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.