Atypical case of neurosyphilis. Picture of Huntington’s chorea.
Case 72. Margaret Green, 28, was received at Danvers State Hospital in an excited and frightened state. She was very talkative and said that she was being bitten by snakes and serpents. She thought every one approaching her was the devil, and sprinkled what she called “holy water” about her for protection. It was clear that she was hallucinated. She heard her child crying, and she saw a woman carrying it away.
After a few weeks, Mrs. Green grew quiet and more rational except for a few spells of violence and noise; she gave the impression of a rather pleasant and agreeable, though somewhat demented, patient. Physically, beyond a tremor of fingers and tongue and lively knee-jerks and some evidence of enlargement of the heart, there was nothing to be found.
Margaret Green is still in the Danvers Hospital, being now 48 years of age. During the twenty years, she has presented,—besides the mental picture of impairment of memory—occasional spells of confusion, a variety of delusions based, at least in part, upon auditory and vivid visual hallucinations, a certain irritability and psychomotor excitement, and a picture of Huntington’s chorea. The diagnosis of Huntington’s chorea has always been in doubt by reason of the lack of any evidences of hereditary taint; it has, however, not been possible to secure a properly intensive account of her relatives.
It appears that the choreic movements were first observed—in the hospital at least—about 16 years ago. The patient has always been decidedly mixed upon dates. From internal evidence derived from her obviously in part erroneous statements, it may be that the chorea began at the age of 23. It appears that she had been often termed a victim of St. Vitus’ dance, and had had to leave her work in the mill on account of the disease. From one source of information, it would appear that the patient began to have what was called St. Vitus’ dance when she was 14 or 15 years of age; so far as this informant knew, no other member of the family had had the affliction.
The first movements observed in the hospital were irregular, jerking movements, more marked in the left arm but also occurring in the other extremities, as well as in the face, wherein were produced peculiar grimaces. The twitching movements would become decidedly worse during spells of irritability. Observation in the patient’s early thirties left the question in doubt whether the left pupil reacted to light or not. In 1904, when the patient was 36, both pupils failed to react to light either directly or consensually. At this time, the jerky movements continued, especially in the left hand and forearm, the tongue was tremulous, test phrases were poorly pronounced, the knee-jerks were exaggerated (especially the left), and both wrist-jerks were exaggerated. The systematic examination, however, revealed no other neurological disorder. Within a year, slight spurious ankle clonus developed on both sides; the eyes, especially the left, gave the appearance of developing cataracts. A slight consensual light reaction was demonstrable on the right side, but all light reactions were absent in the left eye.
At the age of 42, the patient was still disoriented for time, place, and persons and subject to a deep amnesia; was tidy, tranquil, and of a pleasant demeanor, but many of her muscles were in continual motion. There were chewing movements and both hands and feet were rarely still. There were no longer any spells of irritability or violence; and once when found crying on the piazza, Mrs. Green, on being asked the reason, replied that a gray cat had come and looked at her so hard it made her cry. There were other crying spells at times for equally good reasons, or for no reason.
More recently, the patient has become fairly well oriented for time and place, and has acquired a fairly good insight into her condition and a good memory for past events. She has had occasionally auditory hallucinations, as of water running. In 1914, it was reported that the pupils reacted to light, and the rest of the systematic neurological examination was negative except that the knee-jerks were exaggerated; and a re-examination in 1916 showed the pupils still reacted to light. At present, the patient is disoriented for time, stating that her age is about 25; she is no longer subject to auditory hallucinations; she has a marked difficulty in enunciation, emphasized by the lack of teeth and in part due to continual movements of the tongue; the movements appear to be part of a generalized chorea.
In a systematic review of the Wassermann findings in the hospital population, the blood of Margaret Green was examined and found to be positive. Lumbar puncture forthwith performed showed a positive W. R. in the fluid; there was a positive globulin and an excess of albumin; the gold sol was characteristic of paresis; there were, however, but three cells per cmm.
1. Are the choreiform movements related to the demonstrable syphilis of the nervous system? Neither the fluid W. R. nor the gold sol reaction should be regarded as necessarily an indicator of tissue loss. The fluid W. R. is commonly thought to signify merely that the fluid contains substances which are somehow due to the presence of spirochetes in some region pretty closely related with the fluid. The gold sol reaction, although well established to be characteristic of neurosyphilis, is perhaps not so strong an evidence of the existence of spirochetes in the region from which fluid constituents are derived. There is no pleocytosis. However, the positive globulin test and the excess of albumin do indicate a certain amount of destructive process somewhere in the neural tissues. Are we to suppose that these substances have been continually found during the course of this disease? This question cannot be answered with the data in hand, and we can only suspect that these positive tests for albumin and globulin are an effect of tissue destruction caused by neurosyphilis. It must be admitted that the argument here is a little tenuous. The lesson is plain: that in the present stage of our knowledge the W. R. should not be omitted even in cases which present a fairly convincing picture of some well-known entity. Thus, a disease, which looks like Huntington’s chorea, as well as a disease suggestive of multiple sclerosis, requires investigation by the methods of the syphilographer.
2. How shall we explain the changes in pupillary reaction in this case? They cannot yet be explained. A few observers have reported changes in pupillary reflexes in the direction of normality. In our experience such changes have not been noted. It cannot be too strongly emphasized that it is very easy to make errors in judging pupillary reaction if care is not used. For instance, if the patient is accommodating for near vision, light will probably not cause contraction. A frequent cause of error in testing the light reflex arises from using a weak electric light. An electric flash-light is much less efficient than daylight. Probably the most satisfactory method is to take the patient to a window, ask him to look at a distant object, shade the eye with the hand, remove hand, and observe.
3. What is the chief triad of symptoms in Huntington’s chorea? (1) Choreiform movements associated with (2) progressive mental enfeeblement, (3) occurring in a patient whose family history shows a similar condition in a preceding generation.
Differential diagnosis between NEUROSYPHILIS and SENILE ARTERIOSCLEROTIC PSYCHOSIS.
Case 73. Marcus Chatterton was a retired sea captain, 75 years of age. At the age of 71, he had had a seizure with a slight right hemiplegia and inability to talk. He had been slightly confused for a short time but had rapidly recovered. During the intervening four years, there had been three similar attacks, and the last one had caused him to come to the hospital. He was, in fact, confused upon admission but had become perfectly clear by the next day. There was a considerable memory defect, which the patient himself did not entirely appreciate. Possibly his judgment had been deteriorating slightly. He had been irritable of late and sometimes sleepless.
Physical examination showed a rather well preserved man with but slight senile changes. The pupils were equal and reacted readily to light and accommodation. There was no sensory disorder and no disturbance of coördination. There were no tremors. The systolic blood pressure was 205, the diastolic 135. The arteries were sclerotic upon palpation. A sufficient diagnosis would have seemed to be arteriosclerosis, and the hypothesis of syphilis would hardly have been raised off-hand by most practitioners. The W. R. of the serum was negative. What led to lumbar puncture in this case was the fact that the sea captain’s wife had died 15 years before of general paresis. The lumbar puncture was rewarding since the W. R. was positive. There was an increase of albumin and globulin, a “paretic” type of gold sol reaction, and 56 cells per cmm.
Accordingly, we must regard the condition as one of neurosyphilis. Perhaps the arteriosclerosis was of syphilitic origin. If this is a case of general paresis as we suppose, it is one of very long-standing syphilis.
1. Do delusions of grandeur in the senile period suggest syphilis? Not necessarily; it appears that there is a small group of senile cases which might be called cases of senile pseudoparesis in which extravagant delusions of grandeur are entertained, and in which frontal atrophy is found although entirely without evidence of chronic inflammation. It has not been proved that these cases are of syphilitic origin. It is suggestive that the site of the most extensive lesion is precisely the site of the most extensive lesion classically found in paretic neurosyphilis, viz., in the frontal regions.
2. Is neurosyphilis frequently found in both mates? It can hardly be said that this is a usual finding. However, it is far from rare, and it occurs frequently enough to be used in support of the theory that there is a special strain of spirochete that has a predilection for nervous tissue. It must be remembered, however, that the wives of syphilitics are frequently infected without being aware of it. In such cases they receive no treatment and consequently have a larger chance of developing neurosyphilis. It is a good rule to consider the mate of every syphilitic a candidate for neurosyphilis.
An atypical case of recurrent dazed states resembling HYSTERICAL FUGUES. Probably an instance of NEUROSYPHILIS.
Case 74. Abel Bachmann, a man of 40 years, remains doubtful and perhaps belongs to the still unresolved group of mental cases due to syphilis that cannot be placed in any of the well-known categories. Bachmann had been found by the police, working in front of a cowbarn without the consent or even the knowledge of the owner. Bachmann had, in fact, spent the night in the cowbarn and was working with the idea of paying for his night’s lodgings. The situation struck the police as so peculiar, and Bachmann was so confused and irresponsive, that he was brought to the Psychopathic Hospital. The afternoon of his admission, however, he entirely cleared up and was able to give a good account of himself.
His story was that he had been worrying a good deal about a divorce suit, and the morning of his episode he had awakened with peculiar feelings. He walked from Boston to Cambridge, feeling that he was in a strange city. He recognized the places he passed, yet they all seemed to be changed. Upon reaching Harvard Square, he determined to return to Boston and walked and walked, failing to reach Boston. All day he had eaten nothing; when night fell he stole into a field and dug out radishes. A postman stopped and said, “Hello, Bill,” which awakened him as by an electric shock. A barn presented itself, in which he spent the night. In the morning, the barn looked different. In fact, his entire surroundings appeared mysterious. As he felt like working, he went to work in front of the barn.
It seems that in his life there had been two other episodes of a similar nature; in fact, Bachmann had been in a state hospital for six weeks after the first episode. The first episode had lasted a few days only, and followed worry when he learned that the girl with whom he was in love was married. The second attack followed the death of his mother, whereupon he was taken to a state hospital although the total duration of symptoms was only three days. Bachmann had had a chancre or some other form of genital disease at 26, and had at that time been treated with mercury.
Except for irregular and absolutely rigid pupils, reacting neither to light nor to accommodation, Bachmann showed no physical and especially no neurological disease whatever. Moreover, the W. R. in the blood serum was negative.
As to diagnosis, one might consider hysteria, of which, however, there are no visible stigmata. It would not appear that brain tumor would be likely to have lasted so long as eight or nine years, even if we should attempt to make the hypothesis of tumor cover both the non-reacting pupils and the episodes. Bachmann was non-alcoholic, and there was no sign of any other form of intoxication. The spinal fluid showed a negative gold sol reaction, there were no cells in the fluid, there was no globulin; albumin was normal. However, the W. R. was strongly positive.
The situation, then, in this case is that we have somewhat peculiar psychopathic episodes, pupils rigid to light and accommodation, a positive W. R. in the spinal fluid, and extremely little else to permit a diagnosis. We are ignorant as to the course and pathology of such cases. However, we cannot resist the temptation of the diagnosis of neurosyphilis, although further classification is not ventured.
1. What is the significance of stiff pupil as an isolated symptom? Nonne finds that in the end, after years of observation, the Argyll-Robertson pupil turns out to be an advance courier of other more functionally serious signs and symptoms of neurosyphilis. We can confirm this experience and regard it as an established clinical proposition that the Argyll-Robertson pupil cannot be neglected. In this connection, refer to the case of alcoholic pseudoparesis (Murphy, 60), and also to the case of pineal tumor (Donald Falvey, 35). Enthusiastic reports have occasionally been made upon apparent restoration of the true syphilitic Argyll-Robertson pupil to normal light reaction. The difficulties in rendering the symptomatic diagnosis of Argyll-Robertson pupil in a given case are so great, and the chances of complication so numerous, that we are inclined to attach little significance at present to these claims.
It may not be amiss to mention a somewhat humorous incident familiar to some local neurologists. A case was reported by the interne for a number of months as a victim of a pupil stiff to light and accommodation, and the entirely adequate cause of this phenomenon was actually only discovered at autopsy by the triumphant medical examiner, who demonstrated that the patient in question was possessed of a glass eye.
TABETIC NEUROSYPHILIS (“tabes dorsalis”) versus PERNICIOUS ANEMIA with spinal symptoms.
Case 75. Mrs. Brown was a woman of 56, who for the past eight or ten years had been complaining of trouble in her legs. As she described it, at times her legs were so weak she could hardly stand; at other times there was considerable pain and numbness. She has always been considered “high strung”; that is, she had a very bad temper and lost control of herself almost entirely when she became excited. Her legs had been growing progressively worse, and for about a month prior to admission she had been unable to stand or walk. She had also lost control of her bladder. On account of her temper, it had been almost impossible to nurse her; no nurse would stay with her because of her scolding and fault-finding. Recently, she had been having fits of the blues.
Her husband, who was seen before Mrs. Brown, was an old gentleman, over 70, who was chiefly remarkable from the fact that he had unequal, irregular pupils, which reacted neither to light nor accommodation; there was also a speech defect.
The patient herself proved to be extremely irritable, as had been stated,—so much so that at times it seemed almost impossible to do anything for her. She was very querulous, constantly complaining, and not satisfied with anything that was done. Aside from this, her mental examination proved to be entirely negative; that is, there were no psychotic symptoms.
The systematic physical examination gave the following significant findings: blood pressure, 160 systolic, 90 diastolic; no evidences, however, of peripheral arteriosclerosis. Patient was unable to walk or stand, and had no control over her bladder. The knee-jerks and ankle-jerks absent on both sides; ataxia in the leg movements; loss of sense of localization, with no tenderness over the nerve trunks; no atrophy, paralyses, or muscular asymmetry of the parts. The vibratory sense was maintained. Subjectively, the patient thought that the vibratory sense differed in the legs from that in the arms. Localization, touch, pain, heat, and cold responded to correctly. The arms showed nothing abnormal; there was no incoördination, dysmetria, or dysdiadochokinesis. Her pupils were equal, regular, and both reacted normally to light and accommodation.
Diagnosis: The first consideration in the case is naturally tabes dorsalis, especially when one considers that the husband had signs which suggested syphilis of the nervous system. The rapid onset of the acute symptoms in this case, and the absence of the characteristic signs of pain were at least atypical for this diagnosis, as was the absence of any pupillary signs. Further, the W. R. was negative in the blood and spinal fluid; there were no definite signs of inflammatory reaction as shown by the other spinal fluid tests. These findings made a diagnosis of tabes entirely improbable. After tabes, the most frequent cause of the symptoms above enumerated is perhaps to be found in pernicious anemia. Examination of the blood showed that the patient had 2,500,000 erythrocytes per cmm. The hemoglobin by Tallquist scale was 80%. The smear was practically negative; no blasts could be seen. Although this picture is not a typical one for pernicious anemia, at least it is significant in the low number of red cells to be found, and as no causes for anemia were to be found, it seemed probable that we were dealing with a primary anemia. The diagnosis in the case, therefore, is spinal sclerosis of primary anemia. The mental picture was not uncharacteristic of Pernicious Anemia.
1. Could the diagnosis be rendered in this case without a lumbar puncture? In the first place, the emaciation is not entirely characteristic. The pupils react normally to light. Probably such a case might well have been regarded as one of tabes dorsalis in former days, or even at the present time, if a lumbar puncture had not been resorted to.
2. Could this case possibly have been one of tabes dorsalis with negative spinal findings? Such cases have been reported frequently, but, unlike the present case, are apt to be of long-standing and non-progressive, in which the active inflammation is no longer present. The negative findings would not be consistent with tabes, in which the symptoms are of short duration and of sudden onset.
3. If the serum W. R. had been positive would the diagnosis have been neurosyphilis? We are loath to make the diagnosis of spinal syphilis when the spinal fluid is normal. Syphilis may produce a marked anemia, however, and thus produce symptoms such as shown by Mrs. Brown. It is even possible that such is the explanation of this case, taking into consideration the suggestive findings in the husband. However, there is insufficient evidence to make such an hypothesis rock firm, and we do not more than suggest it.
Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.
Case 76. James Seabrook, 20 years of age, obviously showed a number of signs of congenital syphilis. The physical examination disclosed an old scar and indentation in the right mastoid region, another on the right side of the neck, another on the posterior surface of the right forearm, and two on the outer surface of the right upper arm. The lesions were about the size of half a dollar. There was a saddle-shaped nose and a perforation of the palate and uvula; there were palpable cervical and axillary glands, small but numerous. There was a dulness in the region of the right scapula, and slight dulness on both sides behind. There were loud whispering and piping râles and bronchial breathing throughout the chest, more marked on the left; there was much coughing, and the sputum was at times blood-stained. The pupils were irregular but reacted perfectly. The left knee-jerk was slightly more active than the right. The W. R. in blood and fluid was negative; the gold sol, globulin and albumin tests were negative. There were, however, 56 cells per cmm. in the fluid.
We learned that the patient had had several spells of great excitement, with pounding on the door and a desire to fight bystanders. There were spells of headache and vertigo. Mentally the tests showed him to be subnormal.
The diagnosis Of Congenital Syphilis seems established; possibly the pulmonary condition is syphilitic. The mental subnormality as well as the abnormal traits and episodes are probably to be accounted for on the basis of syphilitic involvement of the brain.
1. Are the headache and vertigo connected with syphilis? This is perhaps suggested by the pleocytosis in the spinal fluid.
2. How shall we explain the negative W. R.? This patient had received, shortly before his entrance to the hospital, salvarsan and mercury. Possibly the drug treatment has little or nothing to do with the negative W. R.’s since they not infrequently grow weaker as congenital syphilitics grow older.
3. What is the explanation of the spells of excitement? Compare the spells of excitement in a form of neurosyphilis described by Kraepelin, namely: syphilitic paranoia, discussed in the case of Bridget Collins (59).
4. Is treatment indicated considering the W. R.’s to be negative in blood and fluid? Despite the negative W. R.’s in this case treatment is strongly indicated on account of the pleocytosis. This would seem to indicate that there is an active inflammatory process in the cerebrospinal axis, and it is more than probable that this process is syphilitic. How much real improvement of the symptoms would result from antisyphilitic treatment it is impossible to prophesy. Every case is a special problem, and this case is very unusual in showing a pleocytosis in the absence of other indications of syphilitic nervous disease, viz., globulin, albumin and W. R.’s.
CONGENITAL NEUROSYPHILIS resembling an undifferentiated case of FEEBLEMINDEDNESS—actually PARETIC.
Case 77. John Friedreich, a 7–year old boy, was brought to the Psychopathic Hospital by agents of a charitable society, who found him a neglected child and quite evidently a subnormal one.
The dominance of syphilis in the situation was clear. The boy’s father had died but a few months before of syphilitic heart disease, from which he is said to have suffered for five years. The boy’s mother (the parents were first cousins) had also been treated for syphilis and was excessively alcoholic. The first child of this union—a girl—had died at 6 years, of a disease diagnosticated spinal meningitis. The history indicates that syphilis was acquired after the birth of this first child; but in any event it is possible that the meningitic condition of which the first child had died was syphilitic. The second pregnancy terminated in a stillbirth; the third issued in a girl, who died two weeks after birth of what was termed “inward convulsions.” The fourth pregnancy resulted in a miscarriage; the fifth in our patient, John Friedreich. The sixth pregnancy resulted in a girl, now 5 years of age, who is apparently normal. (Her W. R. was negative and she shows no stigmata of syphilis.)
The patient, John Friedreich, at some very early age had a rash on his body diagnosticated as syphilis. He also had many seizures called fainting spells. Ever since birth he had been taking mercury pills. He had not learned to talk until his third year, and was able then to say only a few disconnected words. In fact, John has never been able to talk in complete sentences, mumbling much that is quite unintelligible. However, he walked at 15 months in a normal fashion and nothing peculiar in his gait was noted until he was 5 years old, when he began walking on his toes, particularly those of his left foot. Shortly thereafter, the seemingly inevitable trauma appeared; John fell out of a window and severely injured his left leg, whereupon the peculiarity of toe-walking became more pronounced and associated with a limp.
Juvenile paresis. 7 years.
The patient strikes one physically as having the development of a child of about five years (actual age, 7). There are a few lymph nodes palpable in the anterior triangles of the neck. The dilated and slightly unequal pupils react neither to light nor accommodation. There is practically complete deafness; loud sounds are not at all noticed.
Withal, the child in a general way presents a somewhat attractive appearance, being very playful and mischievous, lying about on the floor and playing with whatever comes to hand, talking to himself or making a few indistinct remarks to the bystanders. He walks awkwardly, on the toes of the left foot. He pays little or no attention to his toilet and needs to be dressed and cared for in all ways. He is quick-tempered and at times very difficult to manage.
There was, of course, little doubt of the diagnosis of Congenital Syphilis and of Feeblemindedness. The W. R. was positive both in the blood and in the spinal fluid. The gold sol reaction of the fluid was of the “paretic” type; there were 44 cells per cmm. and there was a large excess of albumin and much globulin.
As to prognosis, there is doubt.
1. Is, or is not, this a case of juvenile paresis?
2. Is it, perhaps, a relatively permanent case of feeblemindedness due to congenital syphilis? On the whole, on account of the spinal fluid symptoms, we should be inclined to give the case a relatively poor prognosis, namely, of death in a few years. However, we may perchance be later surprised to learn that the patient has lived on, at least into early adult age.
Note: Mercury tablets in some cases of congenital syphilis do not seem effective. John Friedreich was treated most intensively by syphilographers from birth.
Dr. W. E. Fernald in a personal communication stated that syphilitic cases of feeblemindedness are rather those of the imbecile and idiot groups than of the higher levels. This statement emphasizes again that the true hereditary cases of feeblemindedness are rather those of the higher group, whereas the cases in which special causes have operated in the uterus or in early life eventuate in idiocy and imbecility. However, such a case as that of Friedreich shows that now and then a case of feeblemindedness without evidence of neurological disorder and looking in almost all respects like an hereditary case may be at times produced by syphilis.
3. How often is the central nervous system involved in hereditary syphilis? An interesting table bearing on this point is presented by Veeder.[16] The table concerns the lesions in various parts and systems of the body in 100 cases of late syphilis. It appears that in 47, or approximately one-half of Veeder’s series of 100 late cases, the infection developed some form of lesion of the nervous system. As Veeder remarks, this result runs counter to the common statements of pediatricians, notably of Holt.
| Bones: | |
| Periostitis tibia | 4 |
| Periostitis skull | 1 |
| Osteomyelitis | 1 |
| Joints: | |
| Acute arthritis knee | 8 |
| Acute arthritis ankle | 1 |
| Skin: | |
| Macular eruption | 1 |
| Condyloma anus | 3 |
| Gummata | 3 |
| Alopecia | 3 |
| Eye: | |
| Interstitial keratitis | 24 |
| Choroiditis | 1 |
| Ulcerations: | |
| Nasal | 2 |
| Laryngeal | 1 |
| Pharyngeal | 1 |
| Central Nervous System: | |
| Mental deficiency | 23 |
| Cerebrospinal syphilis | 14 |
| Hemiplegia | 6 |
| Epilepsy | 5 |
| Spastic paraplegia | 4 |
| Chorea | 2 |
| Hydrocephalus | 2 |
| Miscellaneous Conditions: | |
| Ozena | 1 |
| Enlarged spleen (only symptom) | 1 |
| Torticollis | 1 |
| Aortitis | 1 |
| Obscure abdominal pain | 1 |
| Obscure pain in legs | 2 |
| Endarteritis obliterans | 1 |
| Paroxysmal hemoglobinuria | 1 |
| Raynaud’s disease | 1 |
| Hutchinson’s teeth | 4 |
Juvenile paretic neurosyphilis. Quadriplegia.
Case 78. Gridley Ringer, 15 years of age, had the facies of a congenital syphilitic, including Hutchinsonian teeth, rhagades of the face, and the so-called Olympic brow. No secondary sexual characteristics had developed. There was a marked speech defect. Mentally, Ringer was a low-grade imbecile. He had been born at full term, and delivery had been normal. There had never been other pregnancies. He had never developed normally.
The father admitted syphilis 23 years before, namely, 8 years before the birth of his son, but the father had been treated for several years and had been declared cured.
1. What would be expected in the spinal fluid of this case? Without the history, it would perhaps be impossible to say whether the case was one of a quiescent imbecility or one of juvenile paresis. The spinal fluid of the juvenile paretic gives a picture identical with that in the adult. The spinal fluid in this case showed a positive W. R. (as did also the serum), a marked increase of albumin and globulin, 115 cells per cmm., and a “paretic” gold sol reaction. Accordingly, the diagnosis of General Paresis was made.
2. What is the prognosis? The prognosis of juvenile paresis is currently regarded as entirely grave. There is probably less hope for improvement in juvenile paresis than in the acquired paresis of adult life, since it seems to be a general principle that congenital syphilis is always more difficult to cure than acquired syphilis.
This case had seizures a few months after initial observation, and the seizures were followed by a transient right hemiplegia. This right hemiplegia was shortly followed by a left hemiplegia, which remained permanently. Moreover, a few weeks later, a right hemiplegia again developed, leaving the patient with complete paralysis and aphasia. Death followed in six weeks.
3. What effects were shown in the parents? Following up the parents was rewarded by the discovery that the mother was suffering from nerve deafness, probably of syphilitic origin, and that the father had recently begun to suffer from what he considered rheumatism, but which on examination was shown to be tabetic neurosyphilis (“tabes dorsalis”). This family again supports the hypothesis that there is a strain of spirochetes especially prone to attack the nervous system. Here it would seem that the syphilis acquired by the father had infected the mother and been transmitted to the son. In all three infected by the same strain or strains of organisms the nervous system was involved. It is difficult, nevertheless, to explain on this hypothesis why in one case the disease took the form of tabes dorsalis, in the second, eighth nerve involvement and in the third, paresis. This question of strains is really more than academic because it enters deeply into the question of treatment, as well as that of the suggested increased viability of the neural strain.
Is there a relation between epilepsy and juvenile neurosyphilis?
Case 79. John Doran fell off the rear of an ice-wagon, at six years of age, and shortly afterward developed fits. It appears that John was not unconscious at the time of his fall, but that he complained of headache. Although the convulsions were fairly frequent at first, it appears that they later became rare and occurred only when the patient got into a temper. At the stage of exhaustion after violent excitement, John would fall.
Physically, at 9 years a fair development and nutrition were evident. There was a great exaggeration of the frontal bosses; the nose could not be said to be typically saddlebacked, yet there was a suggestion of a sinking of the bridge. The teeth slightly suggested the Hutchinsonian type, but only slightly. There was a slight roughening of the tibia, and there was a slight scar over either knee. The patient graded according to the Binet scale at 9 years, and he was regarded as definitely feebleminded.
The family physician states that, according to his information, the father contracted syphilis when the child was between three and four months of age, and that the mother also was infected at this time. However, the child had not been suckled except immediately after birth, and there had been no evidences, according to the family physician, that John had acquired syphilis.
Ordinarily, one might content himself regarding the case of John Doran as one of idiopathic epilepsy with mental defect or deterioration. However, the frontal bosses, suggestive teeth, the flattened bridge of the nose, the roughened tibiae, and the old scars, though singly not of great significance, collectively make one suspicious. Despite the family physician’s belief that John could not have acquired syphilis from the parents, the infection seems entirely possible despite the fact that no symptoms developed early thereafter.
The W. R. in this case proved positive in both blood serum and spinal fluid.
1. What is the relation of trauma to this case of Juvenile Neurosyphilis? Probably none.
2. What would be the effect of treatment? For a number of years John Doran was lost sight of. He was, however, treated, according to our information, with intraspinous injections of salvarsanized serum, whereupon his convulsions shortly ceased. He has been recently examined mentally once more, and still grades as feebleminded. He still has violent outbreaks of temper.
3. Is such a case as Doran typical? Shanahan has investigated conditions at Craig Colony. There were 22 out of 886 epileptics (at Craig Colony) or 2½%, who showed a positive W. R. Nine of these cases were regarded by Shanahan as cases of epilepsy actually caused by syphilis. Viet had found 7%, and Bratz and Lüth 5% of constitutional epileptics to be syphilitic, but the data of these German authors were obtained before the era of Wassermann tests.
Adrenal tuberculosis complicating juvenile paretic neurosyphilis (“juvenile paresis”). Autopsy.
Case 80. When James Arnold appeared at the Danvers Hospital in his 22d year, he looked as if he were but 12 or 14 years of age. He was excessively fat but of fair muscular development. The left eye diverged outward, and the left pupil was smaller than the right. An odd feature was a high degree of pigmentation of the skin of the genitalia and the groins (the axilla, the mammillary areas, and the oral mucosæ were free from pigmentation). Physically speaking, the patient was practically normal. Neurologically, however, there was much of interest, in the light of which the clinical history was of value.
It seems that after an apparently normal early childhood, the boy had begun, at the age of 11, to experience difficulty in carrying out every-day school tasks; and after this his mental capacity had slowly but progressively deteriorated. The deterioration was not merely intellectual, but the boy became dishonest and untrustworthy and developed a number of untidy and uncleanly habits, behaving at the age of 16, as the parents stated, like a child of six.
In his seventeenth year, the boy had been taken with a severe attack of what was regarded as an “attack of indigestion.” This attack ushered in a gradually developing muscular weakness, especially involving the limbs. By the age of 21 he had become irritable and the paresis was so extreme that the patient was unable to get in or out of a carriage.
This generalized muscular weakness was plain upon admission to the hospital though there seemed to be no actual paralysis. The patient was unable to walk in a straight line and Romberg’s position could not be maintained. Marked tremor was present in the hands and lips. There was bilateral impairment of vision and nystagmus. Reflexes and sensations normal. Speech was markedly affected, all syllables being very much slurred. School knowledge and memory for both recent and remote events very poor. The patient’s habits were very untidy. He was very emotional, easily made to laugh or cry; and in behavior, extremely childish.
Two months after his admission to the hospital, the weakness suddenly became extreme. He was constantly nauseated, refusing food. The face and hands were cyanosed and the heart’s action rapid, weak, and irregular. This attack lasted for a week and was followed by a period of improvement, during which, however, he still remained very weak and apathetic.
One month later he again became so feeble that he was unable to raise himself in bed. He complained persistently of feeling very “sick.” His temperature was elevated and there occurred the same train of circulatory symptoms observed previously, viz., rapid and tumultuous action of the heart, with cyanosis of face and extremities. He soon became unconscious, remaining so until his death, which occurred on the seventh day of the acute attack.
This case was under observation before the days of the W. R., yet clinically the case had been diagnosticated Juvenile Paresis. There was no history of the acquisition of syphilis or any likelihood of its acquisition. Considered clinically, many of the classical features described by Addison were present, viz., marked asthenia and apathy; severe and frequent gastro-intestinal symptoms (the disease probably commencing with the attack of so-called “acute indigestion” six years prior to patient’s death); attacks of extreme cardiac weakness with the characteristic small, low-pressure pulse. On the other hand, pigmentation of the skin (usually the most striking clinical feature) was limited to the external genitalia, these being colored a deep brown.
The most striking feature found at autopsy was a bilateral adrenal tuberculosis (caseation, giant cells, lymphocytosis, tubercle bacilli). The thymus gland was persistent (7×5×.5 cm.), whereas the thyroid gland was smaller than usual. The brain showed macroscopic and microscopic features entirely consistent with the diagnosis of general paresis, including lymphocytosis, plasmocytosis, irregular degrees of nerve cell destruction, and gliosis, with an especially characteristic microscopic picture in the frontal regions.
It may be of note to consider the degree of change undergone by a brain in 11 years or more of deterioration, and the following description of the head findings is therefore included:
Head: Hair abundant, dark. Scalp normal. Calvarium, weight 435 gm., transparent in bregmatic region only, elsewhere thick and dense. The average thickness of the vertical plate of the frontal bone is 7 mm. The frontal bone shows a moderate thickening and hardening of the inner table with obliteration of diploë. Dura mater moderately adherent to the bregmatic region of calvarium. Arachnoidal villi moderately developed. Sinuses not remarkable. Pia mater shows a moderate focal thickening with opacity, especially along sulci. Vessels well injected. Brain: Weight, 1200 gm. The brain shows marked focal variations in sulcation and consistence. Spread on a board, the right hemisphere is obviously somewhat bigger than the left. There is a difference of only 0.5 to 0.75 cm. on measurement of the greatest circumference of the cerebrum, taken from the median line superiorly to the median line inferiorly, but the right hemisphere is throughout slightly more convex than the left. Both postcentral gyri are much narrowed in their superior portions, and the sulci posterior thereto are deeper than the other sulci of the hemispheres. The sulci of the orbital surfaces are asymmetrical and, on the left side, show a tendency to microgyria. The cerebral hemispheres as a whole show a remarkable tendency to slight protrusion of the border gyri; especially those of the two poles, of the free edges along the great fissure, and most strikingly the gyri at the boundary line between the inferior and lateral surfaces. This marginal prominence is slight but obvious and is emphasized by a slightly paler color in some regions. The cerebrum shows a general induration which is greatest in the frontal tips and along the inferior borders of the lateral surfaces of the hemispheres, especially right. The orbital surfaces are firm, especially anteriorly and externally (prefrontal); the tips of the temporal lobes are firm, and the superior temporal gyri are firmer than adjacent gyri. The postcentral gyri are indurated more than the other gyri of the superior surface. The hippocampal gyri are likewise firmer than adjacent gyri.
Cerebellum and pons: Weight, 145 gm. The inequality of the two hemispheres is more marked than in the case of the cerebrum.
Greatest lateral diameter; left, 4.5 cm., right, 5.5 cm.
Anteroposterior diameter adjacent to notch: Left, 5.8 cm., right, 5.5 cm.
There is no appreciable difference in depth. The diminution in volume appears to be chiefly at the expense of the right clivus. The inferior surface is firmer than the superior. The laminæ adjacent to the horizontal fissure are firmer than the remainder of the cerebellum. The pons is small.
There was also a lateral curvature of the spinal column, as well as characteristic adhesions between spinal dura and pia mater which are always suggestive of syphilis. For the rest, there were few findings of note: some adhesions of the left pleura, hypostatic congestion of the lungs, tracheitis, and chronic gastritis. There were four lobes of the right lung but it is doubtful whether this should be regarded as a stigma.
1. Can we separate the symptoms of Addison’s disease from those of paresis in this case? The extreme cardiac weakness with a characteristic, small low-pressure pulse is in point. The asthenia and apathy are consistent enough with Addison’s disease as well as with paresis itself. It would also be possible to ascribe the gastro-intestinal symptoms to either disease.
2. Of what significance is the persistent thymus? Persistent thymus has been observed in a few cases of Addison’s disease, but that it plays any part in the symptomatology thereof is a matter of doubt.
3. How can the obesity be explained? It is of course of note that the thyroid gland was small, but microscopically there were no peculiar features in this gland.
4. Was the adrenal tuberculosis actually primary? Minute search failed to reveal evidences of tuberculosis elsewhere unless we regard the few adhesions binding the lower half of the lung to the chest wall as indicative of an old tuberculosis. In particular, the mesenteric lymph nodes were normal.