no laboratory tests which will suffice in the present - stage of knowledge to differentiate a case of so-called - pseudoparesis from general paresis. We are inclined to term the - case one of GENERAL PARESIS, with recovery, or, at all events, - with remission. - - - =The literature is in doubt concerning (in fact is preponderantly - against) the success of treatment in PARETIC NEUROSYPHILIS (“general - paresis”). Our experience has yielded a number of apparently - successful results through systematic intensive intravenous - salvarsan therapy. Example.= - - -=Case 113.= We present the case of Gussie Silverman, a housewife, 35 -years of age, among other reasons, for its social interest. The case is, -on the whole, sufficiently typical of GENERAL PARESIS. =Physically=, for -example, the pupils failed to react to light and accommodation and were -unequal, the right being larger than the left. The knee-jerks were -sluggish though equal. The ankle-jerks could not be obtained. The -abdominal reflexes were not obtained. Otherwise, there was no reflex -disorder. - -From the =laboratory= point of view, the W. R. was positive in the blood -and in the spinal fluid. There were 80 cells per cmm. and there were an -appropriate globulin and albumin reactions. Mrs. Silverman was rather -poorly nourished and had a slight edema of the ankles. - -=Mentally=, she was found on admission to be markedly depressed. It -appeared that during a recent pregnancy, terminated by the birth of a -7–months child, she had fainted several times a day, that since the -confinement she had been very nervous, that she had been asking her -husband not to send her away, that she had refused to leave the house, -that she had become excited even to the point of injuring herself, -especially at night, and that she would go so far as to scratch her -husband, shortly afterward being very sorry for her performances. Before -this last pregnancy there had been four others and the resulting -children were all apparently in good health. Except for the fainting -spells during the pregnancy, it would not appear that the story just -told is at all characteristic of paresis. - -However, in the hospital Mrs. Silverman could hardly be got to answer -questions, continually saying, “You know what it is; I don’t have to -tell you.” She claimed so marked a degree of confusion as not to know -where she was and what she was doing. She would beg despondently that -something be done for her, and iterate and re-iterate these claims. -There appeared to be a marked degree of amnesia. Some one, she felt, had -controlled her thoughts and made her do things she did not want to do -and say things she did not want to say, things she did not know she was -about to say. She said, “I feel like jumping around. I couldn’t believe -myself as if I am me. Some one is making me jump around. I used to hear -him talking. I don’t know who it is. I used to keep my eyes open and I -couldn’t move. I feel only I would like to talk, and talk, and talk, and -talk all the time. It seems to me that some one talks in me. I couldn’t -sleep for five minutes. My God, I wish I could sleep! I used to feel -something in my heart. I used to faint. It seems to me I used to see a -funny thing. What it was I can’t tell. It used to talk to me, make me -get out of bed, throw me about, make me do things. O, I don’t know what -it was.” - -These not entirely characteristic mental symptoms, together with the -suggestive physical signs and the laboratory examination, caused -treatment to be instituted; under which treatment (intravenous -injections of salvarsan) she improved rapidly. Mental symptoms -disappeared under the administration of 12 injections of salvarsan -within two months. Moreover, the spinal fluid became entirely negative. -Two and a half years have now elapsed since her discharge and she has -shown no return of symptoms. The serum W. R. has always remained -negative although there has been no treatment since leaving the -hospital. There has, however, been no change in the reflexes, which -remain as on admission. The 7–months baby has continued to be perfectly -healthy. Its W. R. is negative, as are the W. R.’s of the husband and -the other three children. It must seem surprising that a healthy child -could have been born from a mother with generalized syphilis as in this -case. However, perhaps there are more instances than we imagine like the -case of baby Silverman. - - 1. May a patient be considered permanently cured although there has - been no recurrence of symptoms for 2½ years and although the - Wassermann has remained negative? One would hesitate to give a - definite statement that the patient was cured until more time had - elapsed. It is quite possible that spirochetes may be lurking in - some portion of the body without causing the production of - symptoms or Wassermann bodies and yet ready to break out at any - time. This hypothesis has added weight from the recent work of - Warthin already quoted. We advise examination of this patient at - intervals of not longer than six months for a good many years. - - 2. Should the course under treatment cause us to change the - diagnosis? It has often been stated that a differential point - between cerebrospinal syphilis and general paresis is the reaction - to treatment, that is, that a case which recovers could not be - general paresis. Head and Fearnsides state that if six months - after beginning of treatment the spinal fluid has become negative, - the case should be considered as one of cerebrospinal syphilis and - not general paresis. We do not feel ready to concur in this view - as we know of no similar logic in medicine. We have many cases in - which a spinal fluid has remained positive for six months and - later become negative, so that where the symptoms shown are those - of paretic neurosyphilis, we are inclined to consider the case - such until such time as more definite evidence checked by post - mortem examination causes us to change this point of view. - - 3. Do the reflexes change under treatment? The signs of spasticity - often do disappear under treatment and also when there is no - treatment. A few instances have been reported in the literature - where Argyll-Robertson pupils are said to have altered to normal. - It has never been our good fortune to see such a change nor have - we seen an absent knee-jerk become normal, as has also been - reported, except where it is the result of pyramidal tract disease - superimposed upon the posterior column sclerosis causing a return - of reflex. This, of course, is not to be considered as a return of - the normal. (See Case 1.) - - - =Some RESULTS of systematic intravenous salvarsan therapy are - PARTIAL (_e.g._, clinical recovery and persistence of positive - laboratory tests).= - - -=Case 114.= Walter Henry was an undertaker in a small town. He was -married and the father of two healthy children. In May, 1914, he began -to lose his appetite. He felt restless and seemed to be losing his grip, -and in August he repaired to a sanatorium, where he remained for two -months. Shortly after leaving the sanatorium, he fainted one day, while -digging a grave, during a spell of great heat. Since that time there had -been numerous “weak spells,” with headaches and general debility, -insomnia, and loss of weight. - -In February, 1916, Mr. Henry came to the hospital for advice, but the -trip from a distant part of the state was apparently such a strain for -him that shortly after admission he collapsed. There were no convulsive -movements in this collapse, but the patient was confused and his -breathing was rapid and stertorous. The semi-stupor lasted for about 48 -hours. Upon recovery from the stupor, Henry was found entirely -disoriented, much confused, and laboring under the belief that he was -digging a grave. After a time he again fell into a stupor and his -temperature rose to 103° F. - -The emaciation of this man was striking and unusual, but systematic -=physical examination= showed no special disease. =Neurologically=, -there were marked tremors, and there were purposeless movements of the -arms. There was a marked speech defect. The pupils were dilated, -regular, and equal, and reacted, though slightly, to light. Nothing -abnormal was noted upon systematic examination of the reflexes. - -The W. R. was strongly positive in the blood and in the spinal fluid; -the gold sol reaction was typically “paretic”; there were 16 cells per -cmm., globulin was present, and albumin was greatly increased. - -The =diagnosis= GENERAL PARESIS was accordingly made, and treatment -instituted. Intravenous injections of arsenobenzol, at first, and later -of diarsenol, were given, as a rule twice a week (usual dose, 0.6 of a -gram). Mercurial injections and potassium iodid were also given. This -treatment was continued as the patient began to improve. The improvement -was of such a degree that at the end of four months, Mr. Henry returned -to his home and his work. He had had 30 intravenous injections of -salvarsan substitutes. Despite the treatment and the clinical -improvement, the laboratory tests remained essentially unchanged. The W. -R.’s of the blood and spinal fluid remained strongly positive, as well -as also the globulin and albumin; the gold sol reaction was still -“paretic”; the cells stood at one per cmm. The patient has continued -antisyphilitic treatment since leaving the hospital, and has remained -apparently well, with good insight into his condition. - - 1. What is the significance of a temperature of 103° in a paretic - without signs of infection and a normal leucocyte count? - Temperatures of this type are not infrequent in the course of - general paresis. They are usually spoken of as “paretic - temperatures.” Their meaning is not understood, but they are often - stated to be due to a disturbance of the heat-regulating - mechanism. Such temperatures may remain elevated for a - considerable period of time, but the elevation may be very - transitory. At times they vary, like septic temperatures. - - 2. What can be argued from the fact that the cell count became - normal? If thorough antisyphilitic treatment is vigorously given, - it will be found that in the vast majority of cases of - neurosyphilis the cell count will return to normal. It matters not - whether the treatment be intravenous or subdural. It is very - difficult, however, to obtain this result in general paresis by - the use of mercury alone. It cannot, however, be urged that this - finding has any great prognostic significance as it occurs in the - cases which do poorly as well as in those which recover - symptomatically. - - 3. Is it safe to give large doses of salvarsan to a patient in a - stupor? It is not a good plan to give a large dose to such a - patient on account of the danger of sudden death. This is probably - due as much to the strain put on the heart as it is to any effect - on the nervous system, or specific arsenic effect. In this - particular instance, a dose of 0.15 gm. was the initial injection - and this was increased five centigrams per injection. - - - =IMPROVEMENT IN PARETIC NEUROSYPHILIS (“general paresis”) may become - evident only after several months of intensive treatment.= - - -=Case 115.= Henry Ryan was a shipping clerk, 54 years of age, who was -brought to the hospital following a convulsion. For a few months -preceding this period, Mr. Ryan had been failing in his abilities. He -had been very forgetful, showed no energy, and had become very -irritable. He also complained of insomnia and of feeling nervous. - -On admission to the hospital, the most striking feature in the mental -situation was that he claimed that he had not slept a wink for three -months, and each day he would solemnly affirm that he had not slept at -all the preceding night, although the records might show that he had -slept eight hours. Argument was of no avail against this conviction. In -addition, his memory was very poor; he showed little knowledge of -current events, and had no ability with arithmetical problems. - -=Neurologically= viewed, the points of chief significance were -contracted immobile pupils and a speech defect, especially noticeable on -the repetition of test phrases. The whole picture was suggestive of -general paresis, and this diagnosis was confirmed by the laboratory -findings. It was found that the W. R. was positive in the blood and -spinal fluid, that there was a pleocytosis, positive globulin reaction, -excess of albumin, and a “paretic” gold sol reaction. Consequently, the -diagnosis of GENERAL PARESIS seemed justified, although the patient -denied any knowledge of a syphilitic infection. - -Treatment in this case consisted of intravenous injections of salvarsan, -diarsenol, or arsenobenzol, whichever drug was most easily obtainable, -given twice a week in doses of 0.6 gram each. In addition, he was given -occasional injections of mercury salicylate as well as potassium iodid -by mouth. Once or twice a week, 40 to 60 cc. of spinal fluid were -withdrawn. Under this treatment for a period of three months, the -patient showed no improvement whatsoever, either in his mental condition -or in the laboratory findings. However, treatment was faithfully -persevered in, and shortly after the three months, improvement began to -be noticed. At first, the patient began to admit that possibly he may -have slept a few winks some time during the previous six months, for he -said he realized it was not possible for a man to live without sleep for -that period. Then he began to admit that he might have slept a few hours -during the night, and later that he was sleeping pretty fairly. His -memory also showed improvement. His general attitude showed alertness, -and he began to interest himself in his surroundings and in the events -of the world, and finally he gained complete insight into his condition. - -In the meantime, that is after three months of treatment, the laboratory -findings began to grow weaker. The gold sol reaction was the first to -decrease in strength, and after four months of treatment, it vacillated -between negative and a mildly positive “syphilitic” reaction. Then the -globulin and albumin became less in amount, and the W. R. began dropping -off in the 0.1 and 0.3 cc. dilutions. As is usually true in those cases -of neurosyphilis that receive adequate treatment, the cell count early -dropped to normal. The W. R. in the blood serum, however, remained -positive. - -As the patient’s condition seemed so much better, he was allowed to -leave the hospital at the end of five months. He took things easily for -the following seven months, and then, after being out of employment for -the period of a year, as his health continued good, he decided to return -to work. Before doing so, he entered the hospital again for a lumbar -puncture. At this time, it was found that the cell count was normal, -there was a very faint trace of globulin, possibly a slight increase -above normal albumin content, and a very mild gold reaction. The W. R. -in the spinal fluid was negative including the 1.0 cc. dilution; the -blood serum remained positive. - -The patient then returned to his old position and has done -satisfactorily for the past six months. During this entire time, he has -been coming to the hospital for treatment: during the major portion of -the time, about once in two weeks; of late, once in four weeks. - -The significant point in this case is that improvement did not show -itself until after more than three months of intensive treatment, and -then the improvement was synchronous with a weakening of the spinal -fluid tests. - -It is further significant that his mental and physical condition was -good before the tests had reached anything like normal; and that under -treatment, these tests continued to grow weaker and weaker, until at the -end of a year, they were practically negative. - -The case further illustrates the enormous number of injections of -salvarsan preparations that may be given to a patient without causing -any appreciable damage to the general health or to the kidney function. -Mr. Ryan has had more than 60 injections. - - 1. How soon after treatment is instituted does improvement usually - occur in paretic neurosyphilis? In our experience improvement - usually shows itself in from two or three months of treatment. - Occasionally the improvement may be very marked shortly after - treatment is commenced, that is, after three or four injections of - salvarsan. This is not, however, the rule and as in the case of - Ryan, it may be only after more than three months that improvement - is seen. This means that in the treatment of these cases patience - must be exercised and much work done. - - 2. What is the point of withdrawing large amounts of spinal fluid as - in the case of Henry Ryan? It has been stated that the withdrawal - of 40 or more cc., of spinal fluid while the patient is under - treatment has the effect of reducing the intraspinous and - intracranial pressure and thereby allowing the drug to diffuse - into the nervous tissue better than it would do under ordinary - conditions. How much truth there is in this contention it is - difficult to say and there is as yet no experimental evidence to - confirm this contention. As a matter of fact, the spinal fluid in - cases of paresis is usually under increased pressure and it is at - least plausible to conceive that a reduction of this pressure may - give some symptomatic relief. - - - =Evidence of the activity of syphilis outside the central nervous - system may be seen in cases of neurosyphilis despite intensive - treatment.= - - -=Case 116.= William Rosetti was a speculator, 43 years of age, when he -was brought to the Psychopathic Hospital on account of an outbreak in -which he smashed a showcase at the store where his sweetheart was -employed; he caused so much commotion that he was arrested. - -On admission, he was very excited, talking loudly and at length. For -some days it was very difficult to manage him, he was so active. At any -moment, he would insist upon undressing and taking physical culture -exercises. He was very euphoric and expansive, and had no insight into -his condition. - -=Physically=, he was a powerfully-built man and in very good physical -condition except for an iritis and moderate thickening of the peripheral -arteries. The =neurological signs= of importance were Argyll-Robertson -pupils, and absent knee-jerks and ankle-jerks. With these findings in -mind, a tentative diagnosis of GENERAL PARESIS was made, and this was -substantiated by the laboratory tests, which gave positive W. R.’s in -blood and spinal fluid, globulin, excessive albumin, slight pleocytosis, -and a “paretic” gold sol reaction. - -When the patient’s mental condition was somewhat better, he gave a -history of syphilitic infection 15 years before, for which he had had -almost continuous treatment. As a matter of fact, treatment had been -pretty strenuous because he had recurring skin lesions and iritis. It -was practically impossible to get the skin lesions to heal with mercury, -and it was not until salvarsan was introduced that a good result was -obtained in this respect. After one or two injections of this drug, the -skin lesion disappeared and has never returned. However, at least once a -year, he has had attacks of iritis, and for this reason was still being -treated for syphilis at the outbreak of his psychosis. - -He was at once placed on more strenuous antisyphilitic treatment in the -form of diarsenol, semi-weekly, aided by mercury injections. After a few -months of this treatment, his mental condition improved so much that he -seemed to be entirely normal. Treatment was continued, however, without -any abatement, and it was of great interest to note at the end of five -months of such treatment that, although mentally he seemed entirely -well, he had an attack of iritis, which was considered as a sign of -active syphilis. This would appear to indicate the great difficulty of -getting results in certain cases of syphilis with any drugs at our -command at present, as in the iritis we are dealing with a condition -which as a rule reacts fairly readily to antisyphilitic remedies. - - 1. Are there different strains of spirochetes showing various - degrees of malignancy? This question has been discussed at length - in the literature but there is no satisfactory answer at the - present time. We must always consider the reaction of the organism - and the host; and it is true in syphilis, as in every other - disease, that in some individuals it is more difficult to get any - therapeutic results than in others. - - 2. Was the failure to obtain results by long years of treatment due - to “drug fastness” of the spirochetes? It has been held that the - organism of syphilis will develop an immunity after a time to - mercury and arsenic preparations. This led Fournier to recommend - intermittent treatment as more efficient than continuous - treatment. Noguchi has shown that in test tube experiments, the - spirochetes develop a tolerance to increasing doses of arsenic. It - must be emphasized, however, that this finding has not been - established for the conditions _in vivo_. Another explanation of - the failure of treatment in certain instances has been offered by - McDonagh, who describes a life cycle of the organism of syphilis - under the name of _cytorrhyctes luis_, of which he believes the - spirochete to be merely one form, the other forms not being - affected by arsenic or mercury. - - - =Some results of systematic intravenous salvarsan therapy in PARETIC - NEUROSYPHILIS (“general paresis”) are partial in the sense that with - clinical recovery the laboratory tests remain partially or less - strongly positive.= - - -=Case 117.= Annie Martin was a charwoman, 37 years of age. She had -applied for relief at a general hospital, to which she was admitted on -the suspicion of nephritis; but upon admission she became markedly -excited and noisy, and spoke of seeing angels and hearing God speak to -her. As the attendants were unable to quiet her, she was promptly -transferred to the Psychopathic Hospital. She maintained that she had -been sent to the Psychopathic Hospital through the spite of the general -hospital doctors, and she claimed that other people were also attempting -to work her harm for the purpose of taking her children from her. Visual -and auditory hallucinations were marked, as was the patient’s loquacity, -irritability, and flight of ideas. However, she seemed entirely oriented -and her memory appeared to be intact. She was able to explain somewhat -clearly her supposed condition. The voices told her that somebody was -after her and that her soul belonged to the devil; that she was to be -married but that her soul was to be damned. These voices probably -belonged to priests. She was under the impression that she was going to -be sent to an electric chair and said, “I think I am coming to the end -and I want a pair of rosary beads before the end comes.” - -This patient’s pupils were markedly unequal and entirely stiff to light -and accommodation. =Neurologically=, however, there were no other -symptoms. There was a slight trace of albumin in the urine and there -were no casts. - -The psychiatric =diagnosis= in this case would off-hand undoubtedly be -dementia praecox. Yet the stiff pupils are almost proof positive of -neurosyphilis. If further proof were necessary, it is found in the -laboratory tests, which showed a positive W. R. of the serum and fluid, -with a “paretic” gold sol reaction; there were 22 cells per cmm., there -was excess albumin, and a positive globulin reaction. - -Under intensive antisyphilitic treatment, there was a slow improvement. -After several months, the patient was entirely free from mental -symptoms; the spinal fluid tests became entirely negative except that -the gold sol reaction has remained strongly positive. - - 1. Should treatment be continued in the case of Annie Martin in - spite of the clinical recovery and the negative tests except the - gold sol? We would again emphasize that it is unreasonable to - suppose that a long-standing case of syphilis can be cured in a - period of a few months of treatment and while the tests may become - negative, it would seem foolhardy to stop treatment on this - account. We do know that in many cases a Wassermann reaction - remaining negative for many months may again become positive, - indicating that the negative reaction did not mean cure but rather - the absence of the Wassermann bodies in the circulation at the - time the test was made. - - 2. What is the significance of the paretic gold sol reaction when - the other tests have become negative? As previously stated, the - gold reducing substance in the spinal fluid seems to be different - from the substances which give the other pathological reactions. - We should feel in this case that the process which was producing - these gold reducing bodies had not been stopped, in other words, - cure was not complete. - - 3. Should one make a diagnosis on the “paretic” gold sol reaction - alone? The so-called paretic gold sol curve is not always - indicative of general paresis or even of syphilis but may occur in - non-syphilitic conditions as brain tumor, multiple sclerosis, etc. - In our experience we have seen no case of _untreated_ - neurosyphilis in which the gold sol alone was positive, that is, - in cases in which therapy has not changed the findings in the - spinal fluid. In our experience the gold sol reaction has been - fortified by one or several of the other tests as the W. R., - globulin test, pleocytosis. - - - =Some effects of systematic intravenous salvarsan therapy in PARETIC - NEUROSYPHILIS (“general paresis”) are limited to the laboratory - findings without clinical improvement.= - -Two examples of such limitation are offered: William Roberts (118) and -John Silver (119). - - -=Case 118.= A bank teller, William Roberts, 39, was sent to the -Psychopathic Hospital for a depression so marked that he had become -entirely unable to work or care for himself. The story was that some -money had been left him by his uncle, that Roberts could not prove his -right to the money, and that depression, insomnia, and occasional -periods of confusion had followed during a period of about five months. - -On admission, Roberts appeared wholly disoriented and unable even to -give his correct age. Attention could not be held, and the patient would -slide off into statements like: “Oh, I made a mistake, I fooled a lot of -people, I have a terrible disease, they are going to get it, they are -going to get me,” etc., etc. There was great difficulty in thinking, and -a marked reaction of fear. This cluster of phenomena certainly suggested -very strongly the diagnosis of manic-depressive psychosis. - -=Neurologically=, Roberts proved quite negative except that the tendon -reflexes were very active and the pupils reacted somewhat sluggishly to -light. The blood serum W. R. was negative. No history of syphilis could -be obtained; nevertheless, Roberts kept dropping remarks about the -terrible disease from which he was suffering. It seemed best to proceed -to lumbar puncture, and the spinal fluid disclosed a positive W. R., -globulin, increased albumin, pleocytosis, and “paretic” gold sol -reaction. - -The =diagnosis= of GENERAL PARESIS was accordingly made. During the next -year and a half, no improvement was made; a slight speech defect was -developed, and tremors of the hand and tongue appeared. - -The effect of treatment is particularly instructive. Only after 18 -months in the hospital was intensive antisyphilitic treatment -instituted; but after a few months of this treatment the W. R. of the -spinal fluid had become negative, the cells normal in number, globulin -absent, albumin present only in normal amount. Only the gold sol -reaction remained positive. It is still of a paretic type. Treatment, -however, did not succeed in altering the patient’s mental condition in -the slightest. At the end of many months of treatment, we still confront -a man showing marked psychic symptoms and a “paretic” gold sol reaction -without other laboratory signs. - - 1. What is the significance of the practically negative tests in - this case without clinical improvement? One must believe that the - tests became negative as the result of treatment, and that this - change in the tests was due to the clearing up of some - inflammatory reactions which were present. This may mean that the - syphilis had been reduced to inactivity or latency if not cured, - or at least that there was no activity sufficient to cause a - positive W. R. in the blood serum, whereas whatever activity was - present in the brain was in such a region that it did not cause - any reacting substances to be cast into the spinal fluid. This - would not mean that there would necessarily be any return of - function already lost, because this may be considered as a - permanent loss which cannot be compensated for. As to these tests, - we now feel that the case should remain stationary; that is, that - no new symptoms will be added. However, we believe that it is - somewhat premature with our present knowledge to make this claim - very forcibly, and would rather suggest that this case be - considered as demonstrating an interesting fact, the meaning of - which can be learned only after a period of years. - - 2. Why does the gold sol reaction remain strongly positive when all - the other tests become negative? As already pointed out, above - (Case Martin (117)) there is no known rule about the disappearance - of one or other of the abnormal findings in spinal fluid under - treatment, and we can at present offer no explanation of this - phenomenon. It does, however, illustrate how careful we must be in - drawing any conclusions from tests in cases that are being - treated. - - - =Diminution in the spinal fluid tests may occur in treated cases of - neurosyphilis without clinical improvement.= - - -=Case 119.= John Silver, a man 29 years of age, presented classical -symptoms of GENERAL PARESIS: He had a convulsion shortly before his -admission to the Psychopathic Hospital, his memory was poor, he was only -partially oriented, he was very euphoric and expansive—thought he had -millions, that he was the Czar of Russia, and so on. His tendon reflexes -were very much increased and there was a marked speech defect. The W. R. -of both blood and spinal fluid were strongly positive; the spinal fluid -showed globulin, increased albumin, pleocytosis, and a “paretic” gold -sol reaction. There was, therefore, no question about the diagnosis, and -the patient was at once put under antisyphilitic treatment. This was -continued for five months; slowly the intensity of the reactions in the -spinal fluid diminished. At the end of the five months, there was the -very slightest possible trace of globulin, with a doubtful increase in -albumin, one cell per cmm., and a mild syphilitic gold sol reaction. The -W. R.’s in the blood and spinal fluid, however, remained strongly -positive. There was no mental improvement coincident with the weakening -of the spinal fluid tests, and at the end of the five months, the -patient had a series of convulsions in which he died. - -This case is given as a contrast to Case Henry (114) in which clinical -improvement occurred without diminution in laboratory tests; in the case -of John Silver, marked diminution in the intensity of these tests had no -prognostic significance. This was in keeping with the condition as shown -in Case Roberts (118) where, while the gold sol was the only test to -remain positive, the patient did not improve mentally. - - 1. What is the explanation of the lessening of the pathological - elements in the spinal fluid under treatment? We have seen that - the various findings may occur independently of one another, and - we must admit that we do not know definitely what it signifies, or - why one may be present or absent. It has been held by Head and - Fearnsides that the findings in the spinal fluid represent - conditions in the spinal cord and spinal meninges, or at the base - of the brain only, and not conditions elsewhere. This is in - keeping with our finding that the gold sol reaction in the spinal - fluid post mortem very often differs from that in the ventricular - fluids or cerebral, subdural, and subpial fluids. And further, we - have found that during life the findings in paresis in the spinal - fluid may differ markedly from those in the third ventricle, and - that the change in the fluid in these two areas under treatment - may not occur simultaneously. - - - =Systematic intensive treatment of PARETIC NEUROSYPHILIS (“general - paresis”), including intraventricular injections of salvarsan, may - entirely fail.= - - -=Case 120.= James McGinnis, aged 39, came to the hospital on a -stretcher, semi-conscious, moaning, unable to reply to questions; there -were signs of a right hemiplegia. - -The next day, McGinnis cleared a little and became able to utter a few -words. His wife said that he had been entirely well up to four years -ago. At that time he was struck in the eye by the head of a hammer that -flew off the handle. Diplopia had developed, but disappeared. - -Only two years later did a marked change appear. McGinnis became -careless as to personal appearance. Seemed absent-minded, apathetic and -drowsy; he would fall asleep in his chair or while at work. He lost his -position and became apprehensive, making not very strenuous efforts to -find work, and finally consulted a physician. The physician told him -that he had a sluggish liver and gave him calomel. - -Six months later, McGinnis was restored to his position as foreman, and -his work remained satisfactory for some six months. Then (about six -months before coming to hospital), his speech became slow and somewhat -unintelligible. He quit work, saying that his speech was going from him -and that he might be considered to be drunk. His memory grew rapidly -worse. There was improvement after a vacation and he returned to work, -but continued to be ataxic, complained of vertigo, and fell down several -times, though without loss of consciousness. On the very day of his -admission to the hospital, in attempting to get out of bed, he fell, and -psychotic symptoms at once appeared. There was slight improvement again -with entire disappearance of all paralysis after a few days, a slow -clearing up of the speech disturbance, and a certain return of memory. - -=Physically=, there was little to note. =Neurologically=, the left pupil -failed to react to light. The tendon reflexes were all very active, and -more active on the left side. Other abnormal reflexes were absent. -Improvement continued for a number of weeks, but the patient never -recovered from his speech defect, and his memory remained impaired. -Irritable at times, McGinnis was for the most part very happy and sure -he would get well. The W. R. of the blood serum was negative, but the -spinal fluid reaction was strongly positive, even down to 0.1 cc. The -globulin and albumin amounts were excessive. There was a “paretic” gold -sol reaction. There were 7 cells per cmm. The diagnosis of GENERAL -PARESIS was made. - -Intravenous injections of salvarsan, arsenobenzol or diarsenol were -made, and intramuscular injections of mercury, and potassium iodid by -mouth were given. No real improvement occurred after a certain initial -betterment; the spinal fluid yielded no changes. Diarsenolized serum -according to the Swift-Ellis technique was then injected into the third -ventricle. Under this treatment also there was no change for the better -over a period of several months. The patient died suddenly after a -series of convulsions, apparently from paralysis of respiration. - - 1. What are the causes of hemiplegia and confusion or - unconsciousness? We must consider epilepsy, brain tumor, cerebral - thrombosis, cerebral hemorrhage, multiple sclerosis, cerebral - spinal syphilis, and general paresis. - - - =MILD TREATMENT, often thought “adequate,” MAY FAIL, WHEN INTENSIVE - TREATMENT PROVES SUCCESSFUL.= - - -=Case 121.= Arthur Bright, a printer, had acquired syphilis in his 49th -year, some six months before examination. He had been treated during -these six months by three injections of salvarsan, injections of -mercury, and mercury by mouth. He had been apparently cured until about -a month before admission. He had fallen without warning from his chair -in a convulsion accompanied by unconsciousness, which lasted about two -hours. The patient had since been feeling rather peculiar. For instance, -time seemed to flow too rapidly. Sometimes the patient had had -difficulty in talking. - -=Physically=, nothing abnormal could be found either in general -condition or =neurologically=. The patient was, however, incontinent. -=Mentally=, he was apathetic and unalert, even paying no attention to -his outside physician when he came to visit him. - -The =diagnosis= of cerebrospinal syphilis already suggested by his -history was confirmed by the laboratory tests, which showed a positive -serum and spinal fluid W. R., paretic gold sol reaction, 41 cells per -cmm., an excess of albumin, and a positive globulin test. - - 1. What is the prognosis in cerebrospinal syphilis in the early - secondary stage? The prognosis appears very good provided that - intensive treatment be given and provided that no vascular insult - or other focal destructive lesion occurs before treatment has had - time to do its work. - - 2. Why did not the “effective” (?) treatment for the syphilis, - dating from the primary lesion, succeed in staving off the - cerebrospinal syphilis? It remains a question whether the - treatment by three injections of salvarsan was efficient in this - particular case. Of course, it may prove true that no treatment - whatever in the present stage of knowledge will stave off - cerebrospinal symptoms in certain cases. - -=Treatment=: Bright was given intravenous injections of diarsenol twice -a week, with occasional injections of mercury salicylate. After two -weeks, the patient seemed markedly improved, and continued to improve -rapidly. He was symptomatically well at six weeks. The spinal fluid had -then become negative, although the serum W. R. had remained positive. - -After discharge from the hospital, Bright returned to his work, but -continued to take the diarsenol treatment weekly, and two months later -the serum W. R. became negative. - -Small injections of diarsenol at intervals of a month were continued, -and Bright remained perfectly well for four months, when a peculiar -seizure developed and lasted for several hours. This seizure consisted -in a sort of somnambulism in which Bright stood up at a table, making -marks on paper, and could not be persuaded to desist. After this -seizure, Bright re-entered the hospital, again showed no mental or -physical symptoms and no abnormalities of blood or spinal fluid. - - 3. What is the explanation of this seizure? It is possibly due to a - small vascular insult, for which potassium iodid may be suggested - with precautions as to hygiene and continued observation. He has - since remained entirely well. - - - =Another example where MILD MEASURES (though conceived to be - “adequate”) SEEMED TO BE LEADING TO FAILURE; INTENSIVE THERAPY - SUCCESSFUL.= - - -=Case 122.= Levi Morovitz, a waiter, 39 years of age, came to the -hospital with evidences of an old left hemiplegia, including the left -side of the face (there was a left-sided Babinski, Gordon, and -Oppenheim, and all the reflexes were fairly active; sluggish pupil -reactions, Rombergism, and speech defect). Morovitz was much depressed, -very slow in thinking processes, had a marked memory disturbance in -general and apparently much deterioration mentally. - -A history was obtained to the effect that Morovitz had acquired syphilis -at about 33, but that he had received practically continuous treatment -ever since at a dispensary. He had, in fact, received four injections of -salvarsan a year before coming to the hospital. Of late, Morovitz had -become much more cheerful and talkative, imagining he could do great -things if he had money. He had begun to eat very rapidly and to be very -nervous. His feet had begun to drag; a distinct speech defect developed, -but from this he had recovered. About six weeks before entrance, -Morovitz had a shock, which left him with the left hemiplegia above -mentioned and with considerable headache. - -Even while the preliminary examination was being performed, Morovitz -developed a minor seizure without loss of consciousness. First came -severe pain over the frontal region, which grew in severity so that the -patient held his head in his hands. A bit later, twitching movements -began in the thumb and in the fingers of the left hand, and the small -muscles of the extensor group of the thumb and third finger showed -contractions. These contractions grew more general and the excursions of -the fingers greater, until finally every finger of the left hand became -involved, whereupon movements of the same sort, though of smaller -amplitude, began in the other hand. Finally the left arm began to jerk -with alternate contractions of the biceps and triceps. The whole seizure -lasted more than five minutes. During the seizure there was dizziness -and pain in the head, chiefly on the right side. - -=Diagnosis=: The attention is at once arrested by the data of the -seizures described. It appeared that we had to assume an irritation of -the right side of the brain, possibly due to vascular disease, or to -brain tumor, or perhaps to syphilis. The shock with residual hemiplegia -would be consistent enough with any of these diagnoses. However, the -history seemed somewhat long for brain tumor. Nor were there any -definite symptoms of intracranial pressure. “Adequate” treatment -unfortunately does not rule out syphilis. The comparatively early age -(39) of the patient makes it difficult to explain the vascular disease -except on the basis of syphilis. Add to the hemiplegia the euphoria and -grandiose ideas of a year’s duration, and we arrive at a diagnosis of -neurosyphilis, probably PARETIC NEUROSYPHILIS. - -The laboratory tests showed the W. R. of the serum and spinal fluid -positive, 80 cells per cmm. in the fluid, large amounts of globulin and -albumin, and a “paretic” type of gold sol reaction. - -To be sure the Jacksonian seizure is not especially characteristic of -paretic neurosyphilis, and even suggests a local irritation in the motor -area, such as a localized meningitis, possibly of a diffuse gummatous -nature. - -This patient was put on intensive antisyphilitic treatment, namely, -salvarsan twice a week and injections of mercury. He recovered rapidly. -After a few months he left the hospital, and after treatment had -continued for a year, he resumed his work by which time both blood and -spinal fluid had become negative. - -It must be recalled that this patient had from the time of his infection -what has been considered good antisyphilitic therapy, in spite of which -he developed after a period of years, the symptoms and signs of -neurosyphilis in its most dangerous form. The conclusion must be drawn -that however good such treatment is for the majority of cases, it was -insufficient for Morovitz. That the early failure to cure was not due to -any “drug fastness” of the spirochete or to any peculiarity of strain is -proved by the result of more vigorous antisyphilitic treatment which -caused an apparent if not a real cure. With our modern methods of -treatment checked by Wassermann reactions and spinal fluid examinations, -treatment is given according to the _needs of the individual patient_ -rather than according to general preconceptions. We have reason to -believe that under these conditions there will be fewer cases developing -late symptoms on account of insufficient treatment given even to -patients who are willing to co-operate to the last degree. - -The fact that Morovitz had no apparent symptoms for several years led to -rather desultory treatment chiefly in the form of mercury by mouth. -Previous to the time when the W. R. and lumbar puncture were available, -the physician had no exact means of determining cure except the -non-appearance of symptoms. But a period of years of quiescence before -the outbreak of symptoms referable to the involvement of the nervous -system is characteristic of syphilis. With this knowledge in mind it is -evident that today the care of a syphilitic patient must be guided, in -part at least, by examinations of the spinal fluid and W. R. - - - =Salvarsan treatment may even occasionally be of value in simple - FEEBLEMINDEDNESS due to congenital syphilis.= - - -=Case 123.= The somewhat unattractive Robert Matthews was brought, at 5 -years of age, to the hospital for backwardness of mind. It appears that -the patient was born at term, with instruments, that he began to talk at -a year, and to walk at 13 months, but that in point of fact, he had not -talked intelligibly to date. Robert had never played with other children -and is regarded by his parents as backward. In fact, Robert’s sister—a -year his junior—is much brighter. Robert had had scarlet fever but -without sequelae. - -Examination by the Binet scale showed that, although he is actually 5½ -years, he graded by the Binet scale at 4 and was regarded as -feebleminded. - -The =physical examination= showed a general adenopathy and prominent -frontal bosses. In the study of the family history in the search for an -etiology for the evident feeblemindedness, little or none could be -found. There were no miscarriages or stillbirths; the parents were -living and well. There was only the one sister above-mentioned, who is -brighter than Robert. - -The advantage of a routine W. R. is here well shown, for the W. R. in -the serum was positive. - - 1. What is the prognosis of cases of syphilitic feeblemindedness? It - would appear that every case is an individual problem. - - 2. What is the effect of treatment? Robert Matthews was given - mercury protoiodid ⅛ gr., three times a day, by mouth, for three - months. The protoiodid was followed by ten injections of - salvarsan, average: 0.15 gram, during six months. At the end of - this period, the W. R. in the blood had become negative. A - re-examination by the Binet scale, when Robert was 6–5⁄12 years of - age, showed him to grade at 5⅖, so that one might conclude that - Robert had shown more mental progress in a year than he had - previously. - -Note: The patient’s sister, 4 years of age, is attractive and bright, -measuring beyond her actual age according to the intelligence tests. -However, the girl was found to have a positive W. R. It may be that -Robert and his sister illustrate the hypothesis of Mott: that the -syphilitic virus becomes less potent as the years go on, and that the -younger children in the family are less affected than the older. -However, in our series, there are a number of instances in which this -hypothesis is not substantiated. - - 3. What is the share of syphilis in the production of - feeblemindedness? The percentage of syphilitic cases found in - institutions is not high. A variety of cases have been proved to - be congenitally syphilitic in the absence of a positive serum W. - R. - -Fernald[19] has charted a comparison of cases diagnosticated “moron” -(that is, feeblemindedness proper, in the narrower English sense) and -“imbecile.” Fernald says that the morons have, as a group, many more bad -family histories than have the imbeciles, to quote—“Only 70% of the -[imbecile] group have bad family histories. This at first seems -surprising, but when we consider that more of our syphilitic, traumatic, -and sporadic cases tend toward the lower end of the feebleminded group, -and when we remember that with such cases there is often a seemingly -normal family tree, the drop in the curve appears logical.” - -The situation with the idiots, of whom only 38 came into Fernald’s -study, was similar; 12 out of 38, or 32%, of idiots, had good family -histories. On these figures, how unfortunate it would be to dub -feeblemindedness hereditary! It is true, however, that 68–70% of the -idiots and imbeciles, judging by W. E. Fernald’s intensive study, do -have bad family histories. - -Goddard[20] states that of all the causes of feeblemindedness, there is -perhaps none for which there is less evidence than syphilis. Goddard -found syphilis in 27 of his intensively charted cases of -feeblemindedness, that is, in 9% of all his charts. He finds the -majority of the syphilis cases occurring in relatives of the -feebleminded to be in the hereditary group; for example, of 164 charts -in the hereditary group, 17, or 10%, showed syphilis. In 34 charts in a -group termed “probably hereditary” 3, or 9%, showed syphilis. Of 37 -charts in the group termed “neuropathic” 4, or 11%, showed syphilis, -whereas in 57 “accident” and 8 “no cause” groups, there were but 2 (4%), -and one, or 13%, showing syphilis. However, Goddard concedes that much -more careful studies are necessary if we are to give an exact evaluation -of syphilogenic feeblemindedness. - -The first ten of the Waverley Anatomical Series are shortly to be -described in a forthcoming publication.[21] Of these ten cases, four -showed some slight evidence of chronic inflammatory changes, indicating -the possibility of a syphilitic or similar infectious condition. These -cases, be it remembered, were not cases of juvenile paresis, but cases -of what, for the lack of a better name, may be called “ordinary” -feeblemindedness. - -If all or any of these processes are syphilitic, the syphilis is -virtually extinct. The cases had not been treated for syphilis and were -not regarded as syphilitic, though several of them showed a few stigmata -somewhat suggestive of syphilis. The anatomical conclusion at this time -is still doubtful. - -As in the text case, the hypothesis of syphilis as a direct cause for -simple feeblemindedness must be entertained for a few cases. In any -event, it would not seem logical to let any institution for the -feebleminded run without a Wassermann analysis of the population. In -addition to the Wassermann data from the blood serum, osteological data -from the X-ray have proved of occasional value for syphilis diagnosis in -this as in other groups. - - - “Within the gates of Hell sat Sin and Death.” - - Paradise Lost, Book X, Line 230. - - - - - VI. NEUROSYPHILIS AND THE WAR - - -Although the American toll of war syphilis has not yet begun and -although the crop of neurosyphilis due to war infections may not arrive -until the mid or late twenties of the century (witness German experience -in the eighties of the last century), it seems proper here to give a -number of abstracts _re_ neurosyphilis as it has developed in the war. -Available reports from English, French, and German sources have been -levied upon for the years 1914–16. - -It is clear that all the armies have had their share of -neurosyphilitics, some clearly diseased before enlistment, some -developing symptoms as a result of training, stress, or shock, others -hastened or made worse by war conditions. - -There are important questions of pension, retirement, and compensation -for neurosyphilitics. No previous war has had the benefit of the -Wassermann reaction and other exact tests bearing upon the nature, -progress, and curability of neurosyphilis. - -That we shall have our fill of pension and other problems can already be -seen from continental reports. Thibierge,[22] for example, states that -syphilis has become a real epidemic among the French soldiers and -mobilized munition workers. - -Hecht[23] of Austria claims that no less than an equivalent of 60 army -divisions have been temporarily withdrawn from fighting on the Teutonic -side for venereal diseases. He commends Neisser’s idea that salvarsan -and mercury should be given in the trenches. While hundreds or thousands -of Austrians are sick with syphilis, sound and healthy men are being -shot down in their stead. The diagnosis of syphilis, according to Hecht, -ought to be a signal for sending the men to the front. He makes even the -somewhat bizarre suggestion that special companies of syphilitics should -be formed, for convenience of treatment, on the firing line. - -Not only is the syphilis problem in the army of importance to the -military authorities, but also to the civil population, and perhaps to -them a greater problem. With the great increase of venereal disease that -is the result of the conditions of army life in war time, there will be -a considerable percentage of cases developing neurosyphilis a number of -years after discharge from the army, but caused by the infection -acquired during service. In addition many men will bring the disease -back to America in an infectious stage and spread it. We would advocate -that the names of all soldiers who had acquired syphilis and were not -considered cured at time of discharge should be given to health -organizations in their home states that they may be given further care. - -These practical and several theoretical questions are raised by the -following fourteen cases which we have condensed from their sources. - - - =A tabetic lieutenant “shell-shocked” into paresis? Case from Donath - of Vienna.= - - -=Case A.=[24] An apparently competent German professor in an -intermediate school, a lieutenant of infantry reserves, 33 years old, on -the 17th August, 1914, was stunned for a while by the shock of a -cannon-firing 25 feet away. Urination became difficult. Headaches and -limb pains ensued, with paralysis of fingers, gastric troubles, -forgetfulness especially for names, insomnia, and general scattering of -mental faculties. - -=Neurologically=, the pupils were irregular, left larger than right; -Argyll-Robertson reaction. Right knee-jerk livelier than left. Achilles -reactions absent. Slow and dissociated pain reactions in feet, lower -thighs and lower quarter of upper thighs, with hypalgesia or analgesia. -Station good; gait steady. Mentally depressed, slow of thought. Speech -poor and of indistinct construction (mild dementia). Calculation ability -poor. No pleasure in work. - -Wassermann reaction of serum weakly positive. - -It seems that for a year the patient had been subject to spells of -anger. He was irritated by his wife who had been nervous since an -earthquake. - -_On the occasion of the earthquake_, 1911, the patient himself had had a -spell of _difficulty with urination_. The spell had lasted two or three -months. The patient had had a chancre in 1902, “cured” in four or five -weeks with xeroform. In 1908, when about to marry, he had had six -mercurial inunctions. - - 1. Is this a case of traumatic paresis? From the somewhat meagre - account it would appear that Donath’s lieutenant should rather be - termed “shell-shock paresis,” in the sense of a paretic - neurosyphilis liberated by shell-shock (using shell-shock in the - sense of a shock _without_ direct brain injury). - - 2. What compensation is due such a man as Donath’s lieutenant? The - ordinary principles applicable to traumatic paresis are not here - in point, since no symptoms pointing to trauma of brain ever - supervened. See discussion under Case G. - - 3. How frequent is paresis in armies? R. L. Richards in White and - Jelliffe’s Treatment of Nervous and Mental Diseases writes as - follows (of course concerning peace times): - - “The French estimate that paresis cases are 7 per cent of all - their military cases. The German estimate is 6.6 per cent. In our - own army at the Government Hospital for the Insane, of 490 cases - of mental diseases among officers and enlisted men, 37, or 7 per - cent, were paresis. During the Russo-Japanese War, in the Russian - Psychiatric Hospital at Harbin, the percentage of paresis was 5.6 - per cent among the cases developing at the front.” - - - =A French soldier “shell-shocked” (also burial) into incipient tabes - dorsalis? Case from Duco and Blum of Paris.= - - -=Case B.=[25] A French soldier was buried by effects of shell explosion -September 8th, 1914. He sustained no wound or fracture. - -Incontinence of urine developed. Anesthesia of penis and scrotum. -Reflexes absent; pupils sluggish. Wassermann reactions suspicious. - -The diagnosis =tabes dorsalis incipiens= was made (hematomyelia of conus -terminalis eliminated). - -The patient was estimated to be “40% incapacitated,” according to the -French “_échelle de gravité_” of conditions. A full pension would not be -justified in the opinion of the French authors. - - 1. Is there evidence of an increase or exacerbation of tabes - dorsalis in the war? Birnbaum,[26] reviewing German war neurology, - quotes Weygandt as believing that the war has probably had to do - with the production of both tabes and paresis in many instances. - Other cases, however, have merely been made worse by the war - stress. Thirdly, there are cases in which the war stress has done - no harm whatever. Westphal has seen both tabes and paresis develop - in men who had never before shown any mental or physical symptoms - whatever, and accordingly, Westphal must be counted among those - who regard war stress as a liberating factor for these diseases. - Redlich and Donath are cited in the same connection. (The case of - Donath is the case presented above as Case A.) - - A very interesting claim was made by Cimbal to the effect that he - found many examples of paresis developing in the early period of - the war, particularly in November and December, 1914. Later, - according to Cimbal, cerebrospinal syphilis and tabes became more - prevalent. - - - =Neurosyphilis in a German recruit, possibly AGGRAVATED ON military - SERVICE. Pension not allowable. Case from Weygandt.= - - -=Case C.=[27] A German, long alcoholic and thought to be weakminded, -volunteered, but shortly had to be released from service. He began to be -forgetful and obstinate, cried, and even appeared to be subject to -hallucinations. The pupils were unequal and sluggish. The uvula hung to -the right. The left knee-jerk was lively, right weak. Fine tremors of -hands. Hypalgesia of backs of hands. Stumbling speech. Attention poor. - -It appeared that he had been infected with syphilis in 1881 and in 1903 -had had an ulcer of the left leg. - -The military commission denied that his service had brought about the -disease. In the phrase of the Canadian Pension Board the German -commission would probably have rendered a report “aggravated on -service,” not “by service.” (See Canadian cases D, E, and F.) - - 1. Has paresis increased in the war? Both French and German figures - controvert the claim. Marie, for example, found not a single - paretic amongst the skull injury cases at the Salpétrière. Most - authors are found demonstrating cases which they clearly regard as - in some way produced or unfavorably influenced by the war. There - seems, therefore, to be a little inconsistency between the general - statement that paresis has not increased in the war and the - somewhat frequent cases described as occurring in and modified by - the war. However, Bonhoeffer, on the basis of nine months’ war - experience, also holds it to be probable that paresis is no more - frequent in the field than in the home population. - - 2. Is the old syphilitic especially liable to break down under war - conditions? According to Richards, Shaikewicz says that in the - Russo-Japanese war paresis was noted especially among the officers - and non-commissioned officers, and that it was undoubtedly - hastened in its development by war conditions. Steida says that - while ordinarily we find paresis developing twelve to twenty years - after the primary sore of syphilis, in these cases it developed in - five to ten years after the primary sore. Some of the cases - progressed with unusual rapidity. It was also noticed that among - soldiers from the front, under treatment, evidences of syphilis - were present in 20%, while among the other soldiers under - treatment, evidences of syphilis were present in 1.6%. Undoubtedly - the old syphilitic is especially liable to break down under war - conditions. - - But, on the whole, the German authors in this war find no evidence - favoring Steida’s claim of the hastened post-infective outbreak. - - 3. How did it come about that the efficient German system - permitted this alcoholic and weakminded syphilitic to enter the - army? As will be seen, he was a volunteer. In general, the German - system has been supplied with army surgeons who have been trained, - not by brief and “brush-up” courses, but by longer periods, - sometimes two years in duration. - - - =Syphilis contracted before enlistment, “AGGRAVATED BY SERVICE.” - Canadian case, courtesy of Dr. J. L. Todd, Canadian Board of Pension - Commissioners.= - - -=Case D.= A laboring man, 42, who always strenuously denied syphilitic -infection, proceeded to France eight months after enlistment. He had not -been in France three weeks when he dropped unconscious. He regained -consciousness, but remained stupid, dull in expression, and with memory -impaired. His speech was also impaired. There was dizziness and a -right-sided hemiplegia. - -He was confined to bed four months and was then “boarded” for discharge. - -=Physically=, his heart was slightly enlarged both right and left; -sounds irregular; extra systoles; aortic systolic murmur transmitted to -neck; blood pressure 140:40. Precordial pain, dyspnea. - -=Neurologically=, there was a partial spastic paralysis of the right -thigh which could be abducted, could be flexed to 120°, and showed some -power in the quadriceps. There was also a spastic paralysis of the right -arm, but the shoulder girdle movements were not impaired. There was a -slight weakness on the right side of the face. There was no anesthesia -anywhere. - -The deep reflexes were increased on the right side, Babinski on right, -flexor contractures of right hand, extensor contractures of right leg, -abdominal and epigastric reflexes absent, pupils active, tongue -protruded in straight line. - -Fluid: slight increase in protein. W. R.+++ - -The Board of Pension Commissioners ruled that the condition had been -aggravated _by_ service. (See Case E, “aggravated _on_ service.”) - - 1. In view of the fact that the majority of the cases here - abstracted happen to be in common soldiers, is there any evidence - bearing on relative incidence in officers and men? Quoting R. L. - Richards: - - “The percentage of paresis cases among officers alone is variously - estimated from 50 per cent in the German army (Stier) to 58.9 per - cent in the Austrian army (Drastich). Since paresis is a disease - of more advanced life, it is but natural that the percentage of - paresis among officers, non-commissioned officers, and older - soldiers should be higher than among the whole military body, - where the average age is, as we have seen, well below thirty - years. Hence the above figures do not mean a greater prevalence of - syphilis among those classes, but that we have no means of knowing - how many of the others develop paresis. If anything it shows that - these ‘soldiers by calling,’ have a more stable mental make-up, - since they succumb chiefly to an exogenous toxin.” - - Rayneau at the 19th Congress of French Alienists and Neurologists - at Nantes in 1909, discussing the insane of the army from a - medicolegal point of view, states that the most frequent mental - disease amongst officers and soldiers is general paresis. At - least, this disease is the most frequent basis of invaliding, - retirement, or placing in the inactive list. He states that French - and foreign statistics are at one upon this matter, quoting - Christian as finding 32% among the soldiers interned at Charenton; - Gamier at Dijon, 59%; Meilhon at Quimper, 42% and Talon at - Marseilles, 33.8%. Grilli found 31 of 40 officers interned in - Florence, Sienna and Milan victims of general paresis. Stier’s - German statistics indicate about 50%. Rayneau himself found 16 of - 20 officers paretic and 17 out of 27 subalterns and _gendarmes_. - - The Neurological Society of Paris held a conference December 15, - 1916, with the chiefs of the neurological and psychiatric military - centres of France, and discussed a variety of questions concerning - invaliding, incapacity, and compensation in neuroses and psychoses - of war. Dupré dealt especially with the psychoses of war as caused - by trauma, strain, infection, and intoxication. General paresis is - regarded by Dupré as the most important of the dementias found in - the army. The medicolegal point of view is, of course, that - general paresis is necessarily related to an old syphilis, but its - late development leads to misinterpretations as to its probable - cause, both by the family and friends and even by magistrates. The - war acts in the French nomenclature as an _agent revélateur_ or as - an _agent accélérateur_. Although its cause is prior and exterior - to the war, general paresis in a majority of cases is brought out - (_revélé_) by the lack of adaptability of the general paretic to - the novelty and difficulties of his surroundings and duties in - war. Trauma, strain, and alcohol in a certain number of cases - accelerate the progress of a general paresis. The aggravation of - paresis is produced by these same factors, but especially by - violent cerebral trauma. According to Dupré, the Val-de-Grace - statistics show that the number of paretics has not been increased - by the war. Medicolegally, the victim of general paresis, like the - victim of traumatic or infectious chronic mental disorder, may be - assigned an incapacity of from 50 to 100%, and these patients are - invalided under _Réforme No. 1_,—a permanent invaliding. - - Lépine of Lyons also discusses the compensation question in - general paresis. Lépine thinks that, although syphilis is - indispensable in paresis, yet the truth is that syphilis plus - something else unknown to us is responsible for general paresis. - This something else is neither a special kind of virus nor is it a - particular kind of prepared soil alone. Trauma, physical, - intellectual, and moral strain, and insomnia are the factors to - which he calls special attention as adjuncts in the production of - general paresis. As to the responsibility of the State for the - production of general paresis, according to Lépine, the maximal - responsibility should be 40% on account of the very considerable - predisposition to paresis created by pre-existent syphilis. - - Marie remarked that, although there had been thousands of head - cases at the Salpétrière, there had not been a single case of - general paresis. Dupré agreed with Marie that trauma was not a - frequent etiological factor; strain and alcohol were more - important. The Society agreed that in exceptional cases, where an - encephalic trauma could be regarded as accelerating or aggravating - the disease, the degree of incapacity might be set at from 10 to - 30 per cent. - - - =Syphilis contracted before enlistment, “AGGRAVATED ON SERVICE.” - Canadian case, courtesy of Dr. J. L. Todd, Canadian Board of Pension - Commissioners.= - - -=Case E.= A laboring man, 44, acquired syphilis at a time unknown. Ten -months after enlistment this man developed symptoms on the firing line. -He was inattentive, irrational, incoherent. The diagnosis was then -“mania.” - -There were, however, scars at angle of mouth and on lower lip. Occipital -glands were palpable, fine tremor of hands. The W. R. was +++. - -Later the patient became violent, destructive, untidy, disoriented. -Auditory hallucinations are recorded. - -He was “boarded” for discharge five months after the first symptoms. The -board agreed that these symptoms would have appeared in civil life. In -view of a difference of opinion as to the part played by stress of -service, his condition was set down as “_aggravated on service_” (not, -it will be noted, _by_ service, see Case D). - - 1. Under what conditions should pensions be awarded for disability - resulting from venereal diseases? According to a personal - communication from Dr. J. L. Todd, Chairman of the Board of - Pension Commissioners for Canada, pensions are awarded for all - disabilities appearing _during_ service, unless they can be shown - certainly to be due to the men’s own fault and negligence. It - would appear that _during_ service covers both aggravations _by_ - and _on_ service. There remains some doubt as to whether - contraction of venereal disease constitutes negligence. - - 2. What have been conditions in the small inactive American army of - the past? Richards has made a study of statistics at the - Government Hospital for the Insane, Washington. - - “The leading features of this mental disease were well exemplified - in our cases the past year. They formed 7.5 per cent of the total - number. They averaged forty years of age, and Ziehen says 80 per - cent of all cases are in the fourth or fifth decade of life. They - averaged ten and a half years’ service, which would indicate that - the military life was their calling. Only one had any serious - hereditary defect. Stigmata of degeneration were infrequent, - averaging only two for each case. 66 per cent had good schooling, - considering their opportunities. Physical signs were frequent in - each case. Only one showed normal light reaction. Ziehen says the - light reaction is retained in only 20 per cent of the cases. - Patellar reflex was absent in one case and normal or exaggerated - in five. The speech defect was slight in four cases. Other - physical signs were present in the usual proportions. Memory - defects existed in all the cases. In four the onset was with - excitement. One began with a character change as the most marked - feature. In only two were the transfer diagnoses correct. One, - beginning as a quiet dementia, was diagnosticated paralysis - agitans, because of a marked tremor. One was excited and euphoric - and was called a manic-depressive psychosis. One with an obscure - onset was diagnosticated as a neurasthenic. The other one was - first observed in this hospital. The physical signs should have - led to a correct diagnosis in each of these cases.” - - - =Duration of neurosyphilitic process important _re_ compensation. - Canadian case, courtesy of Dr. C. B. Farrar, Psychiatrist, Military - Hospitals Commission.= - - -=Case F.= A Canadian of 36 enlisted in 1915, served in England, and was -returned to Canada in February, 1917, clearly suffering from some form -of neurosyphilis (W. R. positive in serum and fluid, globulin, -pleocytosis 108). - -There is no record of any disability or symptom of nervous or mental -disease at enlistment. The first symptoms were noted by the patient in -May, 1916, six months or more after enlistment. The case was reviewed at -a Canadian Special Hospital, October 11, 1916, by a board of examiners. -This board reported that: - -“The condition could only come from syphilitic infection of three years’ -standing” (a decision bearing on compensation); but the general -diagnosis remained: - -“Cerebrospinal lues, =aggravated by service=.” - -The picture which the medical board regarded as of at least three years’ -standing was as follows: - -History of incontinence, shooting pains, attacks of syncope, general -weakness, facial tremor, exaggerated knee-jerks, pupils react with small -excursion. Speech and writing disorder, perception dull, lapses of -attention, memory defect, defective insight into nature of disorder, -emotional apathy. - - 1. Was the conclusion “aggravated by service” sound? On humanitarian - grounds the victim is naturally conceded the benefit of the doubt. - But it is questionable how scientifically sound the conclusion - really was. - - 2. Could the condition come only from syphilitic infection of at - least three years’ standing? Hardly any single symptom in this - case need be of so long a standing; yet the combination of - symptoms seems by very weight of numbers to justify the conclusion - of the medical board. - - - =Can PARETIC NEUROSYPHILIS (“general paresis”) be lighted up by the - stress of military service without injury or disease? A possible - example from P. Marie, Chatelin and Patrikios of Paris.= - - -=Case G.= In apparently good health a French soldier repaired to the -colors, in August, 1914, being then 23 years old. - -Two years later, August, 1916, symptoms appeared: speech disorder with -stammering, change of character (had become easily excitable), stumbling -gait. He became more and more preoccupied with his own affairs, grew -worse, and was sent to hospital in October, 1916. - -He was then foolish and overhappy, especially when interviewed. There -was marked rapid tremor of face and tongue. Speech hesitant, monotonous, -and stammering to the point of unintelligibility. His memory, at first -preserved, became impaired so that half of a test phrase was forgotten. -Simple addition was impossible and fantastic sums would be given instead -of right answers; handwriting tremulous, letters often missed, others -irregular, unequal, and misshapen. - -Excitable from onset, the patient now became at times suddenly violent, -striking his wife without provocation. After visit at home, he would -forget to return to hospital. Often he would leave hospital without -permission (of course the more surprising in a disciplined soldier). - -No delusions were found. - -The serum and fluid W. R. were positive, albumin in fluid, -lymphocytosis. - -=Neurological examination.= Unequal pupils, slight right-side mydriasis, -pupils stiff to light, weakly responsive in accommodation, reflexes -lively, fingers tremulous on extension of arms. - -The patient had, December 5, 1916, an epileptiform attack with head -rotation, limb-contractions and clonic movements. - - 1. Should this soldier recover for disability obtained in service? - Marie was inclined to think military service in part responsible - for the development of the paresis. Laignel-Lavastine thought so - also, but that the amount assigned should be 5%–10% of the maximum - assignable. - - 2. What is the duty of the military authorities relative to so - called traumatic paresis? Medicolegally speaking, Froissart, - quoted by Rayneau, states that a victim of traumatic paresis _may - or may not_ have presented mental disorders before the accident, - that is, that the paretic symptoms may develop out of a clear sky - as a result of the accident. The accident itself must be of a - serious nature. The accident must be followed by phenomena - pointing to brain injury of traumatic nature. These phenomena need - not be characteristic symptoms of general paresis at the outset. - The period elapsing between the trauma and the supervening - condition of paresis must be occupied without notable - interruption, at first by phenomena of a purely traumatic nature, - later by signs indicating the onset and evolution of general - paresis. - - The French invaliding process called _Réforme No. 1_ with pension - is granted according to the governmental instructions only to - officers, subalterns, and soldiers whose disease is due to trauma. - In view of this governmental regulation, the military surgeon must - write out certificates describing every cranial trauma, however - slight, which might have a bearing on the development of paresis. - However, he should not too readily admit trauma as a cause of - paresis. If a long period of quietude, a period in which the - trauma itself seems to have undergone a complete recovery, - supervenes, then general paresis should not be reported by the - surgeon. - - Lépine has recently noted the following features as desirable in - board reports concerning paretics: nature of trauma, length of - service, fatigue endured, insomnia, date of infection, treatment, - W. R. - - - =Can “gassing” light up a paresis? Example from de Massary of - Issy-les-Moulineaux.= - - -=Case H.= A soldier, 35, was sent to the _Centre Neurologique_ with a -hospital ticket reading: - -“Neurasthenia, general weakness following intoxication by gas.” - -The soldier was thought at first to be a neurasthenic. But he soon -showed signs of more pronounced mental trouble. The voice was -suspicious. There was a slight irregularity of pupils. - -An epileptiform attack occurred, followed by aggravation of symptoms. - -Lumbar puncture showed pleocytosis. The W. R. of the serum proved -positive. - -Yet the evident =neurosyphilis=, possibly =paretic= (de Massary’s -diagnosis), was preceded by a neurasthenia and the neurasthenia was -preceded by “gassing.” - -De Massary believes the patient _and his family_ would perhaps be -justified in believing the condition produced by the injury. De Massary -is not clear as to the financial deserts of the patient. It is not a -manifest case of aggravation of antebellum symptoms, even if it be -neuropathologically an instance of acquired loss of resistance to -pre-existent spirochetes in body or brain. - - 1. What adjuvant factors have been recognized in military paresis? - Aside from syphilis, Rayneau finds that alcoholism, malaria, - sunstroke and various intoxications serve as causes for paresis. - Rayneau points out that the apparent integrity of the mind in - general paresis may be such that they last in the army some time - and have their oddities ascribed to misconduct or breaches of - discipline. In fact the Legrande du Saulle called this early - period in general paresis the _medicolegal period_, showing, as it - so often does, thefts, outrages against decency, frauds, assaults, - exhibitionism and the like. To be sure these acts are absurd and - infantile and not difficult to recognize as of psychotic origin. - - - =Syphilis may bring out epilepsy in a subject having taint. Case - from Bonhoeffer, 1915.= - - -=Case I.=[28] A man of 35 in the _Landwehr_ acquired syphilis some time -in the summer of 1914. He was a good soldier, passed through several -clashes, and was promoted to _Unteroffizier_. - -To understand what followed it must be stated that he had been a -bed-wetter to 11, had been practically a teetotaler (Bonhoeffer’s point -is perhaps that otherwise epilepsy might have developed sooner?), and, -when he did drink, vomited almost at once, and had amnesia for the -period of drunkenness. His father had been somewhat of a drinker. His -sister had suffered from convulsions as a child. - -February, 1915, the _Unteroffizier_ lost appetite, got headaches, and -went to hospital for a time. Upon getting better, he was sent on service -to Berlin. In a Berlin hotel he had his first convulsions and -unconsciousness, biting his tongue. He was confused for several days, -and, when he had become clear, had a pronounced retrograde amnesia -together with a tendency to fabricate a filling for the lost period. - -This retrograde amnesia is uncommon in epilepsy and suggests organic -disease. No sign of organic disease was found on neurological -examination. The patient had no signs of the epileptic make-up. The -serum W. R. was negative. On the whole, Bonhoeffer regards the epilepsy -as “reactive” to the syphilis, as a syphilogenic epilepsy. - -As to the amnesia, it is of interest that alcohol should long before -have been able to cause amnesia in this man in the same way as does now -the syphilitic epilepsy. - - 1. In view of the fact that this _Landwehr_ man appears to have - acquired syphilis while on campaign, what is the responsibility of - the government for treatment? The Canadian authorities, as stated - under Case E, are in doubt whether contraction of venereal disease - constitutes negligence on the part of the soldier. It would appear - to us that where a government does not take suitable steps to - prevent the acquisition of syphilis by the soldiers, the - government must assume a measure of responsibility for the - syphilis incurred. The government’s responsibility would be still - greater in equity, it would appear, if commercial opportunities - for the acquisition of syphilis are maintained under more or less - close government supervision or (even as has been claimed for - certain encampments on our own Mexican border) if shelter for - illicit sex relations is afforded within the limits of a military - camp. In a certain community, “E,” for example, it is claimed by - Exner,[29] the district for prostitutes was “situated within the - lines of military camps and protected and ‘regulated’ by the - military authorities.” - - But even if the government has no legal responsibility in this - regard, it would be well to consider the ultimate results of the - syphilis that will probably be acquired by great numbers of - soldiers under campaign conditions. Aside from the ravages of - syphilis outside the nervous system, it is well known, as Weygandt - intimates for German conditions, that the aftermath of war will be - a high proportion of cases of neurosyphilis. - - Weygandt remarks in his review of the influence of the war upon - psychiatry, that the opportunity for syphilitic infection in the - campaign is considerable. In the war of 1870, the conditions in - this regard were extremely unfavorable, and writing in 1915, - Weygandt remarks that at present there should be a prophylaxis - against syphilitic infection by the soldiers, which prophylaxis - should be the most energetic possible. Continence on the part of - the soldiers and the isolation of infected women, with examination - by specialists, have been advocated by Neisser and by Mendel. In - the ’80’s a great number of cases of locomotor ataxia developed in - Germany, which were due to syphilis acquired by the soldiers and - officers in the war of 1870. - - - =Syphilis in a psychopathic subject. Convulsions 5 days after - Dixmude. Case from Bonhoeffer, 1915.= - - -=Case J.=[30] A soldier in the reserves, 23, was, subsequently to his -being brought to hospital, described by his wife as a rather -over-sensitive fellow, who could hardly look at blood and was meticulous -about the household. He had always been subject to headaches, especially -after hard work. However, he had passed through his military training -well in 1910, not even having been _bestraft_. - -He began service in October and fought at Dixmude on the 19th. On the -24th in the trench and while being carried back, he had several spells -of pallor, falling stiff, and then having convulsions. Brought finally -to the Charité in Berlin, he had more spells of sudden pallor, collapse -with brief convulsions, tossings in bed, and absences, post-convulsive -headaches, and mild bad humor. - -There were numerous attacks several days apart in the first seven weeks. -The patient was not of an “epileptic” disposition, though he was rather -readily dissatisfied. Headaches also occurred without relation to -convulsions. - -The serum W. R. was positive. Treatment by mercurial inunctions. No -further convulsions. Prognosis as to the possibility of a constitutional -epilepsy unknown. - - - =SYPHILITIC ROOT-SCIATICA (lumbosacral radiculitis) in a fireworks - man with a French artillery regiment. Case presented from Dejerine’s - clinic by Long.= - - -=Case K.= No direct relation of this example of root-sciatica to the war -is claimed nor was there a question of financial reparation. - -There was no prior injury. At the end of March, 1915, the workman was -taken with acute pains in lumbar region and thighs, and with urgent but -retarded micturition. - -Unfit for work, he remained, however, five months with the regiment, and -was then retired for two months to a hospital behind the lines. He -reached the Salpétrière October 12, 1915, with “double sciatica, -intractable.” - -There was no demonstrable paralysis but the legs seemed to have “melted -away,” _fondu_, as the patient said. Pains were spontaneously felt in -the lumbar plexus and sciatic nerve regions, not passing, however, -beyond the thighs. These pains were more intense with movements of legs; -but coughing did not intensify the pains. Neuralgic points could be -demonstrated by the finger in lumbar and gluteal regions and above and -below the iliac crests (corresponding with rami of first lumbar nerves). -The inguinal region was involved and the painful zone reached the -sciatic notch and the upper part of the posterior surface of the thigh. - -The sensory disorder had another distribution objectively tested. The -sacral and perineal regions were free. Anesthesia of inner surfaces of -thighs, hypesthesia of the anterior surfaces of thighs and lower legs. -The anesthesia grew more and more marked lower down and was maximal in -the feet, which were practically insensible to all tests, including -those for bone sensation. There was a longitudinal strip of skin of -lower leg which retained sensation. - -Position sense of toes, except great toes, was poor. There was a slight -ataxia attributable to the sensory disorder—reflexes of upper -extremities, abdominal, and cremasteric preserved, knee-jerks, Achilles -and plantar reactions absent. - -The vesical sphincter shortly regained its function, though its disorder -had been an initial symptom. - -Pupils normal. - -The “sciatica” here affects the lumbosacral plexus. Signs of disorder at -one time or other affected the first lumbar distribution of the third -lumbar and first and second sacral nerves. - -As to the syphilitic nature of this affection, there had been at -eighteen (22 years before) a colorless small induration of the penis, -lasting about three weeks. There was now evident a small oval pigmented -scar. The patient had married at 20 and has had three healthy children. - -The lumbar puncture fluid yielded pleocytosis (120 per cmm.). Mercurial -treatment was instituted. - -The treatment has not reduced the pains. Long thinks it was undertaken -too long (six months) after onset. The warning for early diagnosis is -manifest. There was somehow a delay under the medical conditions of the -army. - - - =Can the “lighting up” of NEUROSYPHILIS IN CIVIL LIFE be induced by - the domestic stress of war? A possible example from Dr. R. Percy - Smith, London.= - - -=Case L.= A German Jew in London passed into the PARETIC form of -NEUROSYPHILIS shortly after the outbreak of war under conditions -suggesting that the stress of emotions directly or indirectly lighted up -the neural process. - -The man was a bank-officer, 52 years old, and married. He had lived many -years in England and was in fact a naturalized citizen. He had been -under treatment for syphilis by Sir Jonathan Hutchinson, 29 years -before, namely, at the age of 23. Subsequently, Sir John had given him -permission to marry. - -It proved that for years the man had had fixed pupils, absent -knee-jerks, and a perforated ulcer of the foot. However, there had been -no other mental or nervous symptoms preventing bank-officer’s work. - -At the outbreak of war the man was discharged from the bank. He grew -worried and sleepless. He began to charge himself with sex irregularity. -He went down to the city and burned trust documents belonging to others. - -From worry and self-accusation he passed into depression and agitation. -He developed a belief that not only he but also his German wife were to -be executed. He thought he was a criminal and was to be hanged. - -The depression then altered to a condition of hilarity and loquacity. - -In addition to the fixed pupils and absent knee-jerks, a speech disorder -shortly developed. - -The patient was placed under care, but quickly (a few months?) passed -into an advanced stage of paretic neurosyphilis and died. - - - =SHELL-SHOCK PSEUDOPARESIS (non-syphilitic). Recovery. Case from - Pitres and Marchand of Bordeaux.= - - -=Case M.= June 19, 1915, a shell exploded some distance from Lieutenant -R. He remembers the gaseous smell, the bursting of several shells nearby -and a sensation of being lifted into the air. When he recovered -consciousness, he was in hospital at Paris-Plage, covered with bruises -and scratches. They told him he had been delirious and had vomited and -spat blood. - -June 24, his wife came to see him, but this visit he could not remember. -Nor could his wife at first recognize him, he was so thin. He roused a -few moments and recognized his wife, but relapsed into torpor again. -Speech was difficult and ideas confused. - -A few days later he was able to rise; but his mental status grew worse, -especially as to speech and writing; the latter quite illegible. There -was insomnia, or, if he slept, war dreams. - -August 7, he began a period of five months’ convalescence passed with -his family, depressed, given to spells of weeping, confined to bed or -couch, unable to “find words,” conscious of his state and troubled about -it, speaking of nothing but the war, and afraid to go out for fear of -ambuscade. There was at first a slight lameness of the right leg. -Although he could walk, he felt pain in the knee on flexing the right -leg on the thigh. He walked holding this leg in extension. - -On going back to the colors, he was immediately evacuated to the _Centre -Neurologique_ at Bordeaux, January 20, 1916. - -Examination found a bored, impatient, irritated man, vexed that a man -who was not sick should be sent up “_comme fou_.” - -Omitting negative details, =neurological examination= showed slight -lameness as above, body stiff and movements jerky; difficult, unsteady -gait. The lieutenant could stand for some time on either leg, tongue and -face tremulous during speech. Limbs moderately tremulous, especially in -the performance of test movements. - -Knee-jerks and Achilles jerks absent. Other reflexes, including -pupillary, normal. Segmentary hypalgesia of right leg, especially about -knee. Tremulous speech and writing. Patient would stop short in speaking -for lack of words. - -Malnutrition. Appetite good, but a bursting feeling after meals. - -Skin dry, scaly on legs, fissured on fingers. - -Serum W. R. negative. Fluid not examined. - -=Mental examination.= Conscious and complaining of his troubles, -Lieutenant R. claimed persistently that he was not sick. Memory for -recent events was in general poor. Errands easily forgotten. Lost in the -street. Complaint of corpse odors round him. Everybody is looking at him -and making fun of him. He was apt to insult bystanders. He was afraid of -German spies. Things in shops angered him as they seemed to him to be of -German manufacture. - -There were frequent periods of depression, with pallor and no -spontaneous speech for some hours to a half-day. Headaches coming on and -stopping suddenly. - -As to diagnosis, the first impression, say Pitres and Marchand, was that -of general paresis. The progress of symptoms after the shock was -consistent with this diagnosis. The mental state and the physical -findings seemed consistent, although the pupils were normal. His partial -insight into his symptoms was not inconsistent with the diagnosis. He -had a characteristic self-confidence. There had been four stillbirths -(two twins) two children are alive, 11 and 13. Typhoid fever at 30. -Syphilis denied. No mental disease in the family. - -The patient had never done military duty, having been invalided for -“right apex.” But he had volunteered and been accepted in September, -1914. - - 1. Was this diagnosis, general paresis, at any time justified? The - spinal fluid should of course have been examined. The peculiar - lameness of the right leg was certainly not characteristic of - general paresis, and was perhaps hysterical. (There was no - limitation of visual fields or any other definite sign of - hysteria.) Presumably some quality of speech defect, the amnesia, - and the euphoria, together with absent knee-jerks, led to the - diagnosis general paresis. By the 20th of March, 1916, the - knee-jerks had become lively; the Achilles jerks normal. At this - time the patient had gained in weight, could walk though stiffly, - had headache (especially right frontal) and a feeling of lead in - head, less tremor, lack of desire to undertake anything. He still - wanted to go back into service. He still saw spies about. Dreams - terrible; devoured by spiders, leggins instruments of torture. - Skin still atrophic. June 4 there was no more tremor of speech or - face. Symptoms largely disappeared except a few ideas of - persecution. Recovery October, 1916. - - 2. How was Lieutenant R. cured? Apparently by rest in the _Centre - Neurologique_. Pitres and Marchand do not speak of the subtle - relation between mental state and the idea of non-return to - military service. This motive might still work even if Lieutenant - R. kept protesting quite sincerely that he wanted to go back into - military service. - - - =SHELL-SHOCK PSEUDOTABES (non-syphilitic, serum W. R. positive). - Improvement. Case from Pitres and Marchand of Bordeaux.= - - -=Case N.= Innkeeper B., 36, a shell-shock and burial victim June 20, -1915, was looked on by a number of physicians as a case of genuine -tabes. - -Even eight months after the episode, he still showed (when observed by -Pitres and Marchand, February 3, 1916) absence of knee-jerks and -Achilles jerks, a slight swaying in the Romberg position, pupils -sluggish to light, incoordination, delayed sensations. There was also a -history of pains in the legs, compared by the patient to those of -sciatica. These pains came in crises, the longest of which had lasted 30 -hours. - -It seems that this soldier’s troubles began the day after his shock with -a feeling of swollen feet and of cotton wool under them. He stayed on -service, however, walking with increasing difficulty. - -At the time of his evacuation, July 10, he could walk with great -difficulty. “Strips of lead were between his legs.” He could hardly -control movements in the dark, or descend stairs. Often his legs would -bend under him. Vesical function sluggish. - -After a few months the patient could walk better. On February, 1916, he -walked thrusting his legs forward trembling, and dragging toes a little. -He could not support himself on either leg. Jerkiness and incoordination -in extension or flexion of leg on thigh. - -The muscular weakness was decidedly against tabes or at all events a -pure tabes. The incoordination proved to be due, not to loss of position -sense (which was intact) but to unsteady muscular contractions. Deep -sensibility was intact. - -There were no mental symptoms. There was a slight hesitation in speech -and doubling of syllables, but nothing demonstrable with test phrases. - -The serum W. R. was positive. Syphilis denied. - - 1. What is the cause of these phenomena? Pitres and Marchand lean to - the hypothesis of slight internal traumatism. They believe that - there is either (a) slight internal hemorrhage in the nervous - system, or possibly (b) what they call “nerve cell contusion,” or - perhaps (c) caisson-disease-like phenomena from aerial - decompression. Some authors incriminate (d) the gases. It has been - reported by certain French authors that shortly after shell-shock - injury or burial there is a pleocytosis in the spinal fluid as - well as evidence of hemorrhage. The pleocytosis is said to last - only a short time; hence when patient arrives at a base hospital - lumbar puncture usually discloses nothing. - - - Baalim and Ashtaroth - - Paradise Lost, Book I, line 422. - - - - - VII. SUMMARY AND KEY - - -No more important human problem now exists than syphilis. Syphilis of -the nervous system or, briefly, neurosyphilis is a highly important -fraction of the total problem. The few outstanding dates and items which -we present on the following page give but a faint idea of the amount of -observation and thinking which the medical aspects of neurosyphilis -alone have required. The present work deals with but a small fraction of -the results of this work, nor can we more than glance at the scientific -history of syphilis and neurosyphilis—a history that would form an epoch -in itself. - -It is only in the most recent years that syphilology and the narrower -science of neurosyphilology have threatened to become separate -disciplines boasting full time specialized workers. Up to recent years -the contributions to the theory of syphilis have been largely -by-products of work in larger sciences and arts. Thus, the cellular -pathology of syphilis as worked out by Virchow and the more special -vascular features as worked out by Heubner were incidental in the -progress of pathological anatomy and histology. The bold procedure of -Quincke in proposing lumbar puncture also had its more general ground in -the extension of clinical medicine,—an interpretation likewise true of -the French achievements in the cyto-diagnosis and chemical diagnosis of -the lumbar puncture fluids. The careful histological definitions of the -Nissl-Alzheimer group were incidental to the application of approved and -classical pathological methods to neurological and psychiatric material. - -Again, the work of Schaudinn, as well as that of Metchnikoff and Roux, -was ingenious work with the methods of parasitology and experimental -pathology. The great work of Schaudinn in establishing the constancy of -the spirocheta pallida in syphilis may be said to have started -syphilology as something approaching a special discipline. The ideas of -one of the greatest of immunologists, Bordet, were almost immediately -applied to the serum diagnosis of syphilis by Wassermann and the further -application of this method to the problems of neurosyphilis was almost -immediate, with the spirocheta pallida as an object of attack. The -commanding intelligence of Ehrlich could at once seek application of -long incubated ideas of chemotherapy with the startling outcome, -salvarsan. - - - =DATES, NEUROSYPHILIS= - - VIRCHOW PATHOLOGY 1858 - HEUBNER ENDARTERITIS 1874 - QUINCKE LUMBAR PUNCTURE 1891 - RAVAUT, SICARD, NAGEOTTI, WIDAL CYTODIAGNOSIS, C.S.F. 1901 - WIDAL, SICARD, RAVAUT ALBUMIN, C.S.F. 1903 - METCHNIKOFF AND ROUX TRANSMISSION TO APES 1903 - ALZHEIMER HISTOPATHOLOGY, BRAIN SYPHILIS 1904 - SCHAUDINN AND HOFFMANN SPIROCHETA PALLIDA 1905 - WASSERMANN, NEISSER AND BRUCK SERUM DIAGNOSIS 1906 - PLAUT WASSERMANN REACTION, C.S.F. 1908 - EHRLICH SALVARSAN 1909 - SWIFT AND ELLIS SALVARSANIZED SERUM 1912 - NOGUCHI AND MOORE SPIROCHETES, BRAIN TISSUE, 1913 - PARESIS - LANGE GOLD SOL TEST 1913 - - CHART 28 - - -The history of syphilis and neurosyphilis was now to be thickly sown -with ideas and results growing from the achievements of Schaudinn and -Ehrlich. The positive reactions in the blood and spinal fluid in the -most striking of mental diseases, general paresis, led to the impression -that general paresis itself might at last be proved to be what Mœbius -had suspected, namely, 100% syphilitic. We know how difficult is the -technical proof of spirochetosis in the brains of general paretics both -post mortem and ante mortem, but no one doubts the certainty of the -syphilitic hypothesis concerning the origin of general paresis. - -The data of the gold sol reaction ultimately obtained from the ideas of -Thomas Graham concerning colloids, as developed by Szigmondi and -effectively applied by Lange, have broadened and solidified the whole -plane of attack. - -The ingenious suggestions of Swift and Ellis (salvarsanized serum) and -the notable work of Noguchi and Moore (spirochetosis in paretic brains) -indicate to us as Americans what the establishment of scientific -institutes may do to permit the rapid application of new ideas to -branches of inquiry that are opened out. Scientific institutes do not -manufacture a Virchow, a Metchnikoff, a Schaudinn, a Bordet or an -Ehrlich but they directly permit such men to work and indirectly -stimulate the development of more. - -The series of 137 cases here at least presented does not touch -systematically the problems of the neuropathology of syphilis, which -would themselves require a textbook of respectable size. We have, -however, presented in Part I, cases 1 to 8, some indication of the -protean nature of the material and from time to time in the remainder of -the book somewhat fuller accounts of the pathological anatomy and -histology have been presented than are strictly necessary in the -demonstration of the principles of modern systematic diagnosis and -treatment. - -Our work may be said to represent psychopathic hospital practice as -available to us in our official capacities at the Psychopathic -Department of the Boston State Hospital. A word is necessary concerning -the nature of this practice. The dispensary and ward practice of a -modern state psychopathic hospital, such as the Boston institution -(founded in 1912) and the Ann Arbor institution (founded in 1906), is to -be sharply distinguished from asylum practice. Those who have not -followed the evolution of the modern psychopathic hospital with the -lowering of bars to the admission of patients and the extension of its -benefits to a group of sick persons far removed from the medicolegal -concept “insanity” may not soon grasp the general nature of psychopathic -hospital material. Psychopathic hospital practice stands, in fact, -almost midway between asylum practice in the classical sense and private -practice. This has come about through the great extension of the -so-called voluntary relation under which hundreds of patients now resort -to the beds and out-patient rooms of a psychopathic hospital, who would -formerly have remained untreated or inadequately treated. Moreover, the -broadening of the concept of mental diseases as a whole has permitted in -some parts of the world the establishment of laws under which -psychopathic and psychotic patients may be brought to psychopathic -hospitals and even to asylums under the easiest possible conditions and -restrictions, omitting court procedure altogether. The operation of the -voluntary and temporary care provisions of law has accordingly yielded -us, in the Boston institution, a great group of cases formerly not at -all accessible to hospital diagnosis and treatment. Needless to say, as -always under such conditions, we have been able to show not merely that -hospital diagnosis or treatment is of importance to a new group of -cases, but also that home treatment, especially home treatment under -supervision, is possible and even ideal for a large group of cases about -which utter darkness or profound misgivings ruled in the not very -distant past. - -Accordingly, we are fain to insist that our material is of importance in -new programs of community organization for the stamping out of disease. -The work in psychopathic hospitals upon neurosyphilis in particular is -essentially a part of the public health program, although our special -work will not soon be taken over by the public health officers, so -complicated are the ramifications of medical and social diagnosis and -treatment in the neurosyphilis group. - -We have tried in Part IV (medicolegal and social cases) to give a few -examples to illustrate the part played by neurosyphilis in society; but -we regard this part of our work as the least satisfactory and the least -representative in the total work. Our colleagues in social service, in -mental hygiene, in psychopathology and in criminology will easily in the -next few years provide a far more adequate basis for a full account of -the public and social aspects of neurosyphilis. One point we should -emphasize here. The psychopathic hospital worker, whether physician or -social worker, must shortly decide upon and consolidate a program with -relation to the families of neurosyphilitics. - -The syphilographers of the dermatological and special syphilis clinics -have their identical problems with the families of syphilitics; but the -dispensaries for mental cases and in particular the psychopathic -hospital and asylum out-patient departments tap another reservoir of -syphilitic families at a stage when the memory of the initial horrors of -syphilitic infection is dimmed or erased. Any program for the diagnosis -and treatment of syphilis of the innocent must take into account not -only the skin, syphilis, and internal medicine clinics but also the -clinics for mental and nervous diseases wherein neurosyphilitics are not -infrequent. Whether the ultimate percentage will stand at 10, 15 or 20% -for the neurosyphilitics in mental clinics, is of no importance to the -principle. There are enough neurosyphilitics having economical -importance and humanly precious families to warrant definite steps. - -The Massachusetts Commission for Mental Diseases has in the last few -years employed the services of two medical workers whose time has been -largely devoted to the applications of our recent knowledge in -neurosyphilis and has gone so far as to establish a neurosyphilis ward -in one of the district state institutions (Summer Street, Worcester, -under the Grafton Hospital Board). Special social workers in the field -of neurosyphilis have also been available from time to time. These -social workers are enabled with the support of the medical profession to -do a great deal of good, for example, with the slogan THE CHILD OF A -PARETIC IS THE CHILD OF A SYPHILITIC. - -The nature of the intake of patients into psychopathic hospital wards -and out-patient clinics is such that great numbers of non-mental -syphilitics arrive for diagnosis and possible treatment. Moreover, the -existence of syphilis in non-suspects is a fact picked up by the way in -routine Wassermann serum diagnosis. - -The mental clinic in the modern sense with the medicolegal bars lowered -or well nigh removed, turns rapidly into a clinic for neurological cases -as well. The German models for mental and nerve clinics are rapidly -being imitated. The result of this administrative novelty in our -hospital procedure has incidentally yielded us many representative cases -of entirely non-psychotic and even non-psychopathic neurosyphilis. Our -impression grows and deepens that _the neurosyphilitic is seldom merely -a spinal syphilitic_. The neurosyphilitic is nearly always the victim -not merely of spinal disease but also of intracranial disease. Per -contra, the victim of intracranial neurosyphilis is almost always more -or less importantly affected by spinal neurosyphilis. - -The net result of the modern work on neurosyphilis has been to bring the -neurologist and the psychiatrist together upon one platform in diagnosis -and more and more upon one platform in treatment. But aside from the -clinical evidence that the neurosyphilitic is apt to be a victim of both -brain syphilis and cord syphilis, the autopsy evidence is stronger -still. Even the victim of tabetic neurosyphilis (“tabes dorsalis”) -himself is rarely found at autopsy without more or less evidence of -significant encephalic disease of a chronic inflammatory or degenerative -nature. Aside from tabes dorsalis and Erb’s paraplegia, the rule is -almost universal that neurosyphilis is a matter of the entire nervous -system. - -In view of the generalization of neurosyphilitic process, one might -question the advantage of any topical grouping of neurosyphilitic -disease. Practically speaking, however, as we have shown in Chart 5, it -seems advisable to separate the neurosyphilitic diseases into six -roughly distinguishable groups. First, there is the great group that we -have chosen to term =diffuse neurosyphilis=, including many of the cases -of so-called cerebral or cerebrospinal syphilis of the neurological -clinics and the group of cases that have been treated in private -practice by internists and neurologists without recourse to -institutions. These cases have lived at home and have not been socially -hard to manage until the late phases of their disease when the victims, -if poor, are sent to almshouses and infirmaries under municipal or state -care. These are the cases which have been in the past regarded as most -amenable to the classical iodid and mercurial treatment. Indeed there is -record of numerous therapeutic successes in the group. - -Whereas the lesions in diffuse neurosyphilis are chiefly chronic -inflammatory and degenerative changes of a diffuse nature (with vascular -changes incidental or subordinate to the inflammation and the -degeneration), there is an important and large group of cases that we -have termed =vascular neurosyphilis= in which the factors of -inflammation and degeneration are subordinate to vascular insults. These -are cases of syphilitic arteriosclerosis and the best examples are -victims of cerebral thrombosis. The clinical symptoms of the immediate -attacks (of apoplectiform, epileptiform or other acute nature) are not -in themselves distinguishable from the immediate effects of -non-syphilitic vascular disease; nevertheless the establishment of their -syphilitic etiology is of the utmost importance on account of the -possibilities of treatment of the underlying syphilis. For, as the -neuropathologist must always insist, the immediate effects of vascular -insults whether syphilitic or non-syphilitic are much more extensive -than the ultimate paralytic or residual irritative effects; and by -consequence a greater optimism is justifiable in the confronting of -these cases than the nihilistic observer is likely to entertain. - -Physicians dealing with chronic disease in general are apt to be -somewhat nihilistic, but this nihilism is increased a hundred fold in -disease of the nervous system. How important then is any work which -shall demonstrate partial or even complete recovery from serious looking -apoplectic and other seizures, besides all of which the point of -syphilitic treatment naturally lies in the prevention of future insults -of the same sort. Therapeutic experience in this vascular group has -almost as good a toll of successes as in the diffuse neurosyphilis group -above mentioned, that is to say, the modern systematic treatment and -even the old pre-salvarsan treatments have succeeded fairly well in -removing the products of inflammation from the membranes of the nervous -system and in abolishing vascular disease. - -The old principle that the dead neurone in the central nervous system -cannot be regenerated remains a perfectly firm principle; but there are -any number of neurones and even neurone systems that are not essential -to life or to the pursuit of happiness. We accordingly have just as good -a theoretical therapeutic outlook in many instances of chronic -neurosyphilis as we have in chronic diseases of many other organs. Add -to this the fact that a great number of the most sharply-defined and -grave symptoms are probably not due to destruction of neurones but to -irritation and functional disability of neurones, and the conclusion is -compelled that, as hinted above, an entirely unjustifiable pessimism and -nihilism have prevailed in some quarters. Of course, the recoil from -such pessimism with the onset of salvarsan treatment led various -enthusiasts to an undue optimism. - -Another great group distinguished by the existence of spinal cord -disease is the group we have termed =tabetic neurosyphilis=, which group -contains the classical tabes dorsalis or locomotor ataxia and its -congeners. - -The question of therapeutic optimism comes up most forcibly in the field -of tabes. It is hard, however, at this time to give a proper and -scientifically founded estimate of the therapeutic outcome in tabetic -neurosyphilis with modern methods. So much can be said: namely, that the -alleviation of pain and the palliation of other symptoms can be -successfully claimed as a result of the renewed interest in the -treatment of this affection. What was said above concerning the finality -of the death process in a dead neurone is very strikingly true, of -course, of some of the neurones of the posterior columns in tabes -dorsalis. Still only portions of these neurones (namely, those which run -an intradural course) are strikingly altered in a great many cases. Now -and again one is greatly astonished to observe the restoration of the -lost knee-jerk in cases of neurosyphilis (see for instance the case of -Alice Morton (1), with discussion). In short, the relation of several -tabetic symptoms to irritative conditions and functional disability of -neurones may be considered established. Naturally, moreover, if therapy -can stop the upward course of the affection as it passes from lower to -higher nerve roots (according to reasonably well-established ideas of -the genesis and progress of this affection), we are entitled to a -further degree of optimism. - -The question of therapeutic optimism _versus_ pessimism is forced upon -attention in the fourth great group of neurosyphilitic diseases which we -have chosen to distinguish, namely, the group of =paretic neurosyphilis= -including the disease formerly known as general paresis, paralytic -dementia, softening of the brain and the like. - -Of course, no one can gainsay there is a group of cases having in the -natural course of events a prognosis of fatality within a term of years, -say three to five years, and we have cases in our series which go to -show that even with the modern intensive treatment the characteristic -down-grade symptomatic progress and ultimate fatality occur. Still, we -have other cases diagnostically on all fours with the fatal cases that -have seemed to get either entirely well with the laboratory tests -returning to normal and without further mental symptoms, or else lose -mental symptoms on the one hand or laboratory signs on the other. We -should strongly object to any account of paretic neurosyphilis which -should insist that its necessary outcome is fatality within a term of -years. Of course, viewing our knowledge of the affection in the past, we -should be compelled to object to the generalization “_paresis fatal_” on -the evidences of the universally recognized remissions. If nature can -stop a paretic process, why cannot man do as much? Can it be alleged -that our own apparent therapeutic successes and those of others are -merely curious examples of coincidences, namely, that remissions have -chosen to occur precisely when therapy was systematically applied? The -percentage of therapeutic successes with modern intensive treatment, -wherever it may ultimately stand, is already too high for this -hypothesis of fortuitous remissions.[31] - -Moreover, we believe that the details of the clinical progress of some -of the reported cases are convincing on this point. What, however, is -the distinguishing feature of paretic neurosyphilis? It is in one sense -a particular kind of diffuse neurosyphilis. The tissues are apt to show -not only encephalic but also spinal changes. There is apt to be a more -or less well-defined meningitis, but the characteristic feature, without -which the diagnosis of paretic neurosyphilis would hardly be rendered, -is the existence of disease of the cerebral cortex. This disease is -parenchymatous in the sense of showing nerve cell destruction. There is -also an interstitial reaction in the shape of a neuroglia overgrowth, -but the striking and pathognomonic feature is the infiltration of the -sheaths of the small vessels in the cortex, giving evidence of an -inflammation very intimately affecting the cellular mechanisms of the -nervous system. It is striking how often a smaller or larger share of -the cells found in the vessel sheaths are plasma cells. It does not -appear, however, that the diagnosis of paretic neurosyphilis as against -diffuse non-paretic neurosyphilis can be made in the stained sections -with complete safety on the basis of plasmocytosis in the former and -lymphocytosis in the latter. Whatever the results of careful -histological differentiation by future neuropathologists may yield, it -is at all events true that we cannot yet make an important -differentiation clinically on the basis of the differential count of -plasma cells and lymphocytes in the puncture fluids. However this may -be, there is an important distinction between diffuse neurosyphilis of -the non-paretic type and paretic neurosyphilis in that paretic -neurosyphilis rarely if ever fails to show important degrees of -intracortical perivascular inflammation with larger or smaller numbers -of plasma cells. - -What has the therapeutist to face in this matter? The answer, as -elsewhere, depends somewhat upon what the future may decide as to the -habitat and toxic or antitoxic activities of the spirocheta pallida. The -early claims that the spirocheta pallida was extravascular and lay for -the most part in the parenchyma and not in the vessel sheaths were -perhaps overbold, since other workers have found the spirochete in the -vessel sheaths also (Mott). - -Aside from the spirochete and its accessibility to spirochetocidal -drugs, there seems to be no reason for supposing that the perivascular -sheaths cannot be cleansed of their inflammatory contents. There is, -again, no reason why the phagocytic cells should not continue to perform -their scavenger function until such time as the degenerative process in -the parenchyma (a process not necessarily progressive in the absence of -the spirochete or its products) ceases. There is every reason to suppose -that a great many of the clinical phenomena are not necessarily due to -permanent destruction of neurones and neuronic organs (dendrites, -axis-cylinders, nets and the like) but are due to various microphysical -conditions of pressure, intoxication and the like. - -The inflammatory conditions in the spinal cord of poliomyelitis, which -conditions are precisely as striking as those of the paretic cortex, are -beyond a question cleared away in the progress of the affection. -Reference to the paradigm case (1) will show the type of our argument. -There is no manner of doubt that in this paradigm case almost every -portion of the nervous system had been sometime swept by spirochetosis -and many of its small vessel sheaths stuffed with chronic inflammatory -products. As for paretic neurosyphilis itself, a great many of its most -striking clinical phenomena, such as loss of memory and disorientation, -as well as great degrees of apparent dementia, are found virtually as -often in cases with very slight anatomical changes as in cases with -marked cortical devastation. The inference is plain, that these -phenomena are to a degree functional rather than structural. - -In brief, we conclude not only from therapeutic experience but also on -_a priori_ grounds that the histological conditions in paretic -neurosyphilis are not entirely hopeless, and certainly not more hopeless -than conditions in many chronic diseases outside the nervous system. -Accordingly, we plead for a temperate optimism as to therapeutic results -in general paresis. - -A fifth group of neurosyphilitic cases bulking rather largely in -textbooks of pathology is the group of the =gummata=. For a variety of -reasons (therapeutic and otherwise) the actual number of gummata of the -nervous system available for clinical or even for anatomical study is -much smaller than the books might lead one to infer. - -The sixth and last of the main groups of neurosyphilitic diseases is -that of the =juvenile forms=, among which we find not only diffuse forms -without a special and well-defined course, but also characteristic -examples of paretic and tabetic neurosyphilis. The distinction of a -juvenile or congenital group of neurosyphilitics is, on theoretical -grounds, perhaps hardly defensible. On practical grounds, however, the -juvenile neurosyphilitics do form a group having special relations to -feeblemindedness, epilepsy and the like. - -We must be clearly understood as to the rough, six-unit classification -just given. It is practical merely. For comparison we have given in -other charts more expanded lists of the diagnostic entities in -neurosyphilis among which that of Head and Fearnsides is of special -interest, see Chart 2, page 21. - - -We shall now proceed to a brief analysis of the findings in our chosen -series of 137 cases. We shall not reproduce the case headings of these -cases, but expand their statements where necessary and tie them together -so far as possible into a reasonable and systematic statement of the -situation in neurosyphilis. The footnotes will contain references to -other cases in which identical points are illustrated as in the leading -cases. The leading cases will in all instances be placed first in the -footnotes. - -The paradigm[32] shows meningeal, vascular and parenchymatous lesions -and thus illustrates our definition of the term DIFFUSE which means -precisely meningeal, vascular and parenchymatous. The meningeal lesions -gave rise to two prominent sets of lesions, first, the marked tabetic -lesions of the spinal cord (due to the spinal root neuritis incidental -to the spinal meningeal inflammation), secondly, the characteristic -asymmetrical and focal atrophy of cranial nerves incidental to a now -largely extinct meningeal process at the base of the brain. The vascular -lesions are responsible for another important and characteristic factor -in the case, namely, the bilateral pyramidal tract sclerosis; the -bilateral cysts of softening of the corpora striata are characteristic -effects of old syphilitic cerebral thromboses. The parenchymatous -disease in our paradigm is everywhere obvious, less so perhaps in the -cortex itself than elsewhere, although here also evident in the shape of -lesions suggesting an early phase of tissue atrophy. - -The paradigm is of interest in demonstrating what in broad lines must be -taken as an ascending disease proceeding not only from spinal cord to -encephalon but also traceable as proceeding from lower parts of the -spinal cord to upper parts thereof and from the lower encephalon to the -higher structures of the cerebral cortex itself. - -The paradigm insistently calls attention to the advantage of persistent -therapy not only in its display of remarkable successive recoveries from -permanent looking symptoms but also histologically from the remnants of -inflammatory process to be found in an otherwise almost wholly -dismantled nervous system with extinct lesions. - -TABETIC NEUROSYPHILIS[33] (“tabes dorsalis”), of course, often proceeds -to death without special complications of syphilitic nature. We have -chosen a case, however, to demonstrate a terminal complication with -vascular insult. Incidentally the case shows another complication -inasmuch as the cause of death was rupture of aortic aneurysm. It is -important to bear in mind these complications in tabes dorsalis which go -to prove that the spirochetosis of tabetic neurosyphilis is not limited -to the region of the spinal roots or to the spinal region in general. -Tabetic neurosyphilis is apt to be only a part of a total picture of -neurosyphilis just as neurosyphilis itself is only a part of the general -syphilitic process. - -Our case of PARETIC NEUROSYPHILIS[34] (“general paresis”) is a -characteristic one in duration (three years and three months). The -aortic sclerosis almost constantly found in neurosyphilis and especially -in paretic neurosyphilis is here also shown. The spinal cord showed -lesions which are also almost always found in paretic neurosyphilis. The -characteristic frontal emphasis of the atrophic and indurative lesions -is shown. There is also a display of gross changes in the pia mater. The -characteristic so-called granular ependymitis or sanding of the -ventricular surface is shown. The case is distinguishable from the -paradigm in not showing the effects of vascular insults in the shape of -cysts of softening. The cerebellar sclerosis of the case is fairly -characteristic of paretic cases. There is even a suggestion of atrophy -in the temporal region suggesting the so-called Lissauer’s paresis. -Clinically the case belongs in the classical grandiose group of paretics -(“O. K. No. 1 superfine”). - -VASCULAR NEUROSYPHILIS[35] is illustrated in a fourth autopsied case. It -may be noted that the pia mater in this case is practically normal. The -tissues outside the area of softening due to the syphilitic thrombosis -of nutrient vessels are practically normal. The case was one of almost -complete sensory aphasia with word-deafness. The clinical picture is -accordingly quite distinct from those of the paradigm (1) and of the -case of general paresis (3) just discussed. - -JUVENILE PARESIS[36] is illustrated by a case with exceedingly extensive -lesions, largely meningeal and parenchymatous. The cerebral lesions are -atypical since in places they suggest the tuberous sclerosis of -Bourneville. The brain atrophy is extreme (965 grams) and it is possible -that this apparent brain atrophy was in part hypoplasia, since the -spirochetosis of this case was doubtless congenital. However, clinically -the patient was fairly normal up to the age of 18. - -A case of so-called SYPHILITIC EXTRAOCULAR PALSY[37] demonstrates a -characteristic meningeal process more extensive than the clinical -symptoms would have indicated. In fact, focal clinical nerve palsies are -as a rule, if not constantly, partial phenomena of a far more extensive -process of neurosyphilis. They are far more limited clinically than -anatomically and histologically. It seems at first sight improper to -term them cases of diffuse neurosyphilis in view of their clinical -focality, yet they are best described as partial cases of diffuse -neurosyphilis. - -A case of GUMMA[38] of the left HEMISPHERE is presented which appears to -have led to death in about four years from onset. This case, like many -others, is not an example of purely focalized syphilitic process -inasmuch as cysts of softening indicating slight vascular insults are -present elsewhere (pons). There is also a degree of leptomeningitis, -particularly basal. - -Our discussion of the nature and forms of neurosyphilis is completed by -a rare case probably belonging in the so-called _cervical hypertrophic -meningitis of Charcot_ but actually due to a GUMMA OF THE SPINAL -MENINGES.[39] The importance of therapeutic optimism is emphasized in -this case as in the paradigm. Theoretically the meningeal inflammation -of neurosyphilis ought to be almost entirely if not entirely removed by -therapy, and these two cases, like several others in the series, seem to -illustrate this possibility. - - -Neurosyphilis sometimes receives the clinical diagnosis neurasthenia -simply through omission to apply proved diagnostic methods. An instance -is given in which the PARETIC form of NEUROSYPHILIS (“general paresis”) -received the diagnosis _neurasthenia_[40] for a period of five years, at -any time during which period it would doubtless have been possible to -render the correct diagnosis and apply treatment. - -Neurosyphilis may imitate not only the psychoneuroses but also the -psychoses themselves. We present a case of an architect, which looked -almost precisely like _manic-depressive psychosis_[41] and had a history -of attacks, but in which the positive serum W. R. led (in accordance -with hospital rules) to an examination of the spinal fluid. The spinal -fluid tests proved the case to be one of PARETIC NEUROSYPHILIS. - -However, a positive serum W. R., even when associated with mental -symptoms, and when those mental symptoms include grandiosity, does not -prove the existence of neurosyphilis either in its paretic or -non-paretic form. Our instance seems to be one of MANIC-DEPRESSIVE -PSYCHOSIS.[42] The spinal fluid tests were entirely negative. The course -of the disease was also that of manic-depressive psychosis. In the -absence of positive spinal fluid tests, the diagnosis neurosyphilis was -excluded. - -Neurosyphilis and even PARETIC NEUROSYPHILIS may result in symptoms that -would ordinarily lead to the diagnosis _dementia praecox_.[43] - -It is important not to rule out neurosyphilis on the ground of a -_negative serum_ W. R. The fluid W. R. may turn out positive. We present -a case (of a salesman)[44] in which the serum W. R. was repeatedly -negative (even salvarsan did not act provocatively) yet the spinal fluid -W. R. proved positive. The case was clinically one of classical PARETIC -NEUROSYPHILIS (“general paresis”). It is a good rule to proceed to -lumbar puncture, even when the serum W. R. is negative, if there are -suspicious symptoms (e.g., speech defect and memory impairment, -grandiosity) or signs (e.g., marked reflex disorder, especially -pupillary disorder). - -DIFFUSE NEUROSYPHILIS was above defined as -“meningovasculoparenchymatous.” This disease is typically associated -with six positive tests (positive serum W. R., positive fluid W. R., -pleocytosis, gold sol reaction, positive globulin reaction and excess -albumin). One or more and frequently several of these six tests are -likely to run mild in diffuse neurosyphilis; that is to say, these tests -are apt to run milder than the identical tests in paretic neurosyphilis -(“general paresis”). The clinical course of the diffuse, and especially -the meningovascular cases, is likely to be protracted. The prognosis as -to life is good, barring fatal vascular insults. The illustrative -case[45] was a case with slow course. There was a series of attacks -followed by a paralytic stroke, a finding highly typical of the diffuse -form of neurosyphilis. The spinal fluid reactions were mild, suitable to -the general principle above stated. - -These tests are likely to run stronger, as above stated, in paretic -neurosyphilis (“general paresis”), than in the diffuse form. In -particular, the gold sol reaction is likely to be shown in what is -termed “paretic” form rather than in what is termed “syphilitic” form. -The clinical course of PARETIC NEUROSYPHILIS is likely to be brief. A -characteristic case[46] with very heavy globulin and albumin tests is -presented. - -TABOPARETIC NEUROSYPHILIS[47] (“taboparesis”) is clinically a -combination of the symptoms of tabetic (“tabes dorsalis”) and those of -paretic neurosyphilis (“general paresis”). First comes the tabes -dorsalis lasting often for many years. Afterward follows a -characteristic general paresis. The ultimate paretic picture is likely -to retain, however, various characteristics of tabes. The laboratory -tests in the paretic phase of taboparesis are characteristic of general -paresis and not of tabes dorsalis. The prognosis after the paretic phase -has arrived is that of general paresis. - -The diagnosis of the neurosyphilitic forms would be easy if these -principles were always carried out to the letter. The important fact is -as follows: diffuse (that is, meningovasculoparenchymatous -neurosyphilis) may look like paretic neurosyphilis (“general -paresis”)[48] at certain periods of the clinical and laboratory -examination. This fact is of obvious importance. The general prognosis -of diffuse neurosyphilis is regarded as good _quoad vitam_. The general -prognosis of paresis is bad. If, however, the differential diagnosis -cannot be rendered at particular phases of a given case, then no safe -prognosis can be offered in the individual case. In particular no -prognosis affecting the administration or non-administration of modern -systematic treatment can or should be offered in these doubtful phases. - -It is not always safe to exclude neurosyphilis even when the _fluid_ W. -R. is _negative_.[49] Particularly in vascular neurosyphilis the fluid -W. R. and even all the other laboratory signs in the spinal fluid may -sometimes be negative. A positive serum W. R. yields the correct pointer -to diagnosis. Of course, also in many cases of vascular neurosyphilis -one or more of the laboratory signs may be suggestive even when the -fluid W. R. is negative. Theoretically there may be cases in which all -the six tests are negative and yet the diagnosis neurosyphilis be the -correct one. - -A clinically important sign in neurosyphilis is the so-called -_seizures_. These occur both in DIFFUSE NON-PARETIC NEUROSYPHILIS[50] -and in PARETIC NEUROSYPHILIS.[51] - -_Aphasia_ is likewise a symptom in both these forms of neurosyphilis, -namely, in the DIFFUSE non-paretic[52] and in the PARETIC form.[53] - -The literature contains reference not only to seizures and aphasia as -characteristically paretic but also to _remissions_. Remissions like -seizures and aphasia are found in both the PARETIC[54] and NON-PARETIC -forms of neurosyphilis.[55] They have important bearings on prognosis in -all forms of neurosyphilis and are of especial significance in the -evaluation of treatment. (Remissions coincident with apparent cure.) - -So far we have been dealing with cases of neurosyphilis in which there -was no doubt of the existence of mental symptoms. There are cases, -however, in which although the laboratory signs of neurosyphilis exist, -proving beyond doubt the existence of a chronic inflammatory reaction -and allied pathological conditions in the cerebrospinal axis, there are -no mental symptoms of neurosyphilis. We have called some of these cases -PARESIS SINE PARESI[56] and present examples. - -To illustrate complications we give a case of PARETIC NEUROSYPHILIS with -autopsy in which there were ante mortem signs of HERPES ZOSTER[57] or, -at all events, of a skin eruption limited to the area of a thoracic -nerve. - -A case of GUMMA of the brain[58] in which decompression was warranted -and performed is presented. The fluid W. R., as in many such cases, was -negative; serum positive. - -A case of CRANIAL NEUROSYPHILIS (extraocular palsy[59] without mental -symptoms) showed a positive Wassermann serum test and a negative spinal -fluid. - -The laboratory reactions in TABETIC NEUROSYPHILIS[60] (“tabes dorsalis”) -run somewhat like those of diffuse non-paretic neurosyphilis and are -accordingly milder than those of paretic neurosyphilis. The fluid W. R. -and the gold sol reaction in particular are apt to run mild. The -clinical course of tabes dorsalis is well known to be protracted and the -prognosis _quoad vitam_ is good except that we must always bear in mind -the possibility of vascular insults and complications of a syphilitic -origin in the rest of the body. - -It is important to remember that TABETIC NEUROSYPHILIS is often quite -atypical[61] clinically and may even show no single symptom warranting -the old clinical name locomotor ataxia. - -There are even cases in which the name tabes dorsalis is not warranted -in view of the fact that the lesions are not low in the cord but are -higher up (TABES CERVICALIS[62]). - -A rare form of neurosyphilis is ERB’S SYPHILITIC SPASTIC PARAPLEGIA[63] -against which one needs to consider a number of non-syphilitic spinal -cord diseases. Our case showed a weakly positive serum W. R., a negative -fluid W. R., and the other tests of the spinal fluid were moderately -positive. - -SYPHILITIC MUSCULAR ATROPHY[64] is classified by Head and Fearnsides -both in their meningovascular group and in their group of the so-called -syphilis centralis. Our case affecting in large part the small muscles -of the hands in a teamster, may be due either to spinal parenchymal -lesions or to root neuritis or to both. - -It is a little extraordinary and very important that the _laboratory -signs_ are apt to be positive even in the SECONDARY period of SYPHILIS. -Perhaps a third of all cases of syphilis in the secondaries would, if -tested, yield positives precisely like those of full-blown paretic or -diffuse neurosyphilis. Strangely enough, these signs may occur without -clinical symptoms. The illustrative case,[65] a mechanic, yielded -various mental symptoms. The cases of secondary syphilis with laboratory -signs of neurosyphilis but without clinical symptoms are of the greatest -theoretical importance in relation to the problem above mentioned of -_paresis sine paresi_. It may well be inquired whether in some instances -the neurosyphilis of the secondaries does not persist until the -exhibition of mental or physical symptoms of neurosyphilis years later. -It must be remembered that this conception is hardly more than a -hypothesis at the present time. That such signs of chronic inflammation -could exist without symptoms is not so surprising when one thinks of the -startling immediate improvement seen after treatment or even in -remissions without treatment. One is reminded of the crisis in pneumonia -wherein clinical improvement takes place entirely independent of the -mechanical conditions in the lung which just after the crisis remain as -suppurative as before. - -The diagnosis of JUVENILE NEUROSYPHILIS is made upon the same lines as -that of neurosyphilis in the adult. We present two cases, one with optic -atrophy[66] and the other with signs of congenital syphilis antedating -the symptoms of paresis.[67] - -Congenital syphilis is also apparently capable of producing a simple -form of FEEBLEMINDEDNESS,[68] that is to say, a form of disease -non-paretic, non-tabetic, and without special tendency to vascular -insults. - -We present a case of JUVENILE TABETIC NEUROSYPHILIS (“juvenile -tabes”).[69] The tests were all positive. - -The line of separation between typical and atypical cases of -neurosyphilis is vague and indistinct and some of the cases classified -by us amongst puzzles perhaps belong under systematic diagnosis and vice -versa. The section on PUZZLES AND ERRORS in the diagnosis of -neurosyphilis is introduced by six cases of error in the diagnosis of -the paretic form of neurosyphilis.[70] These errors were made known by -autopsy. Aside from the sixth case, whose etiology must remain in doubt -and which was a unique case of PERIVASCULAR GLIOSIS, there is ground for -the belief that the other five cases in this Danvers Hospital study of -diagnostic errors were perhaps actually syphilitic though not of the -paretic form of neurosyphilis. At all events, the brain tissues in these -cases failed to show the plasma cell deposits which are characteristic -in the sheaths of the intracortical vessels in paretic neurosyphilis. - -A case illustrates the complication of TABES by _arteriosclerotic -symptoms_, in which case the arteriosclerosis may naturally have been of -syphilitic origin. Two cases especially illustrate the possibility of -confusing the ataxia of general paresis with CEREBELLAR ATAXIA. These -cases showed lesions of the cerebellar structures, notably of the -dentate nucleus. No one can read these cases or any of the autopsied -cases in our series, without perceiving how fundamental and even -critical is the demand for autopsies in fatal cases of neurosyphilis. -The practitioner who can secure an autopsy in a fatal case of -neurosyphilis and have the tissues worked up by approved -neuropathological methods is almost bound to add his bit to neurological -theory. Even cases of classical tabes dorsalis are often signally -important to the theorist on account of the relations of the neural to -the non-neural complications. - -We then proceed to a group of cases without special order in which a -variety of diagnostic questions arose. - -A case of questionable neurosyphilis in the secondary stage of syphilis -brings up the problems of syphilitic _neurasthenia_.[71] - -Syphilis may act as _agent provocateur_ of HYSTERIA as Charcot -insisted.[72] - -A case illustrative of difficulties in diagnosis between neurosyphilis -and manic-depressive psychosis follows.[73] - -A case for diagnosis is given which shows that errors in the diagnosis -of neurosyphilis are entirely possible even when abundant clinical and -laboratory data are available. A case with a weakly positive Wassermann -reaction in the spinal fluid finally turned out to be one of BRAIN -TUMOR.[74] - -Some questions as to the diagnosis of NEUROSYPHILIS _versus Idiopathic -Epilepsy_ are brought up by a case in which phenomena of paresis seemed -to have occurred very early, about two years after the initial -syphilitic infection.[75] - -A case of PARETIC NEUROSYPHILIS is offered in which _hemiplegia_ and -_hemitremor_ strongly suggested _vascular_ lesions; but the autopsy -showed no coarse lesions and merely confirmed the diagnosis paresis -microscopically.[76] - -An autopsied case of PARETIC NEUROSYPHILIS is given, in which the pupils -persisted in reacting normally. Herpes zoster-like lesions in life -yielded no special signs at autopsy (all root-ganglia looked alike above -and below zone of “shingles.”)[77] - -An example of NEUROSYPHILIS, probably PARETIC, yielded symptoms highly -suggestive of _manic-depressive psychosis_.[78] An interesting feature -in this case was the birth of a healthy child nine months after the -onset of the psychotic attack. - -An example of _exophthalmic goitre_[79] following the acquisition of -SYPHILIS showed at autopsy a heavy scarring of the optic thalamus and -unilaterally atrophic process in the cerebral cortex. - -We come to some questions concerning the _Argyll-Robertson pupil_. It is -agreed on all hands that the Argyll-Robertson pupil is characteristic of -the paretic and tabetic forms, but the sign occurs also in other -neurosyphilitic conditions;[80] in fact the sign does not necessarily -indicate neurosyphilis as an instance of PINEAL TUMOR demonstrates.[81] - -The question raised above as to the possibility that neurosyphilis may -exist in the absence of positive findings in the spinal fluid is -illustrated in a man, a mechanic, who claimed syphilitic infection and -showed an _Argyll-Robertson pupil_ on one side.[82] The serum W. R. was -positive; the _fluid tests_ were _negative_. - -An extraordinary case is given in some detail in which NEUROSYPHILIS in -the form termed DISSEMINATED ENCEPHALITIS[83] proved fatal within seven -months of the initial infection. - -We have frequently mentioned the classical assumption that paretic -neurosyphilis (“general paresis”) is a fatal disease. Some have -suggested that there is another form clinically almost identical with -general paresis except that it pursues a long course and the suggestion -has been made that these cases be termed _pseudoparesis_.[84] We are of -the opinion that this term should be dropped and advocate the use of the -word pseudoparesis only for non-syphilitic disease looking like paresis, -such as alcoholic pseudoparesis and the like. - -The question whether there is a form of mental disease SYPHILITIC -PARANOIA[85] is raised by a case with auditory hallucinations, ideas of -persecution and attacks of excitement. The diagnosis of alcoholic -hallucinosis was actually made although there is no proof that the -patient ever drank alcohol. - -Alcohol may cause symptoms identical with those of paretic -neurosyphilis, including seizures, Argyll-Robertson pupils, speech -defect and mental symptoms. The differentiation is readily made by the -negative laboratory findings. An illustration is given in our case of -the alcoholic teamster. Cases such as this bear the name ALCOHOLIC -PSEUDOPARESIS.[86] - -However, when the clinical picture is the same as in the case of our -teamster, the alcohol may only be a complicating factor in -neurosyphilis, as shown by our next case of the _alcoholic_ steamfitter -who in fact was shown to have NEUROSYPHILIS.[87] - -Sometimes cases of apparently frank _alcoholism_, even with apparently -characteristic delirium tremens and neuritis, prove to be essentially -neurosyphilitic.[88] On the other hand, true combinations of ALCOHOLISM -and NEUROSYPHILIS occur which it would be proper to classify under -either heading and in which therapy must take serious account of both -conditions.[89] - -As above stated, we elect to use the term pseudoparesis only for -non-syphilitic cases. There are other forms of pseudoparesis than -alcoholic pseudoparesis. The question of _Diabetic Pseudoparesis_ is -raised by an exceedingly complicated case of which our best -interpretation is that the patient, a proved syphilitic (with syphilitic -osteomyelitis (?)), a huge doorkeeper, was perhaps suffering from an old -SYPHILITIC scarring of the PITUITARY body.[90] Neither this case nor a -second case, one of PARETIC NEUROSYPHILIS with _glycosuria_ is actually -entitled to the diagnosis diabetic pseudoparesis. The second case of -paretic neurosyphilis with glycosuria brings up some unanswerable -questions as to the pancreatic or basal meningitic or other origin for -the glycosuria.[91] - -_Isolated symptoms_ are often presented by neurosyphilitics (e.g., -hemianopsia);[92] but we tend to regard these cases as due to focal -lesions that are merely part and parcel of DIFFUSE LESIONS. - -A neurosyphilitic case (a steward) with the rather unusual complication -(for our northern region) of severe MALARIA producing cerebral -thrombosis is reported.[93] - -The diagnosis _Dementia Praecox_[94] was actually made in the case of a -young school-teacher in whom the laboratory findings proved conclusively -that the condition was one of NEUROSYPHILIS. The gold sol reaction in -this case was mild. The chief lesion at autopsy was a fresh looking, -gelatinous pial exudate over the spinal cord which turned out to contain -an almost pure display of very numerous plasma cells. - -The question of LUES MALIGNA[95] is brought up in a rectifier of spirits -in whom the characteristic tremendous destruction of tissue, toxemia and -failure to react to antisyphilitic treatment were illustrated. Moreover, -this case had a trauma (cautery) to the tonsil, as in other cases of -lues maligna. - -A case somewhat suggestive of _brain tumor_, of _neurosyphilis_ and of -_multiple sclerosis_[96] turned out to be MULTIPLE SCLEROSIS (the fluid -showed a pleocytosis and a moderate amount of globulin with a paretic -type of gold sol reaction). - -As a foil to this case that we regard as multiple sclerosis, we present -a second case with nystagmus, optic atrophy and spasticity in which the -suspicion of _multiple sclerosis_ might well be raised but which the -tests demonstrated to be NEUROSYPHILITIC.[97] - -An even stranger imitation of well-defined non-syphilitic entities was -presented by a case apparently of _Huntington’s chorea_[98] (except for -absence of the hereditary taint) which case, however, proved to the -surprise of all diagnosticians to be one of NEUROSYPHILIS. - -Frequent errors of diagnosis must occur in the field of the senile -psychoses. We present a case that would at first blush warrant the -diagnosis of _senile arteriosclerotic psychosis_[99] in a sea captain of -75 years (wife dead 15 years before of general paresis) who turned out -to be a characteristic case from the laboratory standpoint of -NEUROSYPHILIS. - -The Protean nature of the symptomatology of neurosyphilis is -sufficiently established. Still, a case that might fit into textbooks -concerning DISSOCIATION OF PERSONALITY[100] is certainly a clinical -oddity, as illustrated by a fugacious musician. - -A case with strong suspicions of _neurosyphilis_ of _tabetic_ type -turned out to be more probably one of neural complications in PERNICIOUS -ANEMIA.[101] - -NEUROSYPHILIS IN JUVENILES presents puzzling conditions. - -One case was marked clinically by _attacks of excitement_.[102] It is -impossible to place this case among the main groups of juvenile -neurosyphilis. - -Another case of FEEBLEMINDEDNESS,[103] also NEUROSYPHILITIC in origin, -presented physical symptoms and laboratory signs of paretic -neurosyphilis; yet this case had been considered one of _simple -feeblemindedness_. - -A case apparently of JUVENILE PARETIC NEUROSYPHILIS in a 15 year old boy -presented the rather unusual complication of shocks with -quadriplegia,[104] a _vascular complication_ not usually expected in the -paretic type of neurosyphilis in adults. - -Epileptic phenomena[105] are rare as the effect of JUVENILE -NEUROSYPHILIS, but occur as demonstrated in a case which slipshod -methods of diagnosis might well have regarded as one of _idiopathic -epilepsy_. - -A case of JUVENILE PARETIC NEUROSYPHILIS with the complication of -ADDISON’S DISEASE[106] is given (autopsy confirmation). - -The puzzle in diagnosis offered by syphilis in the secondary stage[107] -is illustrated by a case which showed the characteristic NEUROSYPHILITIC -complications of the SECONDARY STAGE of syphilis. This patient may well -have been a moron at the outset and exhibited some reactions (refusal to -talk) explicable on the basis of feeblemindedness. She was a -neurosyphilitic only in the sense of the neural complication that we -find in the secondary stage of syphilis. As stated above, we do not yet -know what the fate of these neural complications of secondary syphilis -is to be. The frequency of this finding in secondary syphilis is -probably too great to warrant the hypothesis that it must always go on -to a chronic neurosyphilis; but we certainly are warranted in regarding -these cases as potential chronic neurosyphilitics. - -A case of TABOPARETIC NEUROSYPHILIS in which the heavy exudate -characteristic of paresis became a soil for a growth of the typhoid -bacillus is presented with autopsy.[108] This fatality with TYPHOID -MENINGITIS is merely a concrete example of the many complications which -syphilitics and especially neurosyphilitics have to sustain. - - -The case series then goes on to illustrate, though quite inadequately, a -variety of MEDICOLEGAL AND SOCIAL complications of neurosyphilis. It is -well known that many social complications with grave moral, economic and -even political difficulties occur. - -Our series starts with a “public character”[109] whose eloquence and -reformatory efforts led to a considerable notoriety. The autopsy in this -case showed singularly few lesions despite the fact that the case was -microscopically one of wholly characteristic PARETIC NEUROSYPHILIS. The -question might arise how far we are entitled to correlate the -reformatory efforts of this always eccentric character with syphilis. -The man himself a physician, was aware of the doubt which his -Argyll-Robertson pupils threw upon his medical situation. He explained -them on the basis of an old smallpox! We are inclined to think that the -whole of this man’s life, from his giving up of medical practice to live -as a kind of literary and political hack, was due to subtle changes of -neurosyphilitic origin. The fact that there was a certain delinquent -streak in the man is not inconsistent with this idea. Interestingly -enough, a fall on the ice in the man’s 61st year actually started up the -fatal process, a condition of affairs amply illustrated in cases of -neurosyphilis, brought out by trauma that come to the attention of the -Industrial Accident Board in connection with claims for compensation. - -A case of sudden _grandiosity_[110] illustrates an episode of -NEUROSYPHILITIC origin. Such a person might well be regarded by the lay -newspaper reader as a crank or a grafter but the neurosyphilitic -possibility should always be entertained in cases of this order. - -As against the social difficulties that look in the direction of the -classical paretic grandeur, we present a case of apparent _suicidal -attempt_ by gas, which attempt was followed by a period of amnesia that, -taking into account the laboratory findings, was probably -NEUROSYPHILITIC.[111] - -Vistas of extraordinary interest are opened out by studies of the -relation of neurosyphilis to _delinquency_. The case of the psychopathic -reformer (Case 83) above mentioned was one in which the delinquency may -possibly have been related to acquired syphilis. We present also a case -of juvenile neurosyphilis, a young man of reform school type[112] in -which JUVENILE PARETIC NEUROSYPHILIS was established. This patient, in -fact, deteriorated very rapidly to a condition of considerable dementia -a few months after the diagnosis was established. - -A striking case of so-called DEFECTIVE DELINQUENCY is presented, an -alcoholic prostitute of the reformatory group.[113] The NEUROSYPHILIS in -this case was a complication rather than an original factor in the -delinquency. - -One case of PARESIS SINE PARESI was that of an habitual criminal[114] -and forger who, without showing mental or physical symptoms of -neurosyphilis, yielded the laboratory signs of paretic neurosyphilis. -Again, as in the case of the prostitute just mentioned, the -CRIMINALITY[115] seems to have antedated the neurosyphilis and even to -have been hereditary. - -By way of introducing the next group of Industrial Accident Board cases, -we present a case of JUVENILE PARESIS with initial TRAUM. - -The Industrial Board group is of note in that the signs of the traumatic -form[116] of paretic neurosyphilis do not occur immediately upon the -accident. Some time elapses in which the physical, chemical or -parasitological changes have time to work themselves out in the injured -tissues. Many hypotheses may be raised as to the reason why a trauma -lights up a syphilitic process. Of course, =false claims=[117] =may be -made for compensation by neurosyphilitics= in whom the symptoms were -already in existence before the accident and in whom they may not even -be markedly exacerbated by the accident. The false claimants can -probably not readily frame a story which the expert psychiatrist cannot -discredit if he is allowed to perform laboratory tests and give the -patient the benefit of thorough examination. However, some cases of -established PARETIC NEUROSYPHILIS are perhaps truly subject to -_exacerbations_[118] of the clinical process and it may well be held -that such exacerbations warrant partial compensation. - -The fact that a trauma may light up a syphilitic process is illustrated -in a case that came to the Psychopathic Hospital, in which a SYPHILITIC -LESION developed in the skull AT THE SITE OF SKULL INJURY.[119] - -A case of OCCUPATION-NEUROSIS[120] that might be interpreted as a -_syphilitic neuritis_ is presented. The case is still in doubt as to its -scientific evaluation. - -The workmen’s compensation group of syphilitic cases is of extraordinary -general interest since it indicates that employers may well be on the -lookout not to employ known syphilitics unless fortified by special -insurance arrangements. Whether in future employers may desire =to -employ only W. R. negative workmen= is one of the highly complicated -questions _re_ workmen’s compensation and health insurance. - -But the problems of neurosyphilis are not merely medicolegal and broadly -public or social. The most appealing difficulties lodge within the bosom -of the family. Now and then a case of INCOMPATIBILITY OF TEMPERAMENT, -perhaps complicated by _alcoholism_, occurs which tests prove to be -NEUROSYPHILITIC.[121] - -Special attention should be drawn to a certain NEUROSYPHILITIC -FAMILY[122] in which both parents and five children showed a variety of -syphilitic diseases, including syphilis without apparent neural -complications, paretic neurosyphilis, juvenile paresis, aortic aneurysm, -achondroplasia and caries of the spine, and an as yet indefinite -neurosis. There was a sixth child that died shortly after birth, as well -as three stillborn. - -One =cannot conclude= from the normal[123] look of a neurosyphilitic’s -family =that the normal-looking members are not syphilitic=, as -illustrated by the family of our draughtsman. - -The most =intricate social complications= may arise. We present a case -of a syphilitic man (a well-to-do merchant) who was apparently being -goaded into a second marriage[124] because he was continually being -charged with having caused his first wife’s death. This he had actually -done in a certain sense because his wife had died of general paresis, -having contracted syphilis from him. - - -In the fifth section on THERAPY, we have attempted to outline some of -the principles and problems that arise in the treatment of -neurosyphilis. Enough has probably been said concerning the attitude of -optimism or pessimistic nihilism that may be adopted toward the whole -subject. It must be borne in mind, however, that a great deal of the -work on treatment of neurosyphilis is still in the experimental stage. -As a rule, each case must be considered separately and individually and -the prognosis can be made satisfactorily only after treatment has been -given. This section contains a group of cases that have been treated -rather intensively and the results of this treatment are indicated. The -section is introduced by _five untreated cases_, the brains and cords of -which have been studied post mortem. These illustrate the pathological -conditions which we have to meet, and from these examples we can draw -the theoretical conclusion that some cases are beyond the aid of therapy -on account of the brain destruction. Others, in which the symptomatology -bespeaks just as grave a situation, turn out on autopsy to have very -little actual damage to the brain tissues and therefore should -theoretically at any rate be amenable to antisyphilitic therapy. - -In order to get any adequate conception of the possibilities of -therapeutic results in cases of neurosyphilis, one must consider the -pathological changes that occur and how far these changes are reparable. -In cases in which the destruction of tissue is marked, it is, of course, -out of the question to expect to get any marked clinical improvement. A -case of spastic hemiplegia[125] in paretic neurosyphilis is given with -the autopsy findings as an illustration of irreparable damage that may -occur to the parenchymatous structure, thus precluding any chance of -functional recovery. - -On the other hand, there is a group of cases in which the symptoms may -be exceedingly severe and yet the actual destruction of tissue be almost -nil. This point is illustrated by a case[126] in which _total duration -of symptoms_ terminating in death was _only 22 days_. At autopsy there -was very little in the way of macroscopical lesions, and microscopically -there was no marked evidence of destruction in the parenchymatous -tissue. The lesions were represented chiefly by perivascular -infiltration. According to all our modern ideas, this type of reaction -is resolvable under antisyphilitic treatment. Though this case was one -of very short duration, similar pathological pictures may be obtained in -cases of considerably longer standing. It is also of great importance to -remember that symptomatically such a case may be in no way distinguished -from a case with marked atrophy. - -Another autopsied case is given which shows an exceedingly =marked -meningitis=.[127] The meningitic processes according to the literature -and experience react very readily to antisyphilitic treatment in the -form either of mercury and iodid or in combination with salvarsan. The -lesion here present would probably have improved had intensive treatment -been given. Clinically the diagnosis of general paresis was made and, as -has been the rule in the past, treatment was not given on the ground -that it had no value in paresis. While this is an extreme case of -meningitis, it is to be remembered that the vast majority of cases of -paretic neurosyphilis show some degree of meningitis. Just as in the -marked meningitis of the diffuse neurosyphilis, so with the meningitis -of the paretic form, improvement is expected under treatment. As a part -or even the whole of the symptomatology in a given case may be due to -this meningitic process, we have reason occasionally to expect marked -improvement as the result of antisyphilitic treatment. - -As a contrast to this case with marked meningitis, another case of -=marked atrophy=[128] is given. Here the atrophy was very perceptible on -macroscopical examination and the mere view of the brain at once -indicated that in such a case important results from treatment were not -to be expected. - -The =topographical variation= of the lesions in neurosyphilis must be -remembered when treatment is to be instituted. Thus very marked lesions -may exist in portions of the brain which do not give any very definite -localizing symptoms. As a result, one may be led to believe from -clinical evidence that the case is a very mild one though the lesions -may really be very extensive. The topographical distribution must, -therefore, be taken into consideration in trying to estimate the damage -done. This point of topographical distribution of the lesions is -illustrated by a case.[129] - -It has been generally recognized that =clinical improvement=, if not -cure, may be =readily obtained in the group of diffuse neurosyphilis=, -i.e., so-called cerebral and cerebrospinal forms of syphilis. These are -cases in which the parenchyma is very slightly, if at all, affected and -in which the lesion is chiefly in the meninges and blood vessels, -irritative rather than degenerative. A case[130] is given to illustrate -this point. In our experience systematic intravenous salvarsan therapy -associated with mercury and iodid gives remarkably good results in the -vast majority of this group of cases. - -It is generally conceded that antisyphilitic treatment, particularly -salvarsan, has a very satisfactory result applied to diffuse -neurosyphilis. But the same good results may be obtained in cases which -are not so typically of the diffuse type. An illustration is given in -the case of a machinist in which the diagnosis was in doubt between -paretic, tabetic or diffuse neurosyphilis.[131] The result of treatment -was as satisfactory as could be expected in any type of neurosyphilis -and this in a case of several years’ duration with Argyll-Robertson -pupils. - -As a rule, the Argyll-Robertson pupil is taken as a grave omen for -treatment, an idea based upon a conception that the Argyll-Robertson -pupil so frequently represents the old so-called “parasyphilitic” cases, -which, in the past were taught as being incapable of improvement by the -ordinary antisyphilitic methods. - -A second case[132] with Argyll-Robertson pupil shows again that the -=prognosis may be very good despite the Argyll-Robertson sign=. - -But even in the diffuse neurosyphilis, the symptomatic results of -treatment may not be entirely happy. Under treatment it may be possible -to reduce the spinal fluid tests to negative without, however, as in the -case of our hemiplegic lady,[133] making the physical or mental symptoms -disappear. In other words, it may be possible to stop the active -progress of the disease without removing the symptoms. - -One is always warned of the danger of intravenous salvarsan therapy in -hemiplegic cases due to arteriosclerotic conditions. While this warning -is well justified, it does not mean that the most intensive treatment is -contraindicated, as shown in the case of our hemiplegic machinist.[134] -Such may be given over long periods of time with the most satisfactory -results. - -A case[135] is given which illustrates the value of antisyphilitic -treatment in cases showing symptoms of intracranial pressure due to -syphilitic disease. In the case of the woman which we cite, we believe -that the symptoms of intracranial pressure were probably due to a -gummatous new growth, although it is possible that they were due to a -marked meningitic process. However, the results of a limited amount of -antisyphilitic treatment in this case were very brilliant. Similar -results may often be obtained in gumma of the brain. This is not always -true, however, and it may become necessary to use surgical procedure in -order rapidly to overcome the effects of intracranial pressure. - -While it has always been conceded that treatment would greatly help -cases of diffuse and vascular neurosyphilis, the utmost pessimism has -existed concerning the results to be obtained by treatment in cases of -tabetic and paretic neurosyphilis. Only in the last five or six years, -due to the stimulus of Ehrlich’s discovery of salvarsan and the -introduction of the intraspinous methods of therapy, have intensive work -and study been given to the treatment of these cases. And though it has -been by no means settled in the minds of the various workers in this -field, as to what the ultimate results of such treatment will be and -though some do not believe that there is any good to be expected from -our present methods, still the majority of men who are treating these -cases systematically feel very much encouraged. - -=At times very brilliant results= are to be obtained by intraspinous -treatment =in tabetic neurosyphilis= (“tabes dorsalis”). A very striking -illustration is given of a case of this sort in which the symptoms dated -only a few months but which had all the classical symptoms, signs and -laboratory tests. Five intraspinous injections of mercurialized serum -were sufficient to cause the disappearance of the subjective symptoms -and to reduce the spinal fluid test to negative.[136] - -It must be emphasized that the best results in cases of tabetic -neurosyphilis are usually to be expected in cases in which the symptoms -are of short standing. Where the process is of long duration and much -destruction of spinal cord tissue has occurred, the best one can expect -is that the activity and progress may be halted. This is illustrated by -our case of a baker, 43 years of age, who had been suffering from the -symptoms of tabes for some years. Under treatment it was possible to get -an entirely negative serology of the blood and spinal fluid.[137] -Despite this evidence that the activity of syphilis had ceased, the -symptoms continued unabated. We are ready to believe, however, that much -good was accomplished. For the patient should not have any further -untoward developments or the appearance of any new symptoms. These, -without such treatment, might well be expected. At times excellent -clinical results are obtained in long-standing cases. - -The results of treatment in paretic neurosyphilis (“general paresis”) -have been considered even less hopeful than in tabetic neurosyphilis -(“tabes dorsalis”); indeed, it has often been stated that the patients -are made worse by treatment. Recent work, however, supports a much more -optimistic viewpoint. We feel that =intensive treatment has been of the -greatest value in a number of cases of paretic neurosyphilis=. Two cases -are given which show the most satisfactory and brilliant results of -intensive intravenous salvarsan therapy in cases diagnosed as general -paresis. The first case, an excellent salesman, 46 years of age, with -most aggravated mental symptoms, recovered symptomatically and all his -tests were rendered negative.[138] He has now remained entirely well and -economically efficient for about two years without further treatment. -The other case,[139] a housewife, also with very marked symptoms -suggestive in all ways of general paresis, also recovered rapidly under -treatment and her tests became negative. Her remission has now lasted -for nearly three years without further treatment. - -At times it is not possible to get the spinal fluid tests to become -negative in cases of paretic neurosyphilis under the most intensive -salvarsan therapy. In spite of this, the clinical condition of the -patient may improve so greatly that the patient can be considered -=clinically recovered=. An illustration is given of an undertaker[140] -who was brought from a condition of the greatest cachexia and mental -confusion to a condition of robust appearance and mental efficiency -under intravenous salvarsan therapy, in spite of the fact that his tests -were very slightly if at all reduced in intensity. He has been able to -resume his former occupation and his former life with great satisfaction -to himself and his family. - -Improvement in paretic neurosyphilis under treatment is not to be -expected very early. =Two or three months of active treatment= may -elapse before one sees signs of improvement. Indeed, as illustrated by -our case of the shipping clerk, this improvement may begin to make its -appearance only after more than four months of intensive treatment -consisting of two injections of salvarsan per week.[141] In spite of the -long delay in this case, complete clinical recovery occurred and the -tests became almost negative at the end of a year of treatment. - -It is not only in the central nervous system that the syphilitic process -may resist the most intensive treatment. In the case of the speculator, -a victim of paretic neurosyphilis, which we cite, a perennially -recurrent iritis appeared after several months of the most intensive -salvarsan treatment which was apparently sufficient to reduce the -symptoms of the paretic neurosyphilis,[142] but not of non-neural -syphilis. - -We give the case of a charwoman having the diagnosis of paretic -neurosyphilis, who, under intensive treatment, made a symptomatic -recovery. The interesting point in her findings is that all the tests in -the spinal fluid became negative except the gold sol reaction which -remained of the “paretic” type.[143] There is no general rule as to the -reaction of the spinal fluid tests under treatment. At times one test is -the first to disappear under treatment; again it is another. We have -seen many cases in which the gold sol was the first test to become -negative and others, as the case given, in which it is the last to show -any change. As in our undertaker, symptomatic clinical improvement may -be practically complete without any change in the spinal fluid tests. - -One must remember that it is the condition of the patient that is of -first importance; not so much the laboratory tests. Having shown the -clinical recoveries with the tests remaining positive, we now have to -report two cases in which there was =improvement= as shown =by the tests -but no clinical improvement=. The first patient, a bank teller[144] of -39 years, with a diagnosis of paretic neurosyphilis, received intensive -intravenous salvarsan for several months. Under this treatment all the -tests became negative except the gold sol which remained of the paretic -type. In spite of this, there was not the slightest improvement in his -mental condition. - -The second case, a young man of 29 years in whom the symptoms of -neurosyphilis had recently appeared, under treatment showed a marked -diminution in the intensity of the spinal fluid tests, notwithstanding -which the patient became more and more demented and died after a series -of convulsions.[145] - -Of course, good results indicated above in some of our cases of paretic -neurosyphilis are not to be expected in every case no matter how -intensive the treatment. We give a case of paretic neurosyphilis in -which the most intensive intravenous salvarsan therapy gave no -satisfactory results. This was followed by several intraventricular -injections of salvarsanized serum. The results of this combined -treatment, however, were still not satisfactory, and the patient -died.[146] - -In order to emphasize as strongly as possible what we believe is a great -=advantage of systematic intensive treatment= for neurosyphilis, we -offer two cases in different time periods of neurosyphilis. The first is -a printer with the symptoms of diffuse neurosyphilis six months after -the appearance of his chancre.[147] These symptoms appeared despite -three injections of salvarsan, injections of mercury and mercury by -mouth. Under intensive treatment (meaning injections of salvarsan twice -a week and continued injections of mercury), complete recovery occurred -in a few weeks. - -The second case is that of a waiter with signs and symptoms of -neurosyphilis in whom the diagnosis lay between the diffuse and paretic -forms.[148] This patient developed his symptoms in spite of continuous -antisyphilitic treatment during the six years since his infection. This -treatment had been comparatively mild, consisting in great part of -mercury by mouth. However, he had had courses of injections of mercury -and several injections of salvarsan. Under a systematic course of -intravenous injections of salvarsan twice a week for a number of months, -all symptoms disappeared and the spinal fluid tests became negative as -well as the W. R. in the blood serum. - -A final case is offered which indicates that antisyphilitic treatment -may occasionally be of service in improving the mentality of a -FEEBLEMINDED CONGENITAL SYPHILITIC.[149] - -No attempt has been made in this section to give a per cent evaluation -of the results of treatment in any one group of neurosyphilis. Two -charts (charts 25 and 26), however, are appended which give an -indication of some of our results. It seems to us, however, that it is -too early to make any definite statements as to how far treatment will -take us in the groups of neurosyphilis. We do feel decidedly, however, -that many patients, in whatever group of neurosyphilis the diagnosis may -place them, will respond to intensive systematic antisyphilitic -treatment. =It is unfair to give an entirely grave prognosis in any case -of neurosyphilis until the effect of treatment has been tried.= - - -In a separate section, entitled NEUROSYPHILIS AND THE WAR, we have -presented fourteen cases selected from British, French and German -writers in the war literature of 1914–16. Most of these cases were -naturally somewhat inadequately reported under the critical conditions -of literature made in the war. We present the cases for what they are -worth: at all events they draw attention to the extraordinary interest -of the neurosyphilis problem in relation to the war. - -Such cases as A, one of tabes dorsalis apparently developing paresis by -a process akin to shell-shock, is of value in the interpretation of the -development of paresis in civil life. By “shell-shock” we commonly refer -to a condition in which there is no actual traumatic injury of the -brain. The hypothesis must be then that the explosion in some way -indirectly caused an alteration of living conditions of the spirochetes, -permitting the development of paresis. - -Case B similarly seems to be a case in which a latent syphilis has -turned shell-shock into tabes dorsalis. - -Cases C, D, E bring up the question of aggravation of neurosyphilis _by_ -service and _on_ service, respectively. - -Case F likewise shows how, in the determination of amount of pension, -the probable duration of the neurosyphilitic process is important. - -Case G seems to show that war stress alone, without the emotional or -physical effects of shell-shock, may kindle a latent syphilis into -paretic neurosyphilis. - -Case H similarly suggests that the “gassing” process may effect the same -result. - -Case I seems to show that the neuropathically tainted person may have -latent epilepsy brought out through syphilis, the syphilis in this case -having been acquired during the first summer of the war. - -Case J was an interesting case of a syphilitic who, after the stress of -the Battle of Dixmude, became an epileptic. - -Syphilitic root-sciatica was developed in Case K at work in the war -zone. - -Case L is one of a civilian who apparently would not have developed -paresis at precisely the moment when he did, if he had not been -discharged as a German Jew from his long-held bank position in London. - -Two cases, M and N, are cases of shell-shock, non-syphilitic; yet the -picture of paresis in the one case and of tabes in the other was for a -long time almost convincing to the examiners. They are better termed -cases of pseudoparesis and pseudotabes, using the prefix “pseudo”, as -usual, to signify a non-syphilitic imitation of the disease in question. - -To sum up in the most general way the lessons of this book, we may -emphasize again (1) _the unity-in-variety of the phenomena of -neurosyphilis_, (2) _the value of a hopeful approach to the therapy of -all cases of neurosyphilis_, _even the paretic form_, and (3) _the value -of applying syphilis tests to every case of neurosis or psychosis_. - -(1) RE _unity-in-variety of neurosyphilitic phenomena_. - -The unity of these phenomena is confirmed, theoretically, by the common -factor of spirochetosis: practically, by the Wassermann reaction, -positive in serum or spinal fluid! Almost at this point the unity of -phenomena ceases. Neither chronicity, nor evidence of mononuclear cell -deposits, nor evidence of serious structural damage to the nervous -system, nor presence of other positive tests than the W. R.,[150] nor -existence of mental or nervous symptoms or signs, is a common feature of -neurosyphilis. Sometimes the nervous system appears to harbor -spirochetes in the most cordial manner as guest-friends (_paresis sine -paresi_.) Again, perhaps as an expression of elaborate processes of -immunity, the spirochetes take effect in relatively huge gummata. -Sometimes the neurosyphilitic process rises as if by a regular process -of siege from spinal nerve-root to spinal nerve-root (tabes dorsalis and -diffuse neurosyphilis). Again, the nervous system is taken by storm, as -it were (disseminated encephalitis). Very frequently the neurosyphilis -is simply an indirect effect of blood-vessel disease, and huge masses of -tissue are scooped out in necrosis with dependent secondary -degenerations; and later the extinct lesions of vascular origin may or -may not betray evidence of their syphilitic origin. Sometimes diffuse -processes run on, apparently, with perfect fatalism to a mortal issue in -a few years both with and without treatment. Again treatment appears to -accomplish much (see fuller discussion under 2). The laws governing the -preference of processes to lodge in membranes, vessels, and parenchyma, -and in all combinations of these, have not been worked out. Hardly a -case of neurosyphilis, properly studied ante mortem and post mortem, but -would throw important light on our medical approach to one of the great -problems of civilization, the problem of syphilis as a whole. - -(2) RE _value of a hopeful approach to the therapy of neurosyphilis_. - -The prognosis of neurosyphilis is not worse than that of the chronic -diseases in general. In fact, the prognosis of neurosyphilis _quoad -vitam_ is either good or dubious, certainly not bad. The surprising -reversals of form which the spirochete shows in certain remissions are -always to be awaited. Treatment of neurosyphilis has certainly effected -amazing results, not so much by way of Ehrlich’s _therapia sterilisans -magna_ as by means of systematic intensive treatment. Even paretic -neurosyphilis (general paresis) seems to have been cured. Preparetic -phases are theoretically hopeful. Nor is it so certain that paretic -neurosyphilis will ultimately prove a perfectly distinct species of -neurosyphilis. General paresis seems to us at least to be more closely -related to diffuse neurosyphilis than is tabes dorsalis to diffuse -neurosyphilis. In any particular case, moreover, =during a good part of -the early months or years=, =it is difficult or impossible to tell the -paretic from the non-paretic forms of diffuse neurosyphilis by any -combination of clinical observations and tests=. In the instance of more -protracted neurosyphilis, e.g., tabetic, the outlook for vascular -complications is such that antisyphilitic treatment directed at -prevention of these complications is scientifically warrantable, even if -the tabetic process itself proves unassailable. The old distinction of -syphilis and parasyphilis, so striking and apparently satisfactory when -introduced by Fournier, seems to be a false distinction which should be -dropped. Therapeutically, we should approach all cases of neurosyphilis -without bias or nihilistic prejudgments. - -(3) RE _universal applicability of syphilis tests in nervous and mental -cases_. - -The importance of putting every neurosis or psychosis through syphilis -tests is not based alone on the frequency of neurosyphilis, though -neurosyphilis is surely frequent enough. The importance of universally -applying these tests is established by the experience of lingering -doubts both in the physician’s mind and (nowadays increasingly) in the -patient’s and friends’ minds, so long as these tests are not applied. -Nor should the positive serum Wassermann reaction fail to be followed by -lumbar puncture and appropriate tests. The general practitioner -confronting neuroses or psychoses—and what practitioner does not?—must -not expect valuable results from consultation with neurologists and -psychiatrists when he does not carry to these specialists the results of -at least the serum W. R. in his patient. Not only are practitioners, -specialists, and patients subject to discomfiture on the eventual and -delayed proof of syphilis or neurosyphilis, but valuable time has been -lost to treatment. How often the physician of yore (and really not so -long since) had to be regarded as an eccentric virtuoso if he tested -urine as routine! Well, for routine use in nervous and mental diseases, -the Wassermann serum reaction is at least as important as urinalysis. -Nor would we cease our homily with the general practitioner. We know -neurologists and psychiatrists who use the Wassermann test _only when it -is likely to be positive_! But they are dying out. - - - - - APPENDIX A - - -In appendix A a brief outline is given of the six tests (W. R. on blood -serum and spinal fluid, cell count, globulin test, albumin test, gold -sol test). This is not intended as a complete working manual but rather -as indicating the methods used in diagnosis in the cases presented -herein. For more complete details the reader may be referred to -textbooks on the subject of serology, among which may be mentioned -Kaplan: “Serology of the Nervous System”; Plaut, Rehm and Schottmüller: -“Leitfaden zur Untersuchungen der Zerebrospinalflüssigkeit”; Kolmer: -“Infection, Immunity and Specific Therapy,” and, for the Wassermann -technique, an article by Dr. W. A. Hinton in M. J. Rosenau’s “Preventive -Medicine and Hygiene.” - -Our own W. R’s. have been performed at the Wassermann laboratory of the -Massachusetts State Board of Health (formerly the Neuropathological -Testing Laboratory, Harvard Medical School), under the supervision of -Dr. W. A. Hinton. The other tests are performed at the Psychopathic -Hospital. It is very important that a close relationship should exist -between the clinician and the Wassermann laboratory if the most is to be -obtained from the reactions. This relationship has been effectively -close between the authors and the above-mentioned laboratory; and has -enabled us to get very much clearer ideas about certain cases than could -otherwise have been obtained. - -=Cell Count.= In order to obtain the number of cells per cmm., the -examination should be made of the fresh fluid as soon as possible after -this is withdrawn. The most convenient counting chamber for this purpose -is the so-called Fuchs-Rosenthal counting chamber, the ruled spaces of -which contain slightly over 3 cmm. (an ordinary blood cell counting -chamber may be used). According to the method used by us the cells are -stained in a pipette with Unna’s polychrome methylene blue. Using a -white-counting pipette, stain is drawn up to the first or second marking -and the remainder of the pipette filled with spinal fluid. This makes no -change in the dilution for practical purposes. After two or three -minutes the staining is satisfactory and the counting may be done. With -this stain a differential count may be made. Plasma cells stain a -lavender as contrasted to the blue of the lymphocytes. The -characteristic halo surrounding the eccentric nucleus is visible. The -blood cells do not assume color with this stain; hence it is unnecessary -to add any acetic acid. - -For permanent preparations, and more accurate differential counts of the -spinal fluid, the Alzheimer method may be used. The technique is given -in a paper by H. A. Cotton and J. B. Ayer as follows:[151] - -1. Lumbar puncture in the usual manner. - -2. 96% alcohol, in proportion to twice the amount of cerebrospinal -fluid, is added drop by drop and well mixed. - -3. Centrifuge the mixture for one hour at high speed in a glass tube -with conical end. (An ordinary electric urinary centrifuge apparatus can -be employed, the tube to be well stoppered to prevent evaporation.) - -4. The supernatant fluid is poured off, leaving a small coagulum in the -bottom of the tube. - -5. Add absolute alcohol—alcohol and ether—ether, each separately for one -hour, to dehydrate and harden coagulum. - -6. The coagulum can now be gently loosened from the bottom of the tube -by a long needle. The tube is then inverted, and the coagulum allowed to -fall into the hand by a quick tap on the end of the tube. Care must be -taken not to squeeze or handle the coagulum. The hand is placed over a -small homeopathic vial, containing thin celloidin, and the coagulum -allowed to drop into the celloidin, where it remains over night (twelve -hours usually). - -7. Coagulum is placed in thick celloidin which is allowed to evaporate -slowly. It is then mounted on blocks and sections cut 14µ in thickness. - -8. The sections are stained and mounted according to the following -procedure: - -(_a_) Remove celloidin by absolute alcohol and ether. - -(_b_) 80% alcohol. - -(_c_) Water. - -(_d_) Sections are carried on glass or platinum needle into a dish of -Pappenheim’s pyronin-methyl green stain and kept in a water bath at 40° -C. five to seven minutes. - -(_e_) Quickly cool dish in running water. - -(_f_) Wash off superfluous stain in plain water. - -(_g_) Absolute alcohol to differentiate—until no more stain comes away -from section. - -(_h_) Clear in Bergamot oil. - -(_i_) Mount in balsam. - - -The normal cell count may be stated as being up to 6 cells per cmm.; -from 6 to 12 cells may be considered as suggestive of pathological -condition and more than 12 cells per cmm. as definitely pathological. -The type of cell in syphilitic diseases is preponderantly the small -lymphocyte. A low percentage, that is, very rarely over 20%, of large -lymphocytes, endothelial phagocytic cells, polymorphonuclear leucocytes -and plasma cells may also be found. The finding of plasma cells in any -number in the spinal fluid is suggestive although not conclusive -evidence for the diagnosis of paretic neurosyphilis. - -=Globulin= is an albumin which is precipitated by half saturation with a -salt. A very simple and satisfactory test is known as the Nonne-Appelt -test, which has been modified by Ross-Jones. Into a test tube of small -diameter, run 1 cc. of spinal fluid. Place under this fluid with a -pipette, 1 cc. of a saturated solution of ammonium sulphate -((NH_{4})_{2}SO_{4}). If any globulin is present a white, -sharply-defined ring will form at the junction of the two fluids. -According to our readings, a ring that is just visible with the aid of a -black background is called 1+, a ring that is just visible without the -black background, 2+; a ring easily perceptible, 3+ and a relatively -very heavy ring, 4+. On shaking the tube, if globulin is present, the -fluid will show turbescence. - -Another simple globulin test used in our laboratory as a check on the -Nonne-Appelt test is the Pandy test. A few cc. of a clarified 10% -solution of phenol are placed in a watch glass. One drop of spinal fluid -is run into this solution. A milky turbescence indicates globulin. - -The presence of globulin in the spinal fluid is always an indication of -abnormality of the cerebrospinal axis. There is nothing differential in -this finding as it occurs in all inflammatory processes. However, it is -characteristically present in most cases of neurosyphilis (exception to -the rule: the pure vascular type does not show globulin in a very high -per cent). - -=Albumin Test.= Albumin in small quantities is present in all spinal -fluids. Increase over the normal amount occurs in pathological -conditions such as most cases of neurosyphilis, especially in those in -which globulin is found. Any albumin precipitant may be used for rough -clinical calculation, comparing the amount of precipitate with that from -the normal fluid. Our method is to place 1 cc. of spinal fluid in a -small test tube of about 5 mm. diameter and to precipitate the albumin -by the addition of 3 drops of 33⅓% of trichloracetic acid. This test has -its chief value as confirmatory of the globulin test, since in the vast -majority of instances where globulin is found there will also be found -an increase in albumin. - -The =Gold Sol Reaction= is an empirical test discovered by Carl Lange in -the utilization of the work of Zsigmondi with solutions of colloidal -gold and albumins. Briefly the details of the test are as follows: - -Ten tubes are set up in a rack. To the first tube 1.8 cc. of a 0.4% of -salt solution is added and to each of the following tubes 1 cc. of this -solution. Then to the first tube containing 1.8 cc. of salt solution one -adds 0.2 cc. of the spinal fluid to be tested. This gives a dilution of -1 to 10. From this tube 1 cc. is pipetted into the second tube and this -process continued through the ten tubes. This gives dilutions of spinal -fluid of 1 to 10, 1 to 20, 1 to 40, etc., to 1 to 5120 in the last tube. -Then 5 cc. of colloidal gold solution is added to each tube. A positive -reaction is indicated by the precipitation or throwing down of the -colloidal gold into its metallic form. This produces a change in color. -This precipitation may be partial or complete and the amount of -precipitation is indicated by the color and is read as follows: - -The unchanged fluid is called 0; a slight change giving a red-blue as 1; -a further change giving a blue-red as 2; a straight blue as 3; a -lavender or violet as 4; and the colorless fluid representing complete -precipitation as 5. The numbers are placed in a row, indicating the tube -in which the color occurs. The fluid from a case of paretic -neurosyphilis will give a complete precipitation beginning in the first -tube and running through a number of tubes and then grading off. It may -be indicated 5 5 5 5 4 3 1 0 0 0. The characteristic reaction of fluids -from tabetic and diffuse neurosyphilis is less strong than from the -paretic. The greater part of the reaction will take place, however, in -the first five tubes, but as a rule it will not begin very strongly in -the first two. A characteristic reaction is 1 2 3 3 2 1 0 0 0 0. Another -reaction that may be considered characteristic of the tabetic or diffuse -form is 3 3 3 2 1 0 0 0 0 0. Fluids from non-syphilitic cases as a rule -give a reaction having its greatest intensity beyond the fifth tube, -that is, in the high dilutions. - -A reaction characteristic of brain tumor or tuberculous meningitis is 0 -0 0 0 1 3 3 2 1 0. - -The conclusions that may be drawn from the gold sol reaction have been -summarized by one of the authors as follows: - -1. Fluids from cases of general paresis will give a strong and fairly -characteristic reaction, especially if more than one sample is tested, -in the vast majority of cases. - -2. Very rarely a general paresis fluid will give a reaction weaker than -the characteristic one. - -3. Fluids from cases of syphilitic involvement of the central nervous -system other than general paresis often give a weaker reaction than the -paretic, but in a fairly high percentage of cases give the same reaction -as the paretics. - -4. Non-syphilitic cases may give the same reaction as the paretics; -these cases are usually chronic inflammatory conditions of the central -nervous system. - -5. When a syphilitic fluid does not give the strong “paretic reaction,” -it is good presumptive evidence that the case is not general paresis; -and this test offers a very valuable differential diagnostic aid between -general paresis, tabes and cerebrospinal syphilis. - -6. The term “syphilitic zone” is a misnomer, as non-syphilitic as well -as syphilitic cases give reactions in this zone; but no fluid of a case -with syphilitic central nervous system disease has given a reaction out -of this zone (test thus valuable negatively). Any fluid giving a -reaction outside of this zone may be considered non-syphilitic. - -7. Light reactions may occur without any evident significance, while a -reaction of no greater strength may mean marked inflammatory reaction. - -8. Tuberculous meningitis, brain tumor and purulent meningitis fluids -characteristically, though not invariably, give reactions in higher -dilutions than syphilitic fluids. - -9. The unsupplemented gold sol test is insufficient evidence on which to -make any diagnosis, but used in conjunction with the Wassermann -reaction, chemical and cytological examinations, it offers much -information looking toward the differential diagnosis of general -paresis, cerebrospinal syphilis, tabes dorsalis, brain tumor, -tuberculous meningitis, purulent meningitis. - -10. We believe that no cerebrospinal fluid examination is complete for -clinical purposes without the gold sol test. - - -The =Wassermann reaction= as carried out in the Wassermann Laboratory is -based on the principles of the original method—the only essential -modification consists in the employment of cholesterinized alcoholic -extracts of human hearts as antigen instead of aqueous extracts of -foetal livers from cases of congenital syphilis. Experience has shown -that properly standardized antigens made from human hearts are much more -sensitive in the detection of true cases of syphilis. - -=Antigens.= Three antigens are used, each being an alcoholic extract of -human heart which is saturated at room temperature with cholesterin. -These antigens differ slightly in their sensitiveness. Before the test -is made each antigen is diluted with 0.85% salt solution in the -proportion of four parts of the cholesterinized antigen extract to -sixteen parts of 0.85% salt solution. The amount to be used, the dosage, -is carefully determined by testing each antigen against a large number -of known positive and known negative specimens of blood. The dosage of -the antigens employed is less than one-half the amount which inhibits -hemolysis when the antigen is incubated for one hour with the hemolytic -system which consists of complement, amboceptor and cells in the proper -proportions. These antigens are designated as A, B, and C. Antigen A is -the most sensitive. B and C are very similar to each other -quantitatively and qualitatively. - -=Specimens to be tested.= The serum which separates from the clot is -withdrawn, centrifugalized if necessary, and then heated at 55 degrees -for thirty minutes. 0.1 cc. of serum is used in the test and 0.2 cc. of -each specimen is used as a control to exclude the presence of -anti-complementary substances. Spinal fluids are tested in two ways. As -a routine 0.5 cc. of the spinal fluid is used in the test and 1.0 cc. is -used in the control; or when especially requested spinal fluids are -titrated by using respectively 1.0, 0.7, 0.5, 0.3, and 0.1 cc. of the -spinal fluid for each test and 1.0 cc. of spinal fluid for the control. -Spinal fluids are not inactivated. - -=Complement.= The complement is obtained from the serum of guinea pig’s -blood. No complement is used when older than eighteen hours. A 10% -solution and 0.85% salt solution is used in the test. The amount used is -twice the minimum quantity necessary to hemolyze the sensitized cells. - -=Sheep’s Corpuscles.= A 5% suspension of sheep’s corpuscles in 0.85% -salt solution is prepared from defibrinated sheep’s blood. The -corpuscles are washed three times and for each washing four to five -times as much 0.85% salt solution is used as the original volume of the -defibrinated blood. - -=Amboceptor.= The amboceptor is prepared by injecting sheep’s corpuscles -into a rabbit. The serum of this rabbit which contains amboceptor is -diluted with 0.85% salt solution so that 0.25 cc. will hemolyze 0.5 cc. -of a 5% suspension of sheep’s corpuscles. In the test twice the quantity -or 0.5 cc. of amboceptor is used. - -=Sensitized Cells.= The sensitized cells consist of equal parts of -washed sheep’s corpuscles and diluted amboceptor. This mixture is -incubated in a water bath at 37° C. for a half hour to effect the -sensitization of the cells. - -=Technique of the Wassermann Test.= One-tenth cubic centimeter of each -inactivated specimen of serum and 0.5 cc. of each uninactivated specimen -of spinal fluid is pipetted into a separate tube. A mixture is freshly -prepared in salt solution, each cubic centimeter of which contains the -proper amount of antigen A (the most sensitive antigen), and two units -of a 10% solution of guinea pig serum (complement). One cubic centimeter -of this mixture is pipetted into each test tube. These tubes are then -incubated for forty minutes in a water bath at 37° C. At the end of this -period, sensitized cells are added, and the tubes are again incubated in -a water bath at 37° C. for one hour. Each specimen which shows any -degree of inhibition of hemolysis is retested in the afternoon. For this -second test antigen A is again used and in addition antigens B and C. A -control is also made for each specimen retested to eliminate any -possibility of the inhibition of hemolysis being due to -anti-complementary substances in the serum or spinal fluid tested. The -technique of the second test differs in no wise from that of the first, -except for the use of a control in each retested specimen and the -employment of three antigens instead of one. The degree of positiveness -is noted for each retested specimen and compared with the degree of -positiveness obtained for the corresponding specimen with the same -antigen-complement-salt solution mixture in the morning’s test. The -specimen is retested on the next day when discrepancies occur between -the morning reading for antigen A and the afternoon reading for antigen -A. From the above description it will be noted that the negative -specimens have but a single test with one antigen only, while the -positive specimens are retested, thus permitting a confirmation of any -positive reaction. In this way attention is focalized on the positive -specimens. - -=Interpretation of Results.= Antigen C (the weakest of the three -antigens) is used entirely for diagnostic purposes and any specimen -showing the slightest degree of inhibition with this antigen and -stronger degrees of inhibition with the other antigens is reported as -positive. The specimens which are strongly or moderately positive with -antigens A and B and negative with antigen C are reported as doubtful. -In testing spinal fluids by the titration method, antigen C is used and -the readings are based upon the degree of inhibition of hemolysis noted. -The intensity of this inhibition is indicated by Arabic numerals: “5” -indicates complete inhibition, while “1” means a faint cloudiness, hence -a weak reaction. Intermediate numbers show relative intensity varying -between complete inhibition “5” (strong positive) and slight inhibition -“1” (weak positive); “—” equals no inhibition (negative). - -Although it is commonly believed that the recent administration of -antisyphilitic treatment will affect the reaction by making it negative, -this is not our experience, and it is, therefore, not necessary that -treatment be withdrawn for a short period before the specimen is -submitted for examination. - -The reaction as carried out in this laboratory has the following -diagnostic significance: =Positive indicates syphilis=, except very -rarely in acute febrile conditions such as malaria and pneumonia. -=Negative does not exclude syphilis.= In obscure conditions a series of -less than three negatives has little diagnostic significance. =Doubtful -suggests syphilis.= It is therefore advisable to submit three or more -specimens in such a case, and interpret a persistently or -predominatingly doubtful reaction as indicative of syphilitic infection. - -=Bruck Test.= A new serum test for syphilis has recently been described -by C. Bruck.[152] Following are recent results in our laboratory with -this test.[153] - -This new test for the diagnosis of syphilis by C. Bruck has aroused much -interest. The scientific standing of Bruck and the simplicity of the -technique led us to overcome our prejudice, that has been the offspring -of the numerous tests that have been offered of late. Bruck states that -since the discovery of the complement fixation test for syphilis by -Wassermann, Neisser and himself in 1906, he has been trying to find a -simple chemical reaction that would take the place of the complicated -technique of the Wassermann reaction. This method, as he has published -it, was worked out and is being used at the front, in the present war, -where complete laboratory equipment is not available. - -Commencing our experiments with a great deal of scepticism, we were much -surprised at the results obtained, which are given below. Whatever may -be the final status of the test in the determination of syphilis, we -feel that there is a great deal of interest in the fact that this simple -chemical reaction does pick out certain differences in the composition -of blood sera and that apparently a large number of syphilitic sera -differ in their chemical composition percentage from the majority of -non-syphilitic sera. - -The technique, while exceedingly simple, offers many chances for errors -and individual variations so that we have thought it well to give -directions and cautions at some length. - -Bruck’s[154] technique is described as follows: “The test is made with -0.5 cc. clear serum in a test tube, to which is added 2 cc. of distilled -water, and the whole shaken. Then, with a precision pipette, 0.3 cc. of -the ac. nitr. purum of the German pharmacopeia is added and the whole -thoroughly shaken and then set aside at room temperature for ten -minutes. Then 16 cc. of distilled water at room temperature is added, -and closing the tube with the finger, it is shaken up and down three -times carefully, not vigorously enough to make it foam. This is repeated -ten minutes later, and the tube is then set aside for half an hour. By -this time the precipitate is entirely dissolved in the tube with the -normal serum, while the syphilitic serum shows a distinct, flocculent -turbidity. In two or three hours, or better still, in twelve hours, the -gelatinous and characteristic precipitate is piled up on the floor of -the test tube.” - -The acid is prepared by diluting the Acidum nitricum of the U. S. P. -(Sp. gr. 1.403) with distilled water until the hydrometer shows the -specific gravity 1.149, which corresponds to the nitric acid of the -German pharmacopeia, but since this requires a special hydrometer, a -simpler method is to make a 25 per cent solution of the Acidum nitricum, -which will give about the proper specific gravity. - -The serum is obtained by allowing 10 cc. of blood to stand at room -temperature for an hour, and then centrifuging. Serum that has stood for -some time may be used as well as the fresh, and even bloody serum does -not seem to confuse the results to any great degree. The serum gives the -same results with or without inactivation. Post mortem blood gave -results as constant as that obtained during life, in the few cases that -we had in this series. But the reaction may be influenced markedly by -the size of the test tubes. We have found that the 13×1.9 cm. is the -most favorable size. - -When one first thinks of this test it appears very simple and probably -somewhat crude as a chemical reaction, but there are certain precautions -that must be observed, and several hundred normal and syphilitic sera -should be tried before the investigator can feel that he has a refined -routine technique. There is the personal equation which must be watched, -for here is probably the greatest source of error, and readily explains -why two different persons get widely varying results with the same sera -if they have done only a few dozen tests. We must take it for granted -that the reaction is a quantitative one, where some positive reactions -may differ only slightly from the normal non-syphilitic, and, -furthermore, any normal serum may be made to give a positive reaction, -and almost any positive serum be made to give a negative by improper -manipulation at some point in the test. There are as many places for -error to creep in as there are steps in the process. Bruck has omitted -many details in his publication, which allow personal variations, and so -we have tried to develop a routine process that will eliminate as many -of these as possible. - -We shall here attempt to explain the methods which we have found most -satisfactory and at the same time indicate the places where error is -likely to occur. The 0.5 cc. of serum is added to 2 cc. of distilled -water, and shaken thoroughly. Now add slowly exactly 0.3 cc. of acid -from a precision pipette, care being taken it does not flow down the -side of the tube. The tube should be shaken gently while the acid is -being added, for this prevents the formation of a flocculent precipitate -in normal serum which is difficult to dissolve later. After the acid is -added shake each tube gently to make sure that these flakes do not -persist. It is difficult to shake each tube in exactly the same manner, -as must be done if we expect uniform results. - -The first 250 tests of this series were made by allowing the tubes to -stand for ten minutes as Bruck advocates. Then we found that practically -all sera gave a positive reaction if allowed to stand 15–20 minutes, and -so in the other tests of the series an attempt was made to make the -reaction more sensitive by allowing the tubes to stand only 6–7 minutes. -During this time the tubes should be shaken gently once or twice. The -manner in which the 16 cc. of water is added also influences the -reaction. If allowed to flow freely in upon the precipitate, the -positive may be forced into solution as well as the negative. Both -pipette and tube should be slanted and the water allowed to flow down -the side of the tube without disturbing the precipitate. If all has gone -well up to this point, we may see a marked difference between the normal -and syphilitic precipitates, in that the normal will begin to go into -solution at once, thus clouding the water, while a positive precipitate -will be composed of large flakes which show little or no tendency to go -into solution or cloud the water above. It must be remembered that the -most flocculent positive precipitate will go into solution if the fluid -is splashed or shaken too hard while the tube is being inverted. If any -doubt as to the character of the precipitate now exists, it may be -allowed to stand ten minutes longer, and again inverted as before, or -even repeated several times during the next hour or two. We see no -reason why the tubes should be left to stand over night, for during this -time a precipitate usually settles in the normal tubes. This, however, -differs from the syphilitic precipitate in that it is still finely -granular and goes back into solution readily when the tubes are -inverted. - -In view of these possible grounds for error, it is only logical to run -controls of known positive and known negative sera along with each group -of unknown bloods, and even then certain tubes will seem doubtful, in -which event the test should be repeated with added precaution to see if -a definite positive or negative reaction may be obtained. - -In the last tests of this series we seemed to aid the reaction by -rendering the serum-water solution alkaline by one or two drops of 10 -per cent potassium hydroxide before the acid was added. The positive -sera have a larger precipitate, while the normal seem to dissolve more -readily. - - TABLE I - - Syphilis: nervous system involved. - - General Paresis Wassermann and Bruck agree positively 47 - Wassermann and Bruck agree negatively 7 - Wassermann and Bruck at variance 10 - - Tabes Dorsalis Wassermann and Bruck agree positively 3 - - Cerebrospinal Wassermann and Bruck agree positively 8 - Wassermann and Bruck agree negatively 3 - - Juvenile Paresis Wassermann and Bruck agree positively 1 - - Summary: Wassermann and Bruck agree positively 59 - Wassermann and Bruck agree negatively 10 - Wassermann and Bruck at variance 10 - - TABLE II - - Syphilis: nervous system not involved. - - Syphilis Wassermann and Bruck agree positively 12 - Wassermann and Bruck at variance 5 - - Congenital Syph. Wassermann and Bruck agree positively 3 - Wassermann and Bruck agree negatively 2 - - Summary: Wassermann and Bruck agree positively 15 - Wassermann and Bruck agree negatively 2 - Wassermann and Bruck at variance 5 - - TABLE III - - Non-syphilitic: Wassermann reaction negative. - Doubtful or positive Bruck 86 - Bruck test negative 216 - - Total for three groups: - Wassermann and Bruck agree positively 74 - Wassermann and Bruck agree negatively 230 - Wassermann and Bruck at variance 101 - -The tests here reported were made on blood sera obtained from patients -admitted to the Psychopathic Hospital and its Out-Patient Department. As -a routine Wassermann test is made on each patient who enters the -hospital, it was only necessary to take another tube of blood from each -patient, and check the results in each instance with the Wassermann -reaction. As it takes several days to get the report from the Wassermann -laboratory of the State Board of Health, there was no chance of being -prejudiced by a previous knowledge of the Wassermann reaction. The cases -for the most part were those of mental disease; the majority in good -general physical health. - -A comparison of the total number with the Wassermann reaction shows that -there was a general agreement of 304 of the 405 cases tested, or a -percentage agreement of practically 75%. In considering the cases of -syphilis of the central nervous system in a group by themselves, we find -that the agreement is closer, since 69 of the 79 cases tested, or 87% -agreed without any question of doubt. It will be noted that in several -cases of general paresis, the Wassermann reaction, which was repeated at -intervals, was negative, and in most of these cases the Bruck test was -negative also. Our few cases of congenital and latent syphilis also -checked very closely with the Wassermann test. In the various groups of -mental cases in this series, no factor of interference was discovered. -It is also of interest that in the cases where the blood was obtained -post mortem, the Bruck test agreed with the Wassermann result obtained -on ante mortem blood serum. Further work on post mortem sera will be -reported. Some of the patients not included in the syphilitic groups -that have a negative Wassermann and no clinical signs of syphilis, give -a history of previous infection at some time, which might partly account -for the variations in the two tests. - -CONCLUSIONS - - 1. We present results of the Bruck sero-chemical test in 405 cases. - In 101 of these cases there were definite clinical manifestations - of syphilis, in which the Wassermann and Bruck tests agreed - positively in 74 or 75%. The two tests agreed negatively in 12 - instances, and were at variance in 15. - - 2. In the group which showed syphilis of the nervous system we had - 64 cases of clinically certain general paresis, of which the - Wassermann and Bruck tests agreed in 54 instances, or practically - 85%. In other forms of central nervous system involvement the - agreement was 100% in the 15 cases tested. - - 3. In the cases with no apparent involvement of the nervous system - the agreement was somewhat less, being 76%. This may be in keeping - with the fact that the Wassermann test was not so strongly - positive in these cases. - - 4. The advantages of the test are: (1) the short time required to do - the test; (2) the limited amount of apparatus necessary, and (3) - the simplicity of the technique. - - 5. The disadvantages of the test seem, for the most part, to be - bound up in the personal variations that are apt to occur. - - 6. We are here dealing, most probably, with a quantitative chemical - difference in the protein content of syphilitic and non-syphilitic - sera, the nature of which is not understood by us. It is our hope - that this may be brought to light in the near future in the field - of chemistry. - - - - - APPENDIX B - COMMON METHODS OF TREATMENT USED IN CASES OF NEUROSYPHILIS - - -The =treatment for neurosyphilis= according to the viewpoint of the -authors =is treatment for syphilis=. It is necessary in order to cure a -case of neurosyphilis to cure the syphilis in the patient. Accordingly, -the methods of treatment best adapted for the cure of syphilis are -indicated in the treatment of neurosyphilis. As experience shows that it -is often more difficult to cure the neurosyphilitic cases, treatment -will have to be pushed with greater intensity than in some non-nervous -system syphilis. In general, then, the methods that have been applied by -the syphilologist will be used in the treatment of cases of -neurosyphilis. In addition, methods attempting to bring the drug into -local contact with the central nervous system have been devised. The -methods of treatment have been in part indicated in Chart 27. - -The method chiefly used in treatment of the cases of this book is what -we have called =intensive systematic intravenous treatment=. The -treatment consists of intravenous injections of salvarsan (or a -substitute for salvarsan, as arsenobenzol and diarsenol) given in a dose -of about 0.6 gram and repeated twice a week over a period of a number of -months. In addition, injections of mercury salicylate averaging 0.065 -gram once a week are given and potassium iodid by mouth. As indicated, -the important point is to keep up treatment for a long period of time. -This method has produced practically no untoward results, certainly no -more untoward results than are to be expected with salvarsan in smaller -quantities and it has seemed to us that the therapeutic results have -been as satisfactory as in any other form of treatment. - -Specialized forms of treatment intended to place the drug in contact -with the central nervous system may be described under the headings of -=spinal intradural treatment= and =cerebral subdural= and -=intraventricular treatment=. - -Three main therapeutic agents have been largely used. These are (1) -salvarsanized serum according to the =method of Swift-Ellis= (=in -vivo=). The serum according to this method is prepared as follows: An -intravenous injection of salvarsan is given to a patient and blood -withdrawn at the end of one-half hour. This is allowed to clot. The -serum is removed and after inactivation at 56° C. for one-half hour it -is ready for use. The average dose is 15 to 30 cc. of serum. As a matter -of fact, it is not necessary to use the blood serum from the same -patient to whom the intraspinous injection is to be given. (2) The -salvarsanized serum according to the =method of Ogilvie= (=in vitro=). -Blood serum is prepared from any patient and to it is added salvarsan in -such a strength that the amount to be injected, 10 to 30 cc. of serum, -will contain 0.0001 to 0.001 gm. (3) Mercurialized serum according to -the =method of Byrnes=. Mercury bichloride is added to blood serum in -such proportion that the amount of serum to be injected will contain -from 0.00065 gram to 0.0026 gram. - -The method of intraspinous injection is to perform lumbar puncture, -withdraw an amount of fluid approximately equivalent to the amount to be -injected; then allow the serum to be injected to run in by gravity. - -For the =cerebral=, =subdural and intraventricular= injections, the same -sera may be used as for the intraspinous. Five or six times as much -salvarsan may be given, but a smaller amount of serum may be advisable, -that is, 10 to 15 cc. To perform injections a trephine opening is made -in the calvarium about the size of a dime. The location of choice for -the opening is slightly back of the longitudinal prominence just to the -right of the median line, to avoid the frontal sinus. For subdural -injections a curved needle is thrust between the dura and the brain and -the serum allowed to flow in slowly by gravity. For the intraventricular -injections a blunted spinal puncture needle is thrust through the brain -substance into the 3rd ventricle. When the 3rd ventricle is reached the -clear cerebral fluid will flow out; then after withdrawing a sufficient -amount, the serum may be introduced by gravity. The trephining may be -done under local anesthesia but as a rule it is better to induce general -anesthesia. The subsequent injections can be made without recourse to -any anesthesia whatsoever, as they are practically painless. - -All procedures both in the injections and in the preparation of sera are -naturally to be performed under aseptic conditions. - - - - - INDEX - - - Abscess, tonsillar, associated with neurosyphilis, 250. - - Addison’s disease in juvenile paretic, 279. - - Agraphia, 101. - - Albumin test, 474. - - Allbutt, Clifford, 257. - - Alcoholism, chronic, 227. - - Alcoholic dementia, 237. - epilepsy, 229. - hallucinosis, 225. - pseudoparesis, 222, 223, 451. - - _Allergie_, 129, 204. - - Alzheimer, 428. - method, 472. - - Amboceptor, 477. - - Amnesia, 195. - - Anaphylaxis, 129. - - Anatomical formulae, 25. - - Antigens, 476. - - Aortic aneurysm, 35, 439. - - —— sclerosis, 41, 46, 135. - - Aphasia, 31, 43, 101, 262, 445. - - Apoplexy, 197. - - Argyll-Robertson pupil, 209, 212, 217, 291, 450. - as isolated symptom, 217. - in alcoholism, 214, 229. - - Arndt, Junius and, 249. - - Arsenobenzol, 375, 377, 389, 486. - - Arteriosclerosis, cerebral, 101. - not a contraindication to intensive salvarsan therapy, 359. - radial, 68. - - Ascending lesion, 23. - - Asymmetrical lesions, 19. - - Ataxia, 31, 223. - - Atheromatous degeneration, 35. - - Atrophy, cerebellar, 39. - cerebral, 47, 134, 205. - parenchymal, 41. - pontine, 39. - - Atypical case congenital neurosyphilis, 270. - - Ayer, J. B., 472. - - - Ballet, 72. - - Barrett, A. M., 54, 175, 187, 212, 218, 219. - - Bechterew, 219. - - Binet and Simon, 304. - - Binet scale, 277. - - Birnbaum, 403. - - Blood pressure, high, 70, 262, 124. - - Bly, 252. - - Bonhoeffer, 404, 415, 417. - - Bordet, 427. - - Bratz, 278. - - Bruck test, 479. - - Bruck, C., 479. - - Bumke, 214. - - - Canavan, 256. - and Southard, 70. - - Cell count, 471. - - Cerebral syphilis, see diffuse neurosyphilis. - - Cerebrospinal syphilis, see diffuse neurosyphilis. - - Cervical hypertrophic meningitis of Charcot, 56, 441. - - Chancre, extragenital, 75, 342. - - Character change, neurosyphilis, 314. - - Charcot, 60, 186. - - Choroiditis, 242. - - Christian, 407. - - Cimbal, 403. - - Civilization and syphilis, 76. - - Clinical evidences of syphilis, 131. - - Clouston, 158. - - Collins, Joseph, 145. - - Compensation in neurosyphilis, 309, 402, 456. - - Complement, 477. - - Conduct disorder, 38. - - Congenital syphilis, absence of stigmata, 318. - as cause of feeblemindedness, 159, 447. - involvement of nervous system in, 274. - - Congenital neurosyphilis, 270, 395. - resembling feeblemindedness, 272. - - Conjugal neurosyphilis, 263. - - Convulsions, 43, 101, 248, 362. - cause of in paretic neurosyphilis, 232. - in psychopathic subject with syphilis, 417. - - Corneal opacity, syphilitic, 234. - - Cotard, 73. - - Cotton, H. A., 472. - - Craig, C. B., 152, 196. - - Cramer, 125. - - Cranial neurosyphilis, 140. - tenderness, 139. - - Crises, gastric, 367. - - Cysts, ependymal, 59. - of softening, 27, 36, 54. - - Cytorrhyctes luis, 381. - - - Dana, Charles L., 65, 77, 78. - - Dazed states, 264. - - Deafness, 63. - - Decompression, 138. - - Defective delinquent—diffuse neurosyphilis, 300, 455. - - Dejerine-Tinel, 61. - - Delinquency and juvenile neurosyphilis, 298. - - Delirium tremens, 332. - - Dementia, 137. - - Dementia paralytica, see paretic neurosyphilis. - - Dementia praecox, 74, 185, 247. - - Depression, 95, 126. - - Depressive drugs, 189. - - Diabetes, and neurosyphilis, 240. - insipidus, 190. - - Diabetic pseudoparesis, 238. - - Diarsenol, 377, 389, 391, 486. - - Differential diagnosis, alcoholism and neurosyphilis, 227, 231, 234, - 236. - brain tumor, diabetic pseudoparesis and neurosyphilis, 238. - diffuse and paretic neurosyphilis, 165, 193, 247. - manic-depressive psychosis and neurosyphilis, 69. - multiple sclerosis and neurosyphilis, 253, 255. - neurasthenia and neurosyphilis, 65, 183. - senile arteriosclerotic psychosis and neurosyphilis, 262. - - Diffuse neurosyphilis, cerebrospinal syphilis, cerebral syphilis, - spinal syphilis, 17, 80, 85, 97, 103, 122, 140, 183, 193, 300, 331, - 342, 359, 433, 439, 443. - premonitory symptoms, 342. - prognosis, 80, 103, 124, 433, 443. - spinal fluid findings in, 348. - symptoms, 99. - treatment, 98, 103, 184, 302, 390. - treatment, results, 343. - - Diplopia, 50, 184, 253, 356. - causes, 140. - - Donath, 401, 403. - - Drastich, 407. - - Duco and Blum, 403. - - Dupré, 407. - - Dysdiadochokinesis, 231. - - - Ehrlich, 184, 428, 429. - - Encephalitis, 27, 248. - disseminated, 218. - - Endarteritis, 220. - - Ependymal cysts, 59. - - Ependymitis, 40, 47, 49, 134. - - Epilepsy, 192. - alcoholic, 229. - brought out by syphilis, 415. - Jacksonian, 103. - parasyphilitic, 194. - relation to juvenile neurosyphilis, 277. - syphilitic, 103, 194. - syphilogenic, 415. - - Epileptic neurosis, 195. - - Erb’s syphilitic spastic paraplegia, 147. - treatment of, 148. - - Euphoria, 73. - - Excited states, 95. - - Exner, M. J., 416. - - Exophthalmic goitre, syphilitic (?), 205. - - Extraocular palsy, 140, 441. - - Eye changes in neurosyphilis, 257. - - Eye muscles, paresis of, 17, 50. - - Facial paralysis, 53. - - Families of neurosyphilitics, 275, 316, 318, 320, 373, 431, 457. - - Family of neurosyphilitic, normal-looking, but syphilitic, 318. - - Familial syphilis, 299, 306. - - Farrar, C. B., 411. - - Fearnsides, Head and, 21, 140, 150, 193, 217, 374, 378. - - Feeblemindedness, 395. - and congenital syphilis, 159. - - Fernald, W. E., 159, 273, 396. - - Fildes, McIntosh and, 129, 329. - - Focal changes, 221. - meningitis, 50. - softenings, pontine, 54. - - Fournier, 142, 222, 186, 194, 381. - - Franz, 357. - - Froissart, 413. - - Fugue, hysterical, 264. - - - Garnier, 407. - - General paresis, see paretic neurosyphilis. - - Glands, 270. - - Gliosis, 39, 47, 49, 136, 180. - - Globulin, 229. - tests, 473. - - Glycosuria, 238, 241. - - Goddard, 397. - - Gold sol reaction, 247, 474. - in brain tumor, 100. - paretic, 85, 98. - paretic, other tests negative, 383, 385. - in purulent meningitis, 100. - syphilitic, 85, 98, 345. - - Graham, Thomas, 429. - - Grandiosity, 72, 295, 455. - - Graves, W. W., 157. - - Grilli, 407. - - Gross, 257. - - Gumma, see gummatous neurosyphilis. - - Gumma of tonsil, 250. - - Gummatous neurosyphilis, 53, 56, 137, 138, 140, 221, 362, 438. - - - Hallucinations, 53. - in paretic neurosyphilis, 249. - - Hauptmann, 348. - - Head and Fearnsides, 21, 140, 150, 193, 210, 217, 374, 387. - - Headache, 53, 63, 122, 247, 352. - causes of, 209. - - Hecht, 399. - - Hemianopsia in neurosyphilis, 242. - - Hemiplegia, 31, 45, 80, 122, 262, 360. - causes of, 389. - - Hemitremor, 197. - - Heredity, neuropathic, 84. - - Herxheimer reaction, 152. - - Heubner, 427, 428. - - Hinton, W. A., 471. - - Huntington’s chorea, 258. - - Hutchinsonian teeth, 45. - - Hydrocephalus, 134, 306. - - Hyperreflexia, explanation of, 233. - - Hypochondriacal ideas, 133. - - Hysteria, 185, 301. - - Hysterical symptoms, 18. - - - Incontinence, vesical in tabetic neurosyphilis, 144. - rectal, 56. - - Incubation period of neurosyphilis, 152. - - Infectiousness of neurosyphilis, 95. - - Insight, 95. - - Insomnia, 63. - - Intracranial pressure, 139, 362. - - Intraspinal lesions, 95. - - Intraspinous therapy, 122, 366, 486. - unpleasant results of, 366. - - Intraventricular injections, 389, 487. - - Involution-melancholia, 187. - - Iodine, untoward results, of, 363. - - Iritis, 17. - - - Järisch-Herxheimer reaction, 72. - - Joffroy, 214. - and Mignot, 64. - - Junius and Arndt, 249. - - Juvenile neurosyphilis, 438, 447. - relation to epilepsy, 277. - - Juvenile paresis, see juvenile paretic neurosyphilis. - - Juvenile paretic neurosyphilis, juvenile paresis, 45, 154, 157, 272, - 275, 298, 306, 440. - age of onset, 158. - and Addison’s disease, 279. - and delinquency, 298. - prognosis, 156, 158, 162, 273, 275. - treatment, 154, 161, 278, 299. - - Juvenile paretic neurosyphilis, with initial trauma, 306. - congenital amputation of toes in, 158. - - Juvenile tabetic neurosyphilis, 161, 447. - - - Kaplan, 255, 471. - - Kéraval, 257. - - Key, 427. - - Knee-jerks, absence of, 223. - lively, 75. - return of, 24. - - Koefod, Solomon and, 243. - - Kolmer, 471. - - Kraepelin, 65, 66, 69, 88, 91, 95, 187, 225, 249. - - Krafft-Ebing, 84. - - - Laignel-Lavastine, 413. - - Lange, C., 428, 429, 474. - - Lancinating pains, 92, 141. - - Lépine, 408, 413. - - Leptomeningitis, 47, 54, 135. - - Lewandowski, 210. - - Liability of paretic, 295. - - Lissauer’s paralysis, 38. - - Locomotor ataxia, see tabetic neurosyphilis. - - Long, 418. - - Lucke, Baldwin, 93, 144. - - Lues maligna, 250, 452. - - Lumbar puncture, untoward effects, 352. - treatment of, 354. - - Lüth, 278. - - Lymphocytosis, 23, 30, 40, 49. - - - McDonagh, 381. - - McIntosh, Fildes and, 129, 329. - - Malaria, cerebral, simulation of paretic neurosyphilis, 245. - - Mallory and Wright, 472. - - Manic-depressive psychosis, 68, 71, 77, 187, 202, 291, 384, 442. - - Marie, Chatelin and Patrikios, 412. - - Marie, 408, 414. - - Martin, E. G., 313. - - Massary, de, 414. - - Mattauschek and Pilcz, 347. - - Medicolegal and Social, 454. - period of paretic neurosyphilis, 414. - - Meilhon, 407. - - Memory, failing, 63. - - Meningitis hypertrophica cervicalis of Charcot, 56. - sympathica, 19. - syphilitic, 103. - - Mercurialization, 98. - - Mercury, 58, 83, 85, 98, 148, 193, 235, 376, 377, 389, 391, 395, 486. - untoward results of, 363. - - Metasyphilis, 89. - - Metchnikoff and Roux, 427, 428. - - Microgyria, occipital, 47. - - Mignot, Joffroy and, 64, 66. - - Migraine, 19. - - Mitchell, H. W., 218. - - Mœbius, 429. - - Mott, F. W., 158, 257, 308, 396, 437. - - Multiple sclerosis, 253, 256. - relation of syphilis to, 254. - spinal fluid findings in, 254. - - Muscular atrophy, 149, 446. - syphilitic relation to amyotrophic lateral sclerosis, 150. - - Muscular weakness, 279. - - Myerson, A., 196. - - - Nageotti, 428. - - Nausea, 63. - - Neisser, 399. - - Nerve trunk tenderness, 148, 234. - - Nervousness, 63. - - Nervous indigestion, 63. - - Neurasthenia, 63, 183. - - Neuritis, cranial, 51. - optic, 365. - root, 235. - syphilitic, 235. - - Neurorecidive, 152, 153, 184, 196, 235. - - Neuroses, relation of syphilis to, 186. - - Neurosyphilis, 187, 238, 240, 242. - aggravated on military service, 404. - atypical, 258, 346. - atypical case resembling hysterical fugue, 264. - dates, 428. - forms of, 20, 21, 28, 29, 95. - galloping, 328. - history of, 427. - incubation period, 152. - infectiousness of, 95. - laboratory findings in, 82. - latent, 142, 203. - lesions, 303. - lighted up by stress of military service, 412. - and marriage, 319. - prevention, 320. - onset, 64. - in primary stage, 186. - in secondary stage, 185, 283, 390. - in secondary stage, prognosis, 390. - in secondary stage, treatment, 153. - spinal, 23. - and the war, 399, 466. - - Nissl-Alzheimer method, 427. - - Noguchi, 381. - and Moore, 428, 429. - - Nonne, 82, 125, 152, 186, 195, 196, 214, 216, 235, 254, 265. - -Apelt test, 473. - - Numbness, 56. - - Nystagmus, 45, 253, 256, 279. - - - Obersteiner, 249. - - Occupation-neurosis, 312. - - Ogilvie method, 487. - - Operation for gumma, 139. - - Optic atrophy, 256. - in juvenile paretic neurosyphilis, 154. - - Optic thalamus, syphilitic lesion of, 205. - - Osteitis, syphilitic, 311. - - Ozena, 350. - - - Pains, 31. - - Pandy test, 474. - - Paralysis, 123. - recovery from, 342. - of respiration, 248. - - Paranoia, syphilitic, 225. - - Paraphasia, 19, 43. - - Paraplegia, 26, 30. - - Parasyphilis, 89. - - _Paresis sine paresi_, 126, 186, 204, 303, 445. - - Paresis, see paretic neurosyphilis. - - Paretic neurosyphilis, dementia paralytica, general paresis, softening - of the brain, 37, 63, 68, 74, 78, 80, 85, 97, 131, 188, 192, 197, - 199, 202, 227, 241, 262, 289, 295, 309, 314, 323, 338, 372, 375, - 377, 382, 384, 386, 388, 392, 435, 440, 442. - adjuvant causes of, 414. - causing social complications, 289. - causes of death in, 197. - course, 85. - duration, 88. - forms, 95. - improvement, 377. - incidence among officers, 407. - incidence among soldiers, 402. - lesions of, 131. - “lighted up” by domestic stress in civil life, 420. - “lighted up” by “gassing,” 414. - mortality from, 89. - nomenclature, 88. - onset, 192. - pathology of, 436. - prognosis, 435, 444. - symptoms, 90, 131. - symptoms, mental, 87. - symptoms, physical, 86. - versus diffuse neurosyphilis, 165. - versus vascular neurosyphilis, 169, 172. - with very marked meningitis, 332. - with very marked brain atrophy, 335. - without mental symptoms, 315. - traumatic exacerbation, 310. - traumatic form, 308, 413. - traumatic, shell-shock, 401. - treatment of, 85, 370, 372, 377, 382, 384, 386, 388, 392. - treatment, results of, 351. - - Pensions for disabilities resulting from venereal disease, 409. - - Pensions for neurosyphilis, 411. - - Peripheral neurosyphilis, 19. - - Perivascular infiltration, 41. - - Pernicious anemia with spinal symptoms, 267. - - Petit mal attacks, 195. - - Pförringer, 61. - - Phobia, 67. - - Pilcz, Mattauschek and, 347. - - Pitres and Marchand, 421, 424. - - Plaut, 249, 348, 428. - - Plaut, Rehm and Schottmüller, 471. - - Plasmocytosis, 40, 49, 55. - - Pleocytosis, 23, 220, 247, 344. - effect of antisyphilitic treatment on, 244, 376. - in remissions, 243. - significance of, 243. - spinal fluid otherwise negative, 270. - - Polydipsia, 190. - - Polyuria, 190. - - Pontine hemorrhage, 219. - softening, 54. - - Posey and Spiller, 257. - - Potassium iodid, 58, 85, 98, 193, 222, 376, 377, 389, 486. - - Preparesis, 65, 77, 78. - - Prince, Morton, 195. - - Psammoma, 213. - - Pseudoneurasthenia, 66. - - Pseudoparesis, 449. - alcoholic, 222, 229, 451. - diabetic, 238. - senile, 263. - shell-shock, 421. - syphilitic, 223, 371. - - Pseudoparetic neurosyphilis, 222. - - Pseudotabes, shell-shock, 424. - - Psychogenic neurosyphilis, 189. - - Psychographic disturbance, 228. - - Psychopathic personality, 302. - - Ptosis, 350. - - Pupillary reaction, changes in, 261. - signs, 69. - - Pupils, Argyll-Robertson, see Argyll-Robertson pupils. - irregular, 79, 201. - normally reacting in paretic neurosyphilis, 199. - sluggish reaction to light, 188. - stiff as isolated symptom, 265. - - Purkinje cells, binucleate, 48. - - Putnam, James J., 19, 56. - - Pyramidal tract lesion, bilateral, 326. - sclerosis, 44. - - - Quadriplegia in juvenile paretic neurosyphilis, 275. - - Quincke, 427, 428. - - - _Randsklerose_, 24. - - Ravaut, 428. - - Ravaut, Sicard, Nageotti, Widal, 428. - - Rayneau, 407, 413, 414. - - Recovery, 77. - - Recurrences, 70. - - Redlich, 403. - - Régis, 73. - - Remissions, 122, 435, 445. - - Retardation, 187. - - Retention of urine, 56. - - Retinitis, hemorrhages, 365. - - Richards, R. L., 402, 404, 406, 409. - - Robertson, A. R., 59. - - Rod cells, 226, 297. - - Romberg sign, 141, 216, 279. - - Root-sciatica, syphilitic, 418. - - Rosenau, 471. - - Ross-Jones test, 473. - - “Rum fit,” 229. - - Ryder, Charles T., 42. - - - Saddle-shaped nose, 210. - - Salivation, 98. - - Salmon, Thomas W., 89. - - Salvarsan, 75, 83, 85, 193, 222, 377, 389, 486. - provocative, 78, 79. - untoward results of, 363. - - Salvarsanized serum, 75. - - Schaudinn, 427, 429. - - Sciatic pain in neurosyphilis, 149. - - Seizures, 31, 64, 83, 103, 444. - causes of in paretic neurosyphilis, 194. - Jacksonian, 392. - minor, 392. - - Senile arteriosclerotic psychosis, 262. - - Sensitized cells, 478. - - Sérieux and Ducaste, 96. - - Shaikewicz, 404. - - Shanahan, 278. - - Sheep’s corpuscles, 477. - - Shock, 42, 81. - - Sicard, 428. - - Six tests, 80, 85. - in tabetic neurosyphilis, 141. - - Smith and Solomon, 479. - - Social cases, 454. - service, 232. - - Solomon, 142, 255. - and Koefod, 243. - Smith and, 479. - Southard and, 202, 303. - - Somnolence, 45. - - Southard, E. E., 48, 134, 212. - and Canavan, 70. - and Solomon, 202, 303. - and Taft, 397. - - Spasms, clonic, 326. - - Spastic hemiplegia in paretic neurosyphilis, 323. - - Spastic paraplegia, Erb’s, 147, 306. - - Spasticity, 18, 256. - - Speech defect, 69, 133. - - Spiller, 150. - Posey and, 257. - - Spinal fluid findings in secondary stage of syphilis, 151, 185, 283. - in juvenile paretic neurosyphilis, 275. - negative in diffuse neurosyphilis, 140. - negative in gummatous neurosyphilis, 138. - negative in neurosyphilis, 216. - negative in tabetic neurosyphilis, 269. - in tabetic neurosyphilis, 141. - - Spinal fluid, withdrawal for therapeutic purposes, 377, 379. - - Spinal syphilis, see diffuse neurosyphilis. - - Spirochetes, “drug fastness,” 381, 394. - strains, 76, 263, 276, 381, 394. - - Steida, 405. - - Sterility in tabetic neurosyphilis, 144. - - Stier, 407. - - Stokes, Wile and, 186. - - Suicide, 92, 126, 240, 296, 301. - - Summary, 427. - - Syphilis aggravated by service, 406, 411. - on service, 409. - - Syphilis as cause of diabetes, 241. - as cause of feeblemindedness, 396. - hereditaria tarda, 160, 318. - history of, 427. - lesions in, 329. - of lung, 211. - from Mongolian, 76. - primary, 65. - secondary, 65. - tertiary, lesions in, 329. - - Syphilitic feeblemindedness, pathology of, 160. - neuritis, 312. - psychosis, 91. - - Syphilophobia, 67, 361. - - Syphilotoxins, 72. - - Swift, 129, 212. - - Swift and Ellis, 428, 429. - method, 428, 487. - - - Tabes dorsalis, see tabetic neurosyphilis. - - Tabetic neurosyphilis, tabes dorsalis, locomotor ataxia, 30, 31, 141, - 146, 366, 367, 434, 446. - associated with cerebral symptoms, 177. - atypical, 143. - cervical, 146. - course, 141. - with negative spinal fluid findings, 269. - prognosis, 94. - shell-shock, 403. - “shell-shocked” into paretic neurosyphilis, 401. - symptoms, 93. - symptoms in order of frequency, 145. - treatment, 145, 366, 367. - plus vascular neurosyphilis, 175. - with vascular insult, 30, 439. - versus pernicious anemia, 267. - - Taboparesis, see Taboparetic neurosyphilis. - - Taboparetic neurosyphilis, taboparesis, 92, 135, 195, 284, 443. - course, 92. - nomenclature, 94. - prognosis, 92, 443. - and typhoid meningitis, 284. - - Taft, A. E., Southard, E. E., and, - - Talon, 407. - - Taylor, E. W., 50. - - Temperature, paretic, 376. - - Tests, changes under treatment, 102. - changed to negative in paretic neurosyphilis without clinical - improvement, 385. - changed to less strongly positive in paretic neurosyphilis without - clinical improvement, 386. - - Therapeutic conception, 324. - - Thibierge, 399. - - Thierry, 158. - - Throbbing in head, 63. - - Thrombosis, cerebral, 36, 42, 342, 357, 360, 124. - - Thymus, persistent, 282. - - Tibial exostoses, 100. - - Tigges’ formula, 248. - - Todd, J. L., 406, 409. - - Transient deafness, 18. - blindness, 18. - paralysis, 124. - paralysis, condition in which occurs, 123. - - Trauma and juvenile neurosyphilis, 278, 306. - neurosyphilis, 456. - paretic neurosyphilis, 199, 308, 310. - syphilitic osteitis, 311. - - Treatment of neurosyphilis, 67, 75, 83, 124, 148, 184, 222, 235, 299, - 328, 332, 335, 342, 346, 350, 351, 355, 384, 390, 392, 395, 419, - 439, 457. - case in which theoretically of no avail, 323. - methods, 356, 486. - - Treatment of syphilis, effect on development of neurosyphilis, 142, - 347. - - Tremor, 197. - intention, 256 - - Tubercle, 80. - - Tuberous sclerosis of Bourneville, 47. - - Tumor, cerebral, 53, 191, 238, 253. - pineal, 213. - - - Unconsciousness, 53. - causes of, 389. - - - Vascular changes, 220. - - Vascular neurosyphilis, 31, 42, 72, 296, 359, 433, 440. - plus tabetic neurosyphilis, 175. - prognosis, 433. - versus paretic neurosyphilis, 169, 172. - - Veeder, B. S., 274. - - Vertigo, 122. - - Viet, 278. - - Virchow, 427, 428. - - Vomiting, 53, 63. - - - Warthin, 241. - - Wassermann reaction, 191. - and alcoholism, 230. - in congenital syphilis, 160, 271. - meaning of “doubtful,” 360. - negative in diffuse neurosyphilis, 184. - negative in juvenile paretic neurosyphilis, 298. - negative in spinal fluid in spinal syphilis, 148. - negative in spinal fluid in neurosyphilis, 101. - negative in neurosyphilis, 252. - negative in paretic neurosyphilis, 77. - technique, 476. - titrations in spinal fluid, 348. - - Wassermann, Neisser and Bruck, 428. - - Weiler, 214. - - Weygandt, 403, 404. - - Widal, Sicard, Ravaut, 428. - - Wiles and Stokes, 186. - - Word-deafness, 35, 43. - - - X-ray diagnosis of bone conditions, 136. - - - Yerkes-Bridges, 304. - - - Ziehen, 409. - - Zsigmondi, 429, 474. - ------ - -Footnote 1: - - The cases chosen to illustrate the propositions of the boxed headings - always illustrate several other points. See the footnotes of Section - VI for lists of cases illustrating special points. The names assigned - to the cases are fictitious and chosen to suggest race or descent. - -Footnote 2: - - Notes of Dr. James J. Putnam. - -Footnote 3: - - M = meningeal - V = vascular - P = parenchymatous - -Footnote 4: - - E. E. Southard: Lesions of the granule layer of the human cerebellum; - _Journal of Medical Research_, XVI, 1907. - -Footnote 5: - - Proof of marked parenchymatous lesions must hang on post mortem data; - the inference here as to the presence of parenchymatous lesions is a - clinical inference. - -Footnote 6: - - Reprinted from an article by Southard & Solomon: “Latent neurosyphilis - and the question of _Paresis sine paresi_.” Boston Medical & Surgical - Journal, XXIV, 1. - -Footnote 7: - - Solomon: “How Shall Latent Syphilis be Treated? The Prophylaxis of - Syphilis of the Central Nervous System.” Interstate Medical Journal, - XXIII, 8. - -Footnote 8: - - Joseph Collins: Syphilis of the Brain, _Journal American Medical - Association_, July 10, 1915, Vol. LXV, pp. 139–144. - -Footnote 9: - - A. M. Barrett has recently discussed this subject in a paper in the - _Journal of the American Medical Association_, Vol. LXVII, Dec. 2, - 1916. - -Footnote 10: - - Reprinted from an article by Southard & Solomon: “Latent neurosyphilis - and the Question of _Paresis sine paresi_.” Boston Medical and - Surgical Journal, XXIV, 1. - -Footnote 11: - - E. E. Southard. A case of glioma of the pineal region, _Am. Jour. of - Ins._, Vol. LXI, 1905. - -Footnote 12: - - Since this was written Collins has had further difficulties related to - his neurosyphilis, improving under treatment. - -Footnote 13: - - Warthin: “Persistence of active lesions and spirochetes in the tissues - of clinically inactive or ‘cured’ syphilitics,” _American Journal of - Medical Sciences_, CLII, 1916. - -Footnote 14: - - “The Significance of Changes in Cellular Content of Cerebrospinal - Fluid in Neurosyphilis,” _Boston Medical and Surgical Journal_, - CLXXIII, 27. - -Footnote 15: - - Plaut: Ueber Halluzinosen der Syphilitiker, Berlin, 1913. - -Footnote 16: - - Borden S. Veeder: Hereditary Syphilis in the Light of Recent Clinical - Studies; Am. Jour. of Med. Sc., CLII, 1916. - -Footnote 17: - - Reprinted from article by Southard and Solomon: “Latent Neurosyphilis, - the Question of _Paresis sine paresi_,” _Boston Medical and Surgical - Journal_, XXIV, 1. - -Footnote 18: - - (This case was furnished by Dr. D. A. Haller from the Peter Bent - Brigham Hospital series.) - -Footnote 19: - - Fernald, W. E. Standardized Fields of Inquiry for Clinical Studies of - Borderline Defectives. Mental Hygiene, Vol. 1, No. 2, April, 1917. - -Footnote 20: - - Goddard, H. H., Feeblemindedness, its Causes and Consequences, 1914. - -Footnote 21: - - W. E. Fernald and E. E. Southard. Waverley Research Series in the - Pathology of the Feebleminded. Proceedings of the American Academy of - Arts and Sciences, 1917. - -Footnote 22: - - Thibierge. La Syphilis dans l’armée, 1917. - -Footnote 23: - - Hecht. Wien. klin. Woch., xxix, 51. - -Footnote 24: - - Donath. Beiträge zu den Kriegsverletzungen und -er-krankungen des - Nervensystems. Wiener klin. Wehnschr., No. 27–8, 1915. - -Footnote 25: - - Duco et Blum. Guide pratique du Médecin dans les Expertises - médicolégales militaires. Paris, 1917. - -Footnote 26: - - Birnbaum. Kriegsneurosen und -psychosen auf Grund der gegenwärtigen - Kriegsbeobachtungen: Sammelbericht. Z. f. d. ges. Neurol. u. - Psychiat., Bd. XII, H. 1, 1915. - -Footnote 27: - - Weygandt. Kriegseinflüsse und Psychiatrie. Jahreskurse f. ärztl. - Fortbildung, Maiheft, 1915. - -Footnote 28: - - Bonhoeffer. Erfahrungen über Epilepsie und Verwandtes im Feldzuge. - Monatschr. f. Psychiat u. Neurol., Bd. 38, H. 1–2, 1915. - -Footnote 29: - - Exner, M. J., Prostitution in its relation to the army on the Mexican - Border, _Social Hygiene_, Vol. 3, 2, April, 1917. - -Footnote 30: - - Bonhoeffer, _loc. cit._ - -Footnote 31: - - We have recently reviewed the outcome in 300 _untreated_ cases of - paretic neurosyphilis (Psychopathic Hospital material, strictly - comparable with treated cases) finding but 5 now capable of - self-support and 10 more in normal-looking remission. This percentage - is far lower than that in treated cases (at present, July, 1917, 50 in - 200 capable of self-support). - -Footnote 32: - - Alice Morton (1). - -Footnote 33: - - Francis Garfield (2). - -Footnote 34: - - John Dixon (3). - -Footnote 35: - - James Pierce (4). - -Footnote 36: - - John Lawrence (5). - -Footnote 37: - - Flora Black (6). - -Footnote 38: - - Mrs. Lecompte (7). - -Footnote 39: - - John Wyman (8). - -Footnote 40: - - Greeley Harrison (9). _Also_ - Albert Robinson (45), - Alice Caperson (46), - Abel Bachmann (74). - -Footnote 41: - - Lyman Agnew (10). _Also_ - Ethel Hunter (47), - Bessie Vogel (52), - Isaac Thompson (83), - Juliette Lachine (11). - -Footnote 42: - - Juliette Lachine (11). _Also_ - Lyman Agnew (10), - Ethel Hunter (47), - Bessie Vogel (52), - Isaac Thompson (83). - -Footnote 43: - - Henry Philipps (12). _Also_ - Bridget Curley (59), - Margaret O’Brien (68), - Annie Martin (117). - -Footnote 44: - - William Twist (13). _Also_ - Lester Crane (20), - Thomas Donovan (23). - -Footnote 45: - - John Jackson (14). _Also_ - Martha Bartlett (21), - Paolo Marini (28), - Margaret O’Brien (68). - -Footnote 46: - - Pietro Martiro (15). _Also_ - Meyer Levenson (22), - Achilles Akropovlos (50). - -Footnote 47: - - Joseph Sullivan (16). - -Footnote 48: - - Gregorian Petrofski (17). _Also_ - Richard Lawlor (25), - John Bennett (34), - Julius Kantor (54), - Albert Forest (112). - -Footnote 49: - - Frederick Wescott (18). _Also_ - Martha Bartlett (21), - James Burns (56), - Victor Friedburg (108). - -Footnote 50: - - Agnes O’Neil (19). _Also_ - Michael O’Donnell (24). - John Edwards (104). - Arthur Bright (121). - -Footnote 51: - - Lester Crane (20). _Also_ - Greeley Harrison (9). - David Borofski (49). - David Collins (61). - -Footnote 52: - - Martha Bartlett (21). _Also_ - Agnes O’Neil (19), - Vivian Walker (87). - -Footnote 53: - - Meyer Levenson (22). _Also_ - Albert Forest (112). - -Footnote 54: - - Thomas Donovan (23). _Also_ - William Twist (13), - Bessie Vogel (52), - David Collins (61). - -Footnote 55: - - Michael O’Donnell (24). _Also_ - Alice Morton (1). - -Footnote 56: - - Richard Lawlor (25). _Also_ - Bessie Vogel (52), - —— —— (88). - -Footnote 57: - - John Morrill (26). - -Footnote 58: - - David Tannenbaum (27). - _Also_ Mrs. LeCompte (7), - Annie Rivers (109). - -Footnote 59: - - Paolo Marini (28). _Also_ - Flora Black (6). - -Footnote 60: - - Mario Sanzi (29). _Also_ - Stephen Green (30), - Paul Halleck (31). - -Footnote 61: - - Stephen Green (30). _Also_ - Paul Halleck (31), - Henri Lepère (105), - Ivan Rokicki (111). - -Footnote 62: - - Paul Halleck (31). - -Footnote 63: - - Margaret Neal (32). - -Footnote 64: - - Joseph Graham (33). - -Footnote 65: - - John Bennett (34). _Also_ - Alice Caperson (46), - Florence Fitzgerald (81), - Vivian Walker (87), - Arthur Bright (121). - -Footnote 66: - - Mary Coughlin (35). - -Footnote 67: - - Theresa Mullen (36). _Also_ - John Lawrence (5), - John Friedreich (77), - Gridley Ringer (78), - James Arnold (80). - -Footnote 68: - - Isaac Goldstein (37). - -Footnote 69: - - Archibald Sherry (38). - -Footnote 70: - - Caroline Davis (39). - H. F. (40). - Samuel North (41). - Elizabeth Brown (42). - Robert Allen (43). - John Hughes (44). - -Footnote 71: - - Albert Robinson (45). _Also_ - Greeley Harrison (9). - -Footnote 72: - - Alice Caperson (46). _Also_ - Florence Fitzgerald (81). - -Footnote 73: - - Ethel Hunter (47). _Also_ - Lyman Agnew (10), - Bessie Vogel (52), - Juliette Lachine (11). - -Footnote 74: - - Milton Safsky (48). _Also_ - Daniel Falvey (55). - -Footnote 75: - - David Borofski (49). _Also_ - Lester Crane (20). - -Footnote 76: - - Achilles Akropovlos (50). - -Footnote 77: - - Daniel Wheelwright (51). - -Footnote 78: - - Bessie Vogel (52). _Also_ - Lyman Agnew (10), - Juliette Lachine (11), - Ethel Hunter (47). - -Footnote 79: - - Carrie Pearson (53). - -Footnote 80: - - Julius Kantor (54). _Cf._ - James Burns (56). - Henri Lepère (105). - Frederick Stone (106). - -Footnote 81: - - Daniel Falvey (55). _Cf._ - Francis Murphy (60). - -Footnote 82: - - James Burns (56). _Also_ - Frederick Wescott (18), - Martha Bartlett (21), - Victor Friedburg (108). - -Footnote 83: - - John Summers (57). - -Footnote 84: - - Peter Burkhardt (58). - -Footnote 85: - - Bridget Curley (59). - -Footnote 86: - - Francis Murphy (60). - -Footnote 87: - - David Collins (61). - -Footnote 88: - - Joseph Buck (62). - -Footnote 89: - - Albert Fielding (63). - -Footnote 90: - - Calvin Hall (64). - -Footnote 91: - - Donald Barrie (65). - -Footnote 92: - - Lawrence Washington (66). - -Footnote 93: - - Joseph Temple (67). - -Footnote 94: - - Margaret O’Brien (68). _Also_ - Henry Phillips (12). - Bridget Curley (59). - Annie Martin (117). - -Footnote 95: - - Frank Mason (69). - -Footnote 96: - - Annie Kelly (70). - James Lauder (71). - -Footnote 97: - - James Lauder (71). - -Footnote 98: - - Margaret Green (72). - -Footnote 99: - - Marcus Chatterton (73). - -Footnote 100: - - Abel Bachmann (74). - -Footnote 101: - - Mrs. Brown (75). - -Footnote 102: - - James Seabrook (76). - -Footnote 103: - - John Friedreich (77). _Cf._ - Isaac Goldstein (37). - -Footnote 104: - - Gridley Ringer (78). - -Footnote 105: - - John Doran (79). - -Footnote 106: - - James Arnold (80). - -Footnote 107: - - Florence Fitzgerald (81). _Also_ - John Bennett (34), - Alice Caperson (46), - Vivian Walker (87), - Arthur Bright (121). - -Footnote 108: - - Frederick Estabrook (82). - -Footnote 109: - - Maj. Isaac Thompson, M.D. (83). - -Footnote 110: - - Lester Smith (84). - -Footnote 111: - - Annie Marks (85). - -Footnote 112: - - Frank Johnson (86). - -Footnote 113: - - Vivian Walker (87). - -Footnote 114: - - —— —— (88). _Cf._ - Richard Lawlor (25). - Bessie Vogel (52). - -Footnote 115: - - Margaret Tennyson (89). - John Lawrence (5). - Mary Coughlin (35). - Theresa Mullen (36). - John Friedreich (77). - Gridley Ringer (78). - James Arnold (80). - -Footnote 116: - - Joseph O’Hearn (90). - -Footnote 117: - - Levi Sussman (91). - -Footnote 118: - - Joseph Larkin (92). - -Footnote 119: - - Richard Marshall (93). - -Footnote 120: - - David Fitzpatrick (94). - -Footnote 121: - - Joseph Wilson (95). - -Footnote 122: - - Becky Bornstein (96). - Walter Heinmas (97). - Mr. Jacobs (98). - -Footnote 123: - - Walter Heinmas (97). - -Footnote 124: - - Mr. Jacobs (98). - -Footnote 125: - - James McDevitt (99). - -Footnote 126: - - Jacob Methuen (100). - -Footnote 127: - - John Baxter (101). - -Footnote 128: - - Theodosia Jewett (102). - -Footnote 129: - - A. W. (103). - -Footnote 130: - - John Edwards (104). _Cf._ - Henri Lepère (105), - Frederick Stone (106), - Arthur Bright (121), - Agnes O’Neil (19), - Paolo Marini (28). - -Footnote 131: - - Henri Lepère (105). _Cf._ - Julius Kantor (54). - -Footnote 132: - - Frederick Stone (106). - -Footnote 133: - - Greta Meyer (107). _Cf._ - John Jackson (14). - -Footnote 134: - - Victor Friedburg (108). - -Footnote 135: - - Annie Rivers (109). - -Footnote 136: - - Mr. McKenzie (110). _Cf._ - Ivan Rokicki (111). - -Footnote 137: - - Ivan Rokicki (111). - -Footnote 138: - - Albert Forest (112). _Cf._ - Gussie Silverman (113), - Walter Henry (114), - William Rosetti (116), - Annie Martin (117), - Levi Morovitz (122), - Peter Burkhardt (58). - -Footnote 139: - - Gussie Silverman (113). - -Footnote 140: - - Walter Henry (114). - -Footnote 141: - - Henry Ryan (115). - -Footnote 142: - - William Rosetti (116). - -Footnote 143: - - Annie Martin (117). _Cf._ - William Roberts (118). - -Footnote 144: - - William Roberts (118). - John Silver (119). - -Footnote 145: - - John Silver (119). - -Footnote 146: - - James McGinnis (120). - -Footnote 147: - - Arthur Bright (121). _Cf._ - Levi Morovitz (122), - John Bennett (34). - -Footnote 148: - - Levi Morovitz (122). - -Footnote 149: - - Robert Matthews (23). _Cf._ - Isaac Goldstein (37). - -Footnote 150: - - For cases in which, without autopsy we have risked the diagnosis - neurosyphilis _in the absence of W. R. in serum or fluid_, see William - Twist (13), Frederick Wescott (18), Martha Bartlett (21), Thomas - Donovan (23), Paolo Marini (28), Margaret Neal (32), Bridget Curley - (59), Victor Friedburg (108), Ivan Rokicki (111). - -Footnote 151: - - From Mallory and Wright: Manual of Laboratory Technique. - -Footnote 152: - - Bruck. Münch. med. Wochen. Jan. 22, 1917. - -Footnote 153: - - Smith and Solomon. Boston Medical and Surgical Jour. - -Footnote 154: - - Bruck: Journal of American Medical Association, Vol. lviii, No. 12, - March 24, 1917, p. 944. - ------------------------------------------------------------------------- - - - - - TRANSCRIBER’S NOTES - - - 1. Pg. 456, added footnote anchor for footnote A. - 2. Silently corrected typographical errors and variations in spelling. - 3. Archaic, non-standard, and uncertain spellings retained as printed. - 4. Footnotes were re-indexed using numbers and collected together at - the end of the last chapter. - 5. Enclosed italics font in _underscores_. - 6. Enclosed bold font in =equals=. - 7. 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