Part 19

The =physical examination= was quite negative except that =neurologically= there was lingual and manual tremor, a speech defect, apparent only with test phrases, unsteadiness of handwriting, left knee-jerk greater than right, a left-sided Babinski reflex, and a difficulty in executing rapid successive movements (dysdiadochokinesis). This degree of neurological disorder in our experience warrants lumbar puncture as well as a serum test. The lumbar puncture shortly disclosed a positive globulin and excess albumin, and the returns from the W. R.’s were positive for both spinal fluid and blood serum. The data of the gold sol reaction were not available on account of technical difficulties. However, it appears that the diagnosis of neurosyphilis could hardly be avoided in this case.

David Collins differs from Francis Murphy, then, in showing a positive blood and spinal fluid reaction for syphilis as well as a positive globulin and excess albumin. As above remarked, it is probable that the positive globulin and excess albumin would not warrant more than a suspicion of neurosyphilis taken by themselves.

Unfortunately, we were unable to persuade the patient to submit to treatment, and from the patient’s point of view possibly his decision, not to submit to treatment, was a good one since he has had no symptoms of any sort for a period of 18 months since his episode. However, as abundantly elsewhere demonstrated, we feel that the patient is wrong, and that the physicians are right in urging treatment.

1. Is not the convulsive episode an alcoholic phenomenon in David Collins entirely separate from the patient’s general and neurosyphilis? Possibly; however, an outbreak of neurological symptoms with spontaneous recovery is not only consistent with the diagnosis of syphilis, but somewhat characteristic of neurosyphilis. We suspect that another attack will occur in David Collins.[12] We shall from time to time make use of the social service to suggest his going under treatment, and shall employ his record of contact with a public institution to drive in our suggestion. Still it is clear that there are numerous cases in the community that are not accessible to social service initiated from a public institution. Accordingly, educational propaganda is necessary for salvage of the middle- and upper-class victims of syphilis. It is a little unfortunate that the ethics of the private practitioner make such salvage of middle- and upper-class persons not very likely. Might it not be that an extension of state medicine to this field would incidentally increase the amount of successful private practice?

2. What may be the cause of such a convulsive episode as that of David Collins? It would appear that the convulsions of general paresis and of neurosyphilis in general often occur without gross structural lesions of the brain. It may be suggested that vascular irritation or parenchymal irritation by spirochetes, acting in appropriate parts of the central nervous system, can produce such convulsions.

3. What is the significance of the unilateral phenomenon in David Collins (left knee-jerk greater than right; left-sided Babinski)? The current explanation of hyperreflexia is that somehow inhibitory impulses from upper portions of the nervous system have ceased to influence the local arcs that mechanize reactions like the knee-jerk and the normal plantar reflex. The phenomena are commonly found in cases with pyramidal tract disorder, and in the case of David Collins one may suspect, therefore, that there was a central disorder affecting the right pyramidal tract above its decussation. One might suspect that the convulsions were initiated by a lesion (whether gross or microscopic in range) in the right side of the cerebrum; but whether in the white matter or in the gray matter must be left doubtful. The clearing up of all symptoms suggests either that the lesion was microscopic in range or that the phenomena were transient and functional.

4. Can the dysdiadochokinesis be used to indicate cerebellar lesion in David Collins? Possibly; but it does not appear that the difficulty in executing successive movements was unilateral. It seems impossible to bring into close topographical relation the basis for the Babinski and left-sided hyperreflexia, and the basis for the dysdiadochokinesis. Alcohol is sometimes asserted to exert an especial effect upon the cerebellum.

5. Must we suppose structural lesions, either (a) of the nature of cell losses demonstrable microscopically, or (b) of the nature of secondary degenerations demonstrable by Weigert myelin sheath methods, in the case of David Collins? It appears that we do not need to assert the existence of such lesions.

6. Could the hyperreflexia and the Babinski reaction be due to local spinal cord disease? Possibly; but the existence of other neurological symptoms (lingual and manual tremor, speech defect to test phrases, ataxic handwriting, and dysdiadochokinesis) makes it probable that there were lesions, or at any rate disordered functions, within the cranium; and there appears to be no basis for asserting local spinal cord disease.

=Differential diagnosis between NEUROSYPHILIS and ACUTE ALCOHOLIC PSYCHOSIS.=

=Case 62.= Joseph Buck was a chef of 60 years who came in, seeking advice because his memory was getting poor; he was unable to remember names and what he was about to do. He was tremulous and had much pain in his limbs. He had been drinking heavily for weeks,—probably ten weeks; in fact, he described himself as having had “the shakes” and as having lately seen animals and people that were unreal. He had had the shakes before and the condition had lasted for two to three days after alcohol was discontinued.

=Physically=, Buck was tall, well developed, although poorly nourished, with a skin suggesting alcoholism. There was a slight acne over the back and chest; there was a slight enlargement of the heart, with blood pressure, systolic, 180, diastolic, 120. There was a corneal opacity of the left eye, which the patient said was the result of syphilis following a chancre, which he had acquired at the age of 27. There was also a ptosis of the upper lid of the left eye. The right pupil was irregular and reacted to light sluggishly, and with a very small excursion. The patient was slightly deaf in both ears. The deep reflexes were all lively and equal. The tremor was most marked in finely coördinated movements. There was a slight swaying in the Romberg position but the sign could not be said to be present. The gait was unsteady. There was a marked tenderness over the nerve trunks.

So far as =mental examination= went, it seemed that the patient’s claim of amnesia was subjective. There was certainly no more amnesia than a slight difficulty in recalling details. The diagnosis of alcoholism with convalescence from delirium tremens would certainly seem to have been sufficient for the phenomena, and the suggestion of alcoholic neuritis only confirmed the picture. To be sure, one might expect a diminution or absence of deep reflexes; still, these reflexes may be overactive in an irritative stage of the disease.

Naturally, however, the history of syphilis and the pupillary phenomena and ptosis, made the consideration of neurosyphilis necessary. Both serum and fluid W. R.’s proved positive; there was an excessive amount of albumin and globulin, the gold sol reaction was typically “paretic,” and there were 377 cells per cmm.

The patient improved upon a rest treatment and was given injections of mercury for his syphilis. After a few months he felt well enough to return to work, and continued at work throughout a season, receiving mercurial treatment throughout this time. A spinal fluid examination fifteen months later showed a weaker gold sol reaction, reduction in the amount of globulin and albumin, and but 26 cells to the cmm. The W. R.’s had remained positive.

1. What are the forms of syphilitic neuritis? According to Nonne, syphilitic neuritis and polyneuritis have at last acquired standing in neuropathology. The older claims depended upon findings on palpation and recovery after antisyphilitic treatment. Since the introduction of salvarsan, cases of ophthalmoplegia, facial, acoustic, and optic nerve disease, as well as neuritis of the extremities, have been reported in large numbers. These phenomena are to be regarded as neurorecidives in the modern sense of that term. The neurorecidive is not a salvarsan effect, but is an effect of the syphilitic process itself, settling in the peripheral nerves. Paresthesias are especially prominent in peripheral mono- or polyneuritis, and this point is of some value in differentiating the syphilitic peripheral neuritis from root neuritis. Root neuritis is more often characterized by neuralgic attacks. Objective hyperæsthesia of neuromuscular origin is also found in these cases, demonstrated by pressure on the nerves. The motor phenomena consist in a flaccid paresis or paralysis, especially affecting the radial, ulnar, and peroneal nerves. Nonne states that it is rare for syphilis to affect a single nerve region, and he regards cases in which a single region alone is affected as usually due to a local gummatous process.

2. What is the significance of 377 cells per cmm.? See discussion of Washington (Case 66).

=Differential diagnosis between NEUROSYPHILIS and CHRONIC ALCOHOLISM.=

=Case 63.= Albert Fielding, 46, was an insurance broker, who was brought to the hospital for excessive alcoholism. Indeed, he showed all the signs, both of chronic and acute intoxication, except that there was no nerve trunk tenderness. Fielding was very loquacious though his speech was rather thick. He showed tremor of hands and an alcoholic skin. Physical and neurological examination proved entirely negative.

Fielding claimed that he had had a nervous breakdown at about 36 years of age, after disappointment in love. He had the drinking habit and began to drink more and more. He had now become nervous and tremulous and had to drink in order to brace himself. After a few days, the patient began to be much better, having recovered from acute alcoholism. =Mental examination= now showed good memory with orientation intact. There was a certain tendency to reminiscence and to somewhat childish actions. He had attempted to stop drinking but had been unable to quit. As a matter of fact, his mother and father had been excessive drinkers and he had inherited the tendency, etc.

The =diagnosis= seemed to be plain. The routine W. R. upon the blood serum was negative. However, the patient had remarked during the history taking, that he had had a chancre and secondary symptoms of syphilis. Accordingly, lumbar puncture was resorted to. The fluid showed a slightly positive W. R.; the gold sol reaction was of the syphilitic type; there was a considerable increase in albumin and globulin, and there were 20 cells per cmm. The diagnosis of neurosyphilis seemed clear.

=Course=: The patient received six months’ treatment in a sanatorium but the symptoms remained almost as before, and the patient showed the same childishness and inability to take care of himself. Since the symptoms continued six months after the withdrawal of alcohol, it might well be suspected that the condition was more than a merely alcoholic one. However, in a number of purely alcoholic cases, such long-standing effects are found: even as long as six months or longer after the withdrawal of the alcohol, and one might conclude therefore that Fielding was actually a victim of alcoholic dementia. The spinal fluid after these six months (during which period antisyphilitic treatment was given) showed no change, and the prognosis was offered that the case would probably develop into one of paresis.

A year later, after six months sanatorial care and six months life in the community, the patient returned to the Psychopathic Hospital in an alcoholic condition. The lumbar puncture showed all signs negative except the W. R. which was slightly positive. The W. R. of the blood was negative.

In connection with this case, see the case of _paresis sine paresi_ (25).

1. What is the relation of the syphilitic and alcoholic process in Robert Fielding? One does not like to break the so-called rule of parsimony in diagnosis, but it would seem that the effects in Fielding are the combined effects of syphilis and alcoholism.

=Differential diagnosis between NEUROSYPHILIS, DIABETIC PSEUDOPARESIS and BRAIN TUMOR.=

=Case 64.= A large and imposing person, Calvin Hall, 55, had been employed as a doorkeeper and guard, in which position he was on duty for 12 to 14 hours daily. Eventually, however, he had begun to have a good deal of pain in the legs and a few months before observation, one day, his legs gave way and he fell to the floor. There was, however, no loss of consciousness, and he was carried to a general hospital. The result of an examination there was that his family was informed that he had some nervous trouble.

Hall now began to be melancholy and wept a good deal. His appetite and sleep remained intact. He felt too weak to walk. At the end of about a year, he began to improve and again became able to do a little light work. About a month before coming to the Psychopathic Hospital, about two years after the onset of symptoms, Hall suddenly began to talk excessively, in a rambling and rather senseless way. A fortnight later, he began to suffer from insomnia and restlessness.

Some medical facts were available: It seems that at 25 years this patient had become infected with syphilis though there had never been any secondary signs. He was married four years later but there had not been any children. Moreover, for four years past, the patient had been treated for glycosuria.

Upon admission, the patient’s sensorium was clear, but his orientation was only partial. He could give a fair account of his life, but it appeared that his memory was somewhat impaired. There were auditory hallucinations (voices of relatives). He often mistook the identity of persons about him. He talked in a grandiose fashion of his great strength and especially of a God-given power to read minds. His flow of thought was rapid, rambling, circumstantial, and with traces of irrelevance. He was rather continuously busy and at times restive. There was a good deal of emotional agitation and apprehensiveness, and again the patient would become suspicious and tearful.

=Physically=, there was a discharging sinus connected with the right humerus, close to the elbow. The pupils, though equal and regular, were sluggish in reaction to light. The knee-jerks and ankle-jerks were absent. There was no Romberg sign but there was some swaying in the Romberg position. There was a moderate ataxia in walking. Glycosuria to a moderate degree was determined. There were no casts or albumin in the urine. The W. R. of the blood and of the spinal fluid was negative. The albumin of the fluid, however, was considerably increased. X-ray examination of the skull yielded a suggestion of absorption of the posterior clinoid processes of the sella turcica. The X-ray examination of the arm in the region of the sinus showed a chronic osteomyelitis, possibly syphilitic (or diabetic?).

The diagnostic problems in the case of Calvin Hall are extremely intricate. There are clinical suggestions of general paresis, not confirmed by the laboratory findings.

1. Are we dealing with a case of diabetic pseudoparesis? Is the pain in the legs of like origin, and has a neuritic process led to the absence of the knee-jerks? The Allen treatment appears to have had no beneficial result in this case.

2. Is there a tumor of the sella region, which could account for the mental symptoms and the glycosuria? The spinal fluid albumin might be regarded as consistent with a variety of psychoses, including that of brain tumor. We have to remember the definite history of infection, the sterile marriage and the possibly syphilitic osteomyelitis.

=DIABETES AND NEUROSYPHILIS, relations?=

=Case 65.= Donald Barrie, a man of 61, diabetic for several years, had begun to worry about the diabetes, feeling that he was about to die, and had gone so far as to make several threats of suicide. Hence he was brought to the Psychopathic Hospital for observation.

Barrie was rather well developed and nourished, although he looked far older than he was. There was a marked arcus senilis; the skin was dry and rough; the radial and other accessible vessels were markedly sclerosed; abdomen obese; right testicle very low with thickened and hard epididymis.

=Neurologically= there was little abnormal to discover. The pupils were irregular; both reacted fairly well to light. There was a slight tremor of the extended hands, and still less of the tongue. The voice was slightly thick and the patient stumbled somewhat on test phrases. Urine: specific gravity, 1029; sugar; no acetone; no diacetic acid. Sugar 2 to 11 grams for 24 hours on ordinary diet. It proved impossible to get the patient sugar-free, either by cutting down the carbohydrates or by using the Allen method.

=Mentally=, the depression with reiteration of wrong-doing and self-accusation because of the contraction of syphilis, were the striking features. There was, to be sure, a slight imperfection of memory for remote events; memory for recent events and knowledge of current events was very poor. Barrie claimed that his condition was entirely hopeless, that his memory was exceedingly bad, and that he was no longer capable of supporting his family.

1. What shall be said as to diagnosis in a man of 61 with glycosuria, depression, amnesia, sluggish pupil, slight tremor, slight speech defect, and a history of syphilis? The W. R. of the serum proved positive, and also the W. R. of the spinal fluid. The gold sol reaction of the fluid was of the syphilitic type. There were 112 cells per cmm., there was an excess of albumin, and a large amount of globulin. Accordingly, the diagnosis of PARETIC NEUROSYPHILIS (“general paresis”), especially in view of the laboratory findings, seems necessary.

2. What is the cause of the glycosuria? First: possibly it has no relation with the syphilis; secondly: it may possibly be due to a syphilitic involvement of the pancreas; thirdly: it is barely possible that it is due to syphilitic disease of the fourth ventricle or of the base of the brain, involving the pituitary region. Perhaps our case is too complex for analysis. At all events, the case brings up the possibility of a syphilitic glycosuria.

3. Can the diabetes in the case of Barrie be explained as syphilitic? Warthin of Ann Arbor has recently described somewhat remarkable spirochete findings in his autopsy material. The order of organic infection according to frequency is: aorta, heart, testis, adrenal, pancreas, nervous system, liver, and spleen. Warthin has called attention to the relation of pancreatitis and spirochetosis to diabetes in a recent review[13] of findings in 41 autopsied cases from the University Hospital in Michigan. Warthin found active luetic lesions in the pancreas in 6 cases.

=Hemianopsia in a case of neurosyphilis.=

=Case 66.= Lawrence Washington, a colored cabman, 58 years of age, began to forget addresses given him by his fares. Moreover, he could no longer see as well as before, especially on looking toward the right side. He himself states that the visual trouble dated back as long ago as his 39th year, at which time he had a terrific pain in both temples, leading back from the eyes. Washington thought that his vision had been getting slowly but steadily worse ever since.

We got the impression that the amnesia claimed by Washington was more or less subjective and he was found to be well informed. This association of amnesia and impairment of vision naturally suggests syphilis. The patient himself stated that he had had a chancre at the age of 18.

We found the W. R. of the serum to be appropriately positive. The W. R. of the spinal fluid was also positive though weakly so. There was an excess of albumin; globulin appeared in large amount; the gold sol reaction was of the syphilitic type; there were 186 cells in the spinal fluid.

Is this case one of paresis or of some other form of cerebrospinal syphilis? Let us consider the data of the =physical examination=. On the whole, the patient was well preserved. There was a slight radial arteriosclerosis, but on the whole the cardiovascular system was almost negative. The blood pressure was 100 systolic, 65 diastolic. =Neurologically= the visual field of the left eye was somewhat limited, and there was a temporal hemianopsia of the right eye. The ophthalmoscopic examination showed a disseminated choroiditis on both sides. The right pupil failed to react to light. The left pupil reacted slowly. Both pupils reacted properly to accommodation.

The knee-jerks could be obtained only on reinforcement, and when obtained, the right was apparently more active than the left. The left Achilles was absent; the right present. There were no other abnormal reflexes.

The motility of the facial muscles was somewhat impaired. Finger-to-finger and finger-to-nose tests were rather poorly done. The muscle sense was good; there was no swaying in Romberg position; and there was no speech defect.

We are unable to decide whether the case is one of the =parenchymatous= type (paretic) or of the =meningovascular= type of =neurosyphilis=. It is certainly rather unusual to find hemianopsia in a paretic.

We have been unable to get definite results from the treatment of this case, since the patient would not return for months after getting an injection or two of salvarsan, on the ground that he was improved enough and did not require further treatment.

1. What conclusion can be drawn from the 186 cells per cmm. in the spinal fluid? Ordinarily this finding would indicate an active process. Some writers have claimed that a cell count running above 100 per cmm. was an indicator of diffuse non-paretic neurosyphilis. It does not appear that this claim has been substantiated. It is remarkable that this case shows an interval of 40 years between infection and the occurrence of definite clinical symptoms. With respect to the cell count, both in untreated and in treated cases, the following conclusions from a recent article (Solomon and Koefod)[14] are in point:

1. The number of cells found in the fluid of untreated cases offers no definite information of prognostic value.

2. One is not justified in drawing any conclusions as to whether the case is cerebrospinal syphilis or general paresis, nor the time the process has been active, nor the severity of it, from the cell count.

3. The cell count may vary greatly from month to month, or when the interval is but several days, while at other times it may remain very nearly the same after an interval of months.

4. Cases showing natural remissions may show no reduction in the cell count, or other spinal fluid findings.