  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 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 There were marked firm interadhesions between dura and pia throughout. A lumbar puncture soon after admission in 1907 had shown:
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 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 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 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 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.
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, 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 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. 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 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. 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 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. 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. 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. 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 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. 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. 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 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 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 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 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 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 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. 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 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 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 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 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. Cortical hemiatrophy—A, relatively normal right precentral (“motor”) cortex; B, atrophic left precentral. 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. 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 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 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. 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 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:
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 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. 1. Exudate in pia mater—mononucleosis. 2. Superficial (subpial) cellular reaction of neuroglia tissue (expanded cell bodies). 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.) 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 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. 4. Arteritis of pia mater. 5. Focal vascular lesions. Paretic neurosyphilis (“general paresis”)—cerebral atrophy, without meningitis. Therapeutics cannot hope to restore lost tissue. 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 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. 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 1. What is the diagnosis in this case? The following diagnoses and suggestions for diagnosis were made at the staff meetings:
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 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 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 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. 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. 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 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. 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 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 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 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 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. 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) 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. 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 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 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 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, 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 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. 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 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 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 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, 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 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, 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. 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. 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 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, 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. 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, 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. Juvenile paresis. 7 years. 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 3. How often is the central nervous system involved in hereditary syphilis? An interesting table bearing on this point is presented by Veeder.
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 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. 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 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 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 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 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. 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 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. |
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