Part 21
=Diagnosis=: The nystagmus, optic atrophy, and the reflex disorder suggested multiple sclerosis, although the liveliness of the superficial reflexes, especially the abdominal reflexes, was a point somewhat against any advanced degree of multiple sclerosis. It would appear that the absence of pupillary reaction to accommodation is also rather unusual in multiple sclerosis.
The serum and spinal fluid W. R.’s proved positive. There were 25 cells per cmm., albumin was in excess, and there was a positive globulin reaction.
1. What is the significance of optic atrophy and other optic changes with respect to neurosyphilis? Canavan, from our laboratory, has reported that she found that 40 of 58 unselected cases of mental disease exhibited obvious and undeniably important changes in the optic nerve. She found that optic nerve changes were even more frequent than chronic spinal cord changes as detectable by the same method (Weigert myelin sheath method); there were only 34 of the 58 cases which showed chronic spinal cord changes. Eighteen cases very probably syphilitic (although the clinical evidence was not in all cases supported by the W. R.) failed to show optic nerve changes in but three instances. The 15 syphilitic cases that did show optic nerve changes showed them in but one eye in three cases, in both eyes in 12 cases. Canavan incidentally demonstrated a spirochetosis in the pial sheath of the optic nerve in a case of neurosyphilis, possibly paretic.
2. What is the frequency of eye changes in neurosyphilis? Posey and Spiller (“The Eye and the Nervous System,” 1906) quote Kéraval as finding 42 instances of fundus change in 51 cases of paresis. Clifford Allbutt found 41 cases of atrophy in 53 of paresis; other authors have found far fewer. Optic atrophy sufficiently marked to cause blindness is relatively rare in paresis. Compare table of eye changes from Joffroy under Case Falvey (55).
As for optic atrophy in tabes, Posey and Spiller record statistics as so various as to be on the whole unsatisfactory. The highest percentages found appear to be those of Mott, 80%, and Gross, 88%. It is evident that the standards for measuring optic atrophy must differ very much.
=Atypical case of neurosyphilis. Picture of Huntington’s chorea.=
=Case 72.= Margaret Green, 28, was received at Danvers State Hospital in an excited and frightened state. She was very talkative and said that she was being bitten by snakes and serpents. She thought every one approaching her was the devil, and sprinkled what she called “holy water” about her for protection. It was clear that she was hallucinated. She heard her child crying, and she saw a woman carrying it away.
After a few weeks, Mrs. Green grew quiet and more rational except for a few spells of violence and noise; she gave the impression of a rather pleasant and agreeable, though somewhat demented, patient. Physically, beyond a tremor of fingers and tongue and lively knee-jerks and some evidence of enlargement of the heart, there was nothing to be found.
Margaret Green is still in the Danvers Hospital, being now 48 years of age. During the twenty years, she has presented,—besides the mental picture of impairment of memory—occasional spells of confusion, a variety of delusions based, at least in part, upon auditory and vivid visual hallucinations, a certain irritability and psychomotor excitement, and a picture of Huntington’s chorea. The diagnosis of Huntington’s chorea has always been in doubt by reason of the lack of any evidences of hereditary taint; it has, however, not been possible to secure a properly intensive account of her relatives.
It appears that the choreic movements were first observed—in the hospital at least—about 16 years ago. The patient has always been decidedly mixed upon dates. From internal evidence derived from her obviously in part erroneous statements, it may be that the chorea began at the age of 23. It appears that she had been often termed a victim of St. Vitus’ dance, and had had to leave her work in the mill on account of the disease. From one source of information, it would appear that the patient began to have what was called St. Vitus’ dance when she was 14 or 15 years of age; so far as this informant knew, no other member of the family had had the affliction.
The first movements observed in the hospital were irregular, jerking movements, more marked in the left arm but also occurring in the other extremities, as well as in the face, wherein were produced peculiar grimaces. The twitching movements would become decidedly worse during spells of irritability. Observation in the patient’s early thirties left the question in doubt whether the left pupil reacted to light or not. In 1904, when the patient was 36, both pupils failed to react to light either directly or consensually. At this time, the jerky movements continued, especially in the left hand and forearm, the tongue was tremulous, test phrases were poorly pronounced, the knee-jerks were exaggerated (especially the left), and both wrist-jerks were exaggerated. The systematic examination, however, revealed no other neurological disorder. Within a year, slight spurious ankle clonus developed on both sides; the eyes, especially the left, gave the appearance of developing cataracts. A slight consensual light reaction was demonstrable on the right side, but all light reactions were absent in the left eye.
At the age of 42, the patient was still disoriented for time, place, and persons and subject to a deep amnesia; was tidy, tranquil, and of a pleasant demeanor, but many of her muscles were in continual motion. There were chewing movements and both hands and feet were rarely still. There were no longer any spells of irritability or violence; and once when found crying on the piazza, Mrs. Green, on being asked the reason, replied that a gray cat had come and looked at her so hard it made her cry. There were other crying spells at times for equally good reasons, or for no reason.
More recently, the patient has become fairly well oriented for time and place, and has acquired a fairly good insight into her condition and a good memory for past events. She has had occasionally auditory hallucinations, as of water running. In 1914, it was reported that the pupils reacted to light, and the rest of the systematic neurological examination was negative except that the knee-jerks were exaggerated; and a re-examination in 1916 showed the pupils still reacted to light. At present, the patient is disoriented for time, stating that her age is about 25; she is no longer subject to auditory hallucinations; she has a marked difficulty in enunciation, emphasized by the lack of teeth and in part due to continual movements of the tongue; the movements appear to be part of a generalized chorea.
In a systematic review of the Wassermann findings in the hospital population, the blood of Margaret Green was examined and found to be positive. Lumbar puncture forthwith performed showed a positive W. R. in the fluid; there was a positive globulin and an excess of albumin; the gold sol was characteristic of paresis; there were, however, but three cells per cmm.
1. Are the choreiform movements related to the demonstrable syphilis of the nervous system? Neither the fluid W. R. nor the gold sol reaction should be regarded as necessarily an indicator of tissue loss. The fluid W. R. is commonly thought to signify merely that the fluid contains substances which are somehow due to the presence of spirochetes in some region pretty closely related with the fluid. The gold sol reaction, although well established to be characteristic of neurosyphilis, is perhaps not so strong an evidence of the existence of spirochetes in the region from which fluid constituents are derived. There is no pleocytosis. However, the positive globulin test and the excess of albumin do indicate a certain amount of destructive process somewhere in the neural tissues. Are we to suppose that these substances have been continually found during the course of this disease? This question cannot be answered with the data in hand, and we can only suspect that these positive tests for albumin and globulin are an effect of tissue destruction caused by neurosyphilis. It must be admitted that the argument here is a little tenuous. The lesson is plain: that in the present stage of our knowledge the W. R. should not be omitted even in cases which present a fairly convincing picture of some well-known entity. Thus, a disease, which looks like Huntington’s chorea, as well as a disease suggestive of multiple sclerosis, requires investigation by the methods of the syphilographer.
2. How shall we explain the changes in pupillary reaction in this case? They cannot yet be explained. A few observers have reported changes in pupillary reflexes in the direction of normality. In our experience such changes have not been noted. It cannot be too strongly emphasized that it is very easy to make errors in judging pupillary reaction if care is not used. For instance, if the patient is accommodating for near vision, light will probably not cause contraction. A frequent cause of error in testing the light reflex arises from using a weak electric light. An electric flash-light is much less efficient than daylight. Probably the most satisfactory method is to take the patient to a window, ask him to look at a distant object, shade the eye with the hand, remove hand, and observe.
3. What is the chief triad of symptoms in Huntington’s chorea? (1) Choreiform movements associated with (2) progressive mental enfeeblement, (3) occurring in a patient whose family history shows a similar condition in a preceding generation.
=Differential diagnosis between NEUROSYPHILIS and SENILE ARTERIOSCLEROTIC PSYCHOSIS.=
=Case 73.= Marcus Chatterton was a retired sea captain, 75 years of age. At the age of 71, he had had a seizure with a slight right hemiplegia and inability to talk. He had been slightly confused for a short time but had rapidly recovered. During the intervening four years, there had been three similar attacks, and the last one had caused him to come to the hospital. He was, in fact, confused upon admission but had become perfectly clear by the next day. There was a considerable memory defect, which the patient himself did not entirely appreciate. Possibly his judgment had been deteriorating slightly. He had been irritable of late and sometimes sleepless.
=Physical examination= showed a rather well preserved man with but slight senile changes. The pupils were equal and reacted readily to light and accommodation. There was no sensory disorder and no disturbance of coördination. There were no tremors. The systolic blood pressure was 205, the diastolic 135. The arteries were sclerotic upon palpation. A sufficient diagnosis would have seemed to be arteriosclerosis, and the hypothesis of syphilis would hardly have been raised off-hand by most practitioners. The W. R. of the serum was negative. What led to lumbar puncture in this case was the fact that the sea captain’s wife had died 15 years before of general paresis. The lumbar puncture was rewarding since the W. R. was positive. There was an increase of albumin and globulin, a “paretic” type of gold sol reaction, and 56 cells per cmm.
Accordingly, we must regard the condition as one of neurosyphilis. Perhaps the arteriosclerosis was of syphilitic origin. If this is a case of general paresis as we suppose, it is one of very long-standing syphilis.
1. Do delusions of grandeur in the senile period suggest syphilis? Not necessarily; it appears that there is a small group of senile cases which might be called cases of senile pseudoparesis in which extravagant delusions of grandeur are entertained, and in which frontal atrophy is found although entirely without evidence of chronic inflammation. It has not been proved that these cases are of syphilitic origin. It is suggestive that the site of the most extensive lesion is precisely the site of the most extensive lesion classically found in paretic neurosyphilis, viz., in the frontal regions.
2. Is neurosyphilis frequently found in both mates? It can hardly be said that this is a usual finding. However, it is far from rare, and it occurs frequently enough to be used in support of the theory that there is a special strain of spirochete that has a predilection for nervous tissue. It must be remembered, however, that the wives of syphilitics are frequently infected without being aware of it. In such cases they receive no treatment and consequently have a larger chance of developing neurosyphilis. It is a good rule to consider the mate of every syphilitic a candidate for neurosyphilis.
=An atypical case of recurrent dazed states resembling HYSTERICAL FUGUES. Probably an instance of NEUROSYPHILIS.=
=Case 74.= Abel Bachmann, a man of 40 years, remains doubtful and perhaps belongs to the still unresolved group of mental cases due to syphilis that cannot be placed in any of the well-known categories. Bachmann had been found by the police, working in front of a cowbarn without the consent or even the knowledge of the owner. Bachmann had, in fact, spent the night in the cowbarn and was working with the idea of paying for his night’s lodgings. The situation struck the police as so peculiar, and Bachmann was so confused and irresponsive, that he was brought to the Psychopathic Hospital. The afternoon of his admission, however, he entirely cleared up and was able to give a good account of himself.
His story was that he had been worrying a good deal about a divorce suit, and the morning of his episode he had awakened with peculiar feelings. He walked from Boston to Cambridge, feeling that he was in a strange city. He recognized the places he passed, yet they all seemed to be changed. Upon reaching Harvard Square, he determined to return to Boston and walked and walked, failing to reach Boston. All day he had eaten nothing; when night fell he stole into a field and dug out radishes. A postman stopped and said, “Hello, Bill,” which awakened him as by an electric shock. A barn presented itself, in which he spent the night. In the morning, the barn looked different. In fact, his entire surroundings appeared mysterious. As he felt like working, he went to work in front of the barn.
It seems that in his life there had been two other episodes of a similar nature; in fact, Bachmann had been in a state hospital for six weeks after the first episode. The first episode had lasted a few days only, and followed worry when he learned that the girl with whom he was in love was married. The second attack followed the death of his mother, whereupon he was taken to a state hospital although the total duration of symptoms was only three days. Bachmann had had a chancre or some other form of genital disease at 26, and had at that time been treated with mercury.
Except for irregular and absolutely rigid pupils, reacting neither to light nor to accommodation, Bachmann showed no physical and especially no neurological disease whatever. Moreover, the W. R. in the blood serum was negative.
As to diagnosis, one might consider hysteria, of which, however, there are no visible stigmata. It would not appear that brain tumor would be likely to have lasted so long as eight or nine years, even if we should attempt to make the hypothesis of tumor cover both the non-reacting pupils and the episodes. Bachmann was non-alcoholic, and there was no sign of any other form of intoxication. The spinal fluid showed a negative gold sol reaction, there were no cells in the fluid, there was no globulin; albumin was normal. However, the W. R. was strongly positive.
The situation, then, in this case is that we have somewhat peculiar psychopathic episodes, pupils rigid to light and accommodation, a positive W. R. in the spinal fluid, and extremely little else to permit a diagnosis. We are ignorant as to the course and pathology of such cases. However, we cannot resist the temptation of the diagnosis of neurosyphilis, although further classification is not ventured.
1. What is the significance of stiff pupil as an isolated symptom? Nonne finds that in the end, after years of observation, the Argyll-Robertson pupil turns out to be an advance courier of other more functionally serious signs and symptoms of neurosyphilis. We can confirm this experience and regard it as an established clinical proposition that the Argyll-Robertson pupil cannot be neglected. In this connection, refer to the case of alcoholic pseudoparesis (Murphy, 60), and also to the case of pineal tumor (Donald Falvey, 35). Enthusiastic reports have occasionally been made upon apparent restoration of the true syphilitic Argyll-Robertson pupil to normal light reaction. The difficulties in rendering the symptomatic diagnosis of Argyll-Robertson pupil in a given case are so great, and the chances of complication so numerous, that we are inclined to attach little significance at present to these claims.
It may not be amiss to mention a somewhat humorous incident familiar to some local neurologists. A case was reported by the interne for a number of months as a victim of a pupil stiff to light and accommodation, and the entirely adequate cause of this phenomenon was actually only discovered at autopsy by the triumphant medical examiner, who demonstrated that the patient in question was possessed of a =glass eye=.
=TABETIC NEUROSYPHILIS (“tabes dorsalis”) versus PERNICIOUS ANEMIA with spinal symptoms.=
=Case 75.= Mrs. Brown was a woman of 56, who for the past eight or ten years had been complaining of trouble in her legs. As she described it, at times her legs were so weak she could hardly stand; at other times there was considerable pain and numbness. She has always been considered “high strung”; that is, she had a very bad temper and lost control of herself almost entirely when she became excited. Her legs had been growing progressively worse, and for about a month prior to admission she had been unable to stand or walk. She had also lost control of her bladder. On account of her temper, it had been almost impossible to nurse her; no nurse would stay with her because of her scolding and fault-finding. Recently, she had been having fits of the blues.
Her husband, who was seen before Mrs. Brown, was an old gentleman, over 70, who was chiefly remarkable from the fact that he had unequal, irregular pupils, which reacted neither to light nor accommodation; there was also a speech defect.
The patient herself proved to be extremely irritable, as had been stated,—so much so that at times it seemed almost impossible to do anything for her. She was very querulous, constantly complaining, and not satisfied with anything that was done. Aside from this, her =mental examination= proved to be entirely negative; that is, there were no psychotic symptoms.
The systematic =physical examination= gave the following significant findings: blood pressure, 160 systolic, 90 diastolic; no evidences, however, of peripheral arteriosclerosis. Patient was unable to walk or stand, and had no control over her bladder. The knee-jerks and ankle-jerks absent on both sides; ataxia in the leg movements; loss of sense of localization, with no tenderness over the nerve trunks; no atrophy, paralyses, or muscular asymmetry of the parts. The vibratory sense was maintained. Subjectively, the patient thought that the vibratory sense differed in the legs from that in the arms. Localization, touch, pain, heat, and cold responded to correctly. The arms showed nothing abnormal; there was no incoördination, dysmetria, or dysdiadochokinesis. Her pupils were equal, regular, and both reacted normally to light and accommodation.
=Diagnosis=: The first consideration in the case is naturally tabes dorsalis, especially when one considers that the husband had signs which suggested syphilis of the nervous system. The rapid onset of the acute symptoms in this case, and the absence of the characteristic signs of pain were at least atypical for this diagnosis, as was the absence of any pupillary signs. Further, the W. R. was negative in the blood and spinal fluid; there were no definite signs of inflammatory reaction as shown by the other spinal fluid tests. These findings made a diagnosis of tabes entirely improbable. After tabes, the most frequent cause of the symptoms above enumerated is perhaps to be found in pernicious anemia. Examination of the blood showed that the patient had 2,500,000 erythrocytes per cmm. The hemoglobin by Tallquist scale was 80%. The smear was practically negative; no blasts could be seen. Although this picture is not a typical one for pernicious anemia, at least it is significant in the low number of red cells to be found, and as no causes for anemia were to be found, it seemed probable that we were dealing with a primary anemia. The diagnosis in the case, therefore, is spinal sclerosis of primary anemia. The mental picture was not uncharacteristic of PERNICIOUS ANEMIA.
1. Could the diagnosis be rendered in this case without a lumbar puncture? In the first place, the emaciation is not entirely characteristic. The pupils react normally to light. Probably such a case might well have been regarded as one of tabes dorsalis in former days, or even at the present time, if a lumbar puncture had not been resorted to.
2. Could this case possibly have been one of tabes dorsalis with negative spinal findings? Such cases have been reported frequently, but, unlike the present case, are apt to be of long-standing and non-progressive, in which the active inflammation is no longer present. The negative findings would not be consistent with tabes, in which the symptoms are of short duration and of sudden onset.
3. If the serum W. R. had been positive would the diagnosis have been neurosyphilis? We are loath to make the diagnosis of spinal syphilis when the spinal fluid is normal. Syphilis may produce a marked anemia, however, and thus produce symptoms such as shown by Mrs. Brown. It is even possible that such is the explanation of this case, taking into consideration the suggestive findings in the husband. However, there is insufficient evidence to make such an hypothesis rock firm, and we do not more than suggest it.
=Atypical case of CONGENITAL NEUROSYPHILIS—peculiar mental state.=