Part 16
4. Would it be proper to call Borofski a case of taboparesis? Absent knee-jerks in a victim of paretic neurosyphilis should not be used to suggest a diagnosis of taboparesis. This question of terminology has been discussed above, under Sullivan (16).
5. What is the mechanism by which the amnesia of a case like Borofski is produced? The answer runs in the same terms as the answer to the questions concerning the cause of convulsions. The amnesia in general paresis has surprising functionality. A study of autopsied cases of general paresis has shown that amnesia is practically as common in cases without marked destruction of brain tissue as in cases with atrophy of classical extent and depth. The clinical recovery in this case was practically complete in respect to memory. We must regard the amnesia as not due to the destruction of storage cells bearing the so-called neurograms (Morton Prince).
6. What is the explanation of the persistently positive W. R.’s of the serum and spinal fluid associated with diminished globulin and albumin tests, a negative gold sol reaction, and normal cell count? See discussion under Case Martha Bartlett (21).
7. How atypical is the early development of paretic symptoms in David Borofski? C. B. Craig has collected, in 100 cases of brain syphilis (a list including both paretic and non-paretic cases), some data on this point. The shortest period reported by Craig was in a case in which the neurosyphilitic symptoms appeared one month after infection. Craig found three cases where symptoms appeared in six months, and six cases within a year. The longest post-infective period of Craig’s list was thirty years. Our case of Chatterton (73) developed symptoms 33 years after infection and Washington (66), forty years after infection. Nonne casts some doubt on statements to the effect that tabetic symptoms may occur three to four months after infection. It seems to be admitted that pupillary anomalies and reflex changes may occur in the early secondaries and may recover under antisyphilitic treatment. Nonne’s case of longest post-infective interval, like that of Craig, was one of 30 years.
Myerson has reported a 20–year old patient who acquired chancre April 1, 1911 (spirochetes demonstrated); salvarsan was administered April 20th. There were no secondary symptoms, but in May, headache, visual disturbance, vertigo, and other symptoms developed (neurorecidive). Upon June 20th, that is, 11 weeks after development of the chancre, aphasia and astasia developed, with numbness of the left side. At this time, the pupils were slightly irregular and unequal but reacted normally. The signs in the fluid were positive. Upon this question see our cases of Bright (121) and Bennett (34).
=Hemitremor following hemiplegia in PARETIC NEUROSYPHILIS (“general paresis”). Autopsy.=
=Case 50.= Achilles Akropovlos, 39 years, had symptoms six months before commitment to Danvers Hospital. There were attacks of confusion, difficulty in walking, and speech defect, resulting in an entire incapacity to work and eventual commitment. Rather unusual and striking was a very marked tremor, apparently limited to the right side of the body. =Physically=, Akropovlos was normal, but =neurologically= he showed, in addition to the marked right-sided tremor, a marked speech defect, and a degree of ataxia. The tendon reflexes were very active, but there were no abnormal reflexes, and the pupils reacted normally. According to the history, the difficulty had followed a slight attack of apoplexy. =Mentally=, there was a marked confusion. The blood serum and the spinal fluid were both positive to the W. R.; globulin was present, and albumin was increased; there were 43 cells per cmm. There was hardly any diagnosis to make except general paresis.
Death followed 18 months later, or two years after onset of symptoms. Increasing weakness, emaciation, and dementia preceded death. Autopsy confirmed the diagnosis of PARETIC NEUROSYPHILIS.
1. What is the usual cause of death in general paresis? Intercurrent disease very frequently occurs in general paresis, and such intercurrent disease is then given as the cause of death. As a matter of fact, however, one feels that in many of these cases the intercurrent pneumonia or infection—frequently of the bladder,—bedsores, sepsis, and the like, are merely accidental incidents in a condition that is leading to death, and which has caused a lowered resistance to infection. In certain instances where nursing is exceptionally good and where no such infection occurs, the patient continues to grow weaker and weaker, paralyses of all the muscles follow and finally paralysis of deglutition or respiration may lead to death. The emaciation and paralyses may be of such a grade that the patient is entirely devoid of fat and unable to move at all. Not infrequently vascular crises occur, and one of these may be responsible for death.
2. What was the cause of the hemitremor? The hemitremor suggested an irritative or destructive lesion in the motor path. Delving into the history it was learned that the patient had had a shock followed by a right hemiparesis. This had cleared up leaving the tremor as a residuum. The autopsy disclosed a reddish-brown pigmentation and fibrous thickening of the pia over the left motor area, confirming the idea of a previous hemorrhage. As a rule the shock phenomena occurring in paresis clear up more completely and no gross lesion is visible post mortem. However, cerebral hemorrhage must be expected in any person suffering from syphilis, and is no rarity in paretic neurosyphilis.
=PARETIC NEUROSYPHILIS (“general paresis”) with NORMALLY REACTING PUPILS. History of trauma. Autopsy.=
=Case 51.= Daniel Wheelwright, a barber of English extraction, 57 years of age, had had a sunstroke at 15. At 42, there had been pneumonia, after which an attack of rheumatism was said to have kept the patient from work for a year. There was trauma of head (falling wrench) at 44. This blow on the head was the assigned cause of the mental disease, symptoms of which, however, did not develop until about the first of September, 1905, about three months before entrance, January 9, 1906, and about six months before death, March 20, 1906.
It seems that the patient had begun to change in manner; he had become despondent and apathetic, silent, and somnolent. Two weeks later, he stopped working, began to read the papers once more, and became somewhat more cheerful.
About Thanksgiving, Wheelwright got up at midnight, and remained up, lighting all the fires and talking continuously. During the next two weeks, he talked much to himself, laughing out at times. About two weeks before Christmas he went out and started to make a sidewalk of old boards, working in his shirtsleeves, without a hat. He would work until midnight making screens for windows. During the day, he would go out and give money to passing children; would offer to pay the grocer twice as much as articles were worth.
On the day before Christmas, he put out all the fires and lights in the house, sent all the family to bed, and opened all the doors. Christmas morning, he rose early and got the washtubs ready. He helped his compliant wife to do the washing, then put out all the fires and opened the windows. After Christmas, he began to tell how rich he was going to be through starting a garden and by making butter. He bought six or seven quarts of milk daily, and procured carrots and oranges, grinding them up to color the milk. January 9th he was committed to Danvers Hospital.
=Physically=, there were few symptoms. =Neurologically=, there was a tremor of tongue, fingers, and face. The knee-jerks were lively. The pupils reacted normally; the patient was restless, pacing up and down. There was a speech defect demonstrable with test phrases. Orientation was imperfect for time and for place. Handwriting was poor, memory impairment was marked, but the patient was given to fabrication as to past events. A characteristic sample of statements:
“Do you know that this is an insane hospital?” “Yes; there are two or three men here out of their heads. I could cure them with my hands but they won’t let me. I could get all the sick men on their feet just by rubbing them. I can do anything with my hands. I can build a house by just sitting down and thinking about it. I can whip all the men in this place. I have better sense now than I ever had in my life.”
Again, “How long have you been here?” “Over three months; they have put me in heaven three times since I have been here. They killed me, crushed my heart, and turned my blood to water. I am all right now. I let the sun shine on my heart and it brought it together. I can whip every man in here as fast as they come up.”
Again, “I will make a million dollars on my garden when I get it. I can make a million dollars on half an acre. I can do anything. I can move this house by just thinking of it.”
During a special examination, the patient told how he had fastened wings on his hands and feet, and how he had gone to heaven; he told how he had soared high above the earth, and how differently the stars look when up near heaven than they do from the earth. He spoke of seeing angels and of the beauties of heaven.
The diagnosis of PARETIC NEUROSYPHILIS was confirmed at autopsy.
1. What is the significance of the normally reacting pupils? While it is usual to find pupillary anomalies in neurosyphilis, these changes are not an essential part and it is not rare to find normal pupils in all forms of neurosyphilis. It is less frequent to find a normal pupil in tabetic than in diffuse or paretic neurosyphilis. In paretic neurosyphilis it is the rule to find pupillary changes during some stage of the disease, but not necessarily early. At times the pupillary sign may be one of the earliest signs of neurosyphilis—again it may occur only as a late symptom, if at all. One of the most important of the pupillary signs is irregularity of contour. While this does not always mean neurosyphilis it is highly suggestive and certainly indicates careful examination even though the W. R. in the blood be negative.
2. What was the relation of trauma to the development of the neurosyphilitic symptoms? It is, of course, the rule in all forms of mental disease to have some factor offered by the patient or relatives as the cause of the psychosis. Often these assigned causes are minor events thought of only after the later appearance of symptoms. In this case it was not thought that the trauma had any causal effect. For a discussion of trauma and neurosyphilis see cases Joseph O’Hearn (90), Levi Sussman (91), and Joseph Larkin (92).
=NEUROSYPHILIS, probably PARETIC, with symptoms highly suggestive of MANIC-DEPRESSIVE PSYCHOSIS.=
=Case 52.= Bessie Vogel[10] was admitted to the Psychopathic hospital New Year’s day, 1915, in a very much excited condition. The family history is very meagre, and all that is of significance is that mother has always been very “nervous.” The records in part:
=Past History.= Very healthy as a child, and except for occasional throat trouble and headache had no physical ailments until eight years ago, when she had an operation for appendicitis, and two and one-half years ago was operated upon for hernia and adhesions. Following this she began to show a lack of energy, neglected her housework, was much depressed, wept frequently, complained constantly of pain in various places, and was ill-tempered. In about five months she improved, and then after a couple of weeks at the shore seemed entirely well.
=Present Illness.= In November, 1914, that is, about seventeen months after the recovery from the previous depression, she again began to show practically the same symptoms. She was depressed, could not sleep, and would get up in the night and sew; was self-centered and hypersensitive, then became restless and nervous; wanted to go shopping and out for dinner; went to New York and then to New Bedford. Symptoms became more marked; she became very ill-tempered, threatened her husband when angry over trifles, threatened suicide, then began to get active and spent money extravagantly. At the end of two months, that is, Jan. 1, 1915, she was admitted to the hospital.
=Physical Examination.= A small, thin woman, appearing to be about 45 years old (actual age 37). Aside from the absence of teeth and the operation scars, the general examination is negative. =Neuromuscular system=: The pupils are round, regular, equal, and react to light and accommodation, but do not hold very well. Extraocular movements well performed, no palsies of facial muscles, tongue protruded medially without tremor. Uvula is raised symmetrically. Biceps and triceps and supinator reflexes are present and brisk. Patellar and Achilles reflexes are equal on the two sides and brisk. Abdominal skin reflexes not obtained. Plantar reflex active and flexor in type. No Babinski, Gordon, or Oppenheim. No tremors.
Wassermann reaction serum positive. Examination of spinal fluid: clear, globulin ++++, albumin ++++; cells, 130 per cmm.; small lymphocytes, 79.9%; large lymphocytes, 14.1%; polymorphonuclear leucocytes, 4.6%; plasma cells, 0.7%; endothelial cell, 0.7%. W. R. positive. Gold sol reaction, 55555522 +-.
=Mental Examination.= On admission patient showed great psychomotor activity, was very playful, marked flight of ideas, was expansive, very emotional, very erotic. She slept very little, appetite was poor, and she lost weight rapidly. Orientation and memory intact. No hallucinations elicited. In about three weeks improvement began, and at the end of eight weeks she appeared practically recovered. On April 9, 1915,—that is, 13 weeks after admission,—she was allowed home on visit. On leaving, she appeared normal in every way. There was no evidence of psychotic symptoms, she had good insight, and physically there was absolutely nothing of a neurological nature that was abnormal.
This case, with the history of a previous depression and its clinical picture during the acute stage, and its recovery, is certainly in every respect typical of manic-depressive insanity, and only the positive result of the six tests causes us to put it in the group of GENERAL PARESIS. Only the further course will shed any light as to the correct significance of these findings, and even then we shall not be too sure that we had not been dealing with a manic-depressive psychosis in a latent neurosyphilitic. We would strongly emphasize the point that at the present time this patient presents no mental or physical signs of cerebrospinal syphilis or general paresis; but the six tests are still positive. This case differs from the ordinary general paresis remission in that there is not a single physical sign of paresis present.
There are many transitional cases between this case which shows no symptoms or signs of neurosyphilis except the laboratory tests, and the typical case of general paresis. Thus we have cases with slight character change and no physical signs except rare “seizures.” On the other hand, in many cases the presence of abnormal neurological phenomena without definite mental signs is first noted. Certain remitted cases show only some slight pupillary or reflex abnormality. We believe we have here added the last link in the chain between the primary and quaternary symptoms.
This case is illustrative of several which we have published elsewhere under the name of _paresis sine paresi_ or latent neurosyphilis to illustrate how all the laboratory signs of neurosyphilis may be present in a patient without any physical or mental symptoms that may be correlated with these findings.
We summarize our discussion of this as follows:
1. There is a group of cases showing the laboratory signs characteristic of central nervous system syphilis: (_a_) positive W. R. in the serum, (_b_) positive W. R. in the spinal fluid, (_c_) pleocytosis, (_d_) excess of albumin, and (_e_) of globulin in the spinal fluid, (_f_) gold sol reaction of central nervous system syphilis, and which show no sign or symptom of neurosyphilis.
2. We believe these cases represent a form of chronic cerebrospinal syphilis, probably paretic in type.
3. They have the greatest theoretical and practical significance in the consideration of the life history of neural syphilis, in the concept of _Allergie_, in regard to results of treatment, and finally as to the evaluation of the laboratory tests.
4. Here is perhaps offered the last link to form a complete chain between the symptoms of the primary stage of syphilis and its final termination of life as the result of the diseases cerebrospinal syphilis or general paresis.
=SYPHILIS (?); EXOPHTHALMIC GOITRE; neurosyphilitic old lesion of optic thalamus; unilateral induration and atrophy of left cerebral cortex. Autopsy.=
=Case 53.= Carrie Pearson, a housewife 25 years of age, died at Danvers Hospital less than a week after admission, and it was at first stated that her symptoms had lasted but two weeks before admission. In point of fact, a further investigation showed an important succession of symptoms, lasting some four years.
Carrie had been considered a healthy child, going to school at the usual age, and progressing well with her studies. She however, left school in the ninth grammar grade, at the age of 15, and went to work in a milltown. She married a worthless person at the age of 18, and lived with her husband for three years. There was one child born a year after marriage. Two years later, however, a tremendous goitre had developed such that her neck was described as “out square with the face,” and at the same time the patient’s eyes had become prominent.
About two weeks before admission, she had gone to a neighboring town to take care of a sick woman, but during her endeavor to be a nurse, she had broken out into a mania, tearing up furniture and bedding, and talking irrelevantly for a period of four days. She also showed insomnia and continually tore off her clothing from her body.
Upon =examination=, the marked enlargement of the thyroid gland together with the prominent eyeballs, husky voice, and pulse rate of 150 per minute, were entirely consistent with the diagnosis of exophthalmic goitre. The patient described herself as “Carrie Nation.” Asked to write her name, she took the pen and tried to spatter ink, wrote hurriedly and carelessly her maiden name and several words without apparent meaning. Asked to write, “God save the Commonwealth of Massachusetts,” she wrote: “God save the common pal U S Spe Manor Gen, or til pat. Since Lord, or no prime in Hear to the God Tel. Ho. n and or Mabel, or gal.” After this, she took paper and wrote meaningless scrawls, saying that it was Japanese writing. There was much motor restlessness with distractibility, pointing and grimacing, mimicking the actions of those about her.
Death occurred from exhaustion, and the case might not have been regarded as unusual except for the autopsy, which showed a peculiar brain lesion, described below. The point of greatest interest in the case was the fact that syphilis is, although not proved to exist by laboratory tests, beyond question a factor in the case. Although the woman had given birth to a normal child, who is still alive, yet in the period of a few years her breasts had atrophied, her hair had disappeared from the axilla and from the pubes; varicose veins had developed in both legs. Whereas there was little or no fat over the chest or back, the omentum and mesentery were very plentifully supplied with fat. It is probable, then, that we are dealing with a case of exophthalmic goitre somehow of syphilitic origin. The brain lesion is consistent with this hypothesis.
=Autopsy=, March 3, 1907. Four hours post mortem.
Body length, 165 cm. Body of a well developed and well nourished young woman. Lividity in dependent parts. Purplish discoloration of left thigh to knees. Skin rough and scaly. Petechial eruption over chest. Neck thick, protrudes anteriorly. Varicose veins over upper parts of calves on both legs. Eyes protruding, not covered entirely by lids. Pupils equal, dilated. Subcutaneous fat very deep over lower part of body. Very little fat over chest and back. Breasts are very small, apparently atrophied. Normal amount of hair on head, slight amount over pubes. Axillary hair absent. Fat on section of a light yellow color. Omentum extends to pubes, plentifully supplied with fat. Large amount of mesenteric fat. Appendix normal. Intestines smooth and glistening. Slightly injected. No fluid in peritoneum. Uterus small, retroverted.
HEAD: HAIR in good quantity. SCALP normal. CALVARIUM shows diploë. DURA MATER over left cerebral hemisphere inseparably adherent to calvarium, over right hemisphere normal. Arachnoidal VILLI moderately developed. PIA MATER shows injected veins, notably in the sulci of the right hemisphere. Pia mater everywhere thin and clear. VESSELS at base of normal appearance.
BRAIN weight 1180 grams. Spread on a board, the right hemisphere tends to flatten so that it measures 1.5 cm. more from side to side than its fellow. Besides more marked venous injection, the right hemisphere shows also flatter and slightly more plastic convolutions. The posterior poles of the hemispheres are a little firmer than the parts anterior. The orbital and hippocampal gyri on the right side are a little firmer than the surrounding parts. On section the gray and white matter shows no lesions, excepting the slight plasticity of the tissues at large on the right side and a well marked induration, with retraction under the knife, of the occipital and hippocampal white matter. The basal ganglia of the left side are normal. On the right side a sagittal section demonstrates a rounded area of induration, with ill-defined borders, measuring perhaps 1.5 cm. from above downwards by 2 × 2 cm., situated largely in the lenticular nucleus and involving the greater portion of the globus pallidus, a small segment of the putamen below and behind and the regionary part of the anterior commissure with surrounding tissues. The most striking feature of this lesion is the occurrence in the middle of a cluster of vacuoles or cystic clefts, with smooth pale interiors, ranging from pinhead to 0.25 cm. or even 0.5 cm. in greatest diameters. There are six to eight clefts to a surface of section. The color of the lesion differs little from that of the globus pallidus itself, but the tissue is a trifle translucent. It is impossible to demarcate the lesion with the eye. Induration is demonstrable several mm. beyond the visible part of the lesion. The consistence of the lesion slightly surpasses the usual consistence of the olivary bodies.
CEREBELLUM, PONS and BULB weight 165 grams. Cerebellar tissue a trifle more plastic than usual. The right olive is not so prominent as usual.