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.
5. Cases treated with salvarsan, either intraspinously or intravenously, tend to show a more or less rapid fall in the cell count. This count will, as a rule, remain low during treatment, but is likely to rise when treatment has been discontinued, but may rise during treatment after having first fallen.
6. Cases may show remissions during treatment and still have a pleocytosis.
7. Treated cases having the cell count fall to normal may at the same time become very much worse and develop more marked paralytic symptoms.
8. In general paresis the cell count in no way parallels the other spinal fluid findings.
9. In cases in which the other tests show an improvement, for instance cerebrospinal syphilis, the cell count also readily and early drops to normal. At times it may drop to normal before other spinal fluid tests become negative; again it may be last to reach normal.
10. The change in cell count seen in syphilitic disease untreated is also found in non-syphilitic diseases, as brain tumor.
11. The cell count offers nothing of prognostic importance in syphilis of the nervous system unless accompanied by improvement of the other laboratory signs.
12. The cell count is not an index to the predominance of irritative or degenerative changes.
Case of CEREBRAL MALARIA and SYPHILIS: simulation of PARETIC NEUROSYPHILIS (“general paresis”).
Case 67. Joseph Temple, 45, who had been a sea-going steamboat steward, was brought to the hospital in a semi-stupor. He was entirely uncoöperative, often resistive, attempting to bite the physician’s fingers, and for the most part lying curled up. He was incontinent and tube-fed. This phase, it seems, had begun the night before entrance to the hospital. Twenty-four hours later, an extraordinary change was noted. Temple became alert and attended to his wants, began to eat well, and began to behave as normally as probably he ever behaved.
He was now able to give a coherent history. It was now January. In the previous September, he had left for Mexico; he was returning when he suddenly fell to the deck, unconscious. After this fall, he had not been well, having had chills and fever. At the Marine Hospital, he had been diagnosed as suffering from malaria, and was given quinine. He had been delirious a short time in the hospital, not being able to recognize his wife, who called. He shortly improved so that his wife was able to take him home. Nevertheless, headache, gastric distress, and intermittent vomiting continued. A spell of confusion took place, two days before admission. The patient tossed about, moaned, and failed to recognize anyone. Malaria of the æstivo-autumnal type was demonstrated in the hospital. The temperature always remained at normal. He was somewhat emaciated and pale. The pupils were small, somewhat unequal, and reacted though poorly to light and distance. The tendon reflexes were lively.
The W. R. of the serum was positive, and information from the patient’s physician runs to the effect that there was a syphilitic infection some seven or eight years ago, followed by secondary symptoms, but the patient had refused to take any protracted treatment. The spinal fluid examination was practically negative.
Mentally, the patient was euphoric, expansive, boastful, and showed a marked emotional instability and considerable memory defect.
1. Can the diagnosis of general paresis be made in Joseph Temple? Certainly the acute confusion and the syncope are consistent enough with the diagnosis, yet the severe malaria makes it seem likely that the phenomena were due to a cerebral attack of malaria, and such occurrences are found in the æstivo-autumnal form of malaria. Yet malaria would hardly explain the euphoria, memory defect, and the pupillary findings, to say nothing of the irritability and the active tendon reflexes. Even if we regard the active tendon reflexes and the irritability as malarial, the other phenomena remain outstanding as exceedingly suspicious of paresis.
On the other hand, if we try to support forcibly the diagnosis of general paresis, we are hardly able to explain the negative findings in the spinal fluid.
In point of fact, a study of the patient’s past life revealed a story that the mental traits of euphoria, irritability, and memory defect had been characteristic of the patient for many years. In fact, there is some question whether the patient is not really to be regarded as a moron of high grade.
Upon this basis, if we regard the confusional phenomena as malarial and the persistent mental phenomena as characteristic of a moron and somewhat exaggerated by the disease, we have merely to explain the suggestive pupils. As to these, it must be remembered that though they reacted poorly to light, still they reacted somewhat, so it is not a question of explaining an Argyll-Robertson pupil, but only an impaired pupillary reaction. Of course, some workers are of the opinion that pupillary changes, perhaps even the Argyll-Robertson pupils, may occur in syphilitic cases that are not neurosyphilitic, or at all events are not victims of central neurosyphilis. Finally, we must remember that there are cases of neurosyphilis of a vascular type which yield negative spinal fluids. The case leaves many questions unanswered.
Can paretic and non-paretic neurosyphilis be differentiated by means of the gold sol reaction? The gold sol reaction in this case was an extremely mild one and would not at all have warranted the diagnosis GENERAL PARESIS, yet the discovery of a heavy meningeal exudate including an unusually heavy deposit of plasma cells even in the spinal pia mater will perhaps warrant us in making a final retrospective diagnosis of paretic neurosyphilis. Autopsy.
Case 68. We would like to give the full effect of our surprise at the outcome of the case of Margaret O’Brien, a school-teacher, 26 years of age. To be sure, Miss O’Brien developed symptoms at 22 or 23 which we can now explain consistently with the outcome of the case; for at that time, she began to complain of severe pain in the head, especially in the forehead and temples, and also became nervous, unable to remain quiet, and given to insomnia. She was markedly depressed at the time and would refuse to talk at times. However, only the headache in this prodromal period could be regarded as particularly suggestive of syphilis, and headache in an over-worked school-teacher is not uncommon.
In fact, the picture presented by the patient was one of catatonic dementia praecox. The patient was admitted to the hospital after a sudden onset of excitement. At first she was very restless, continually looking about and getting up and walking away from the examiner, giving the impression of understanding all questions but preserving an air of indifference. A few days later, the patient was gotten to answer more coöperatively. She remarked that the hospital was heaven although in Boston; that it was summer time (correct) and that her memory was greatly impaired. The physician was a messenger of God (delusion later corrected). The patient had not done God’s will; her breath was leaving her; God’s voice was heard from time to time, and Miss O’Brien had heard it for a long time. God tells her to do His will. However, as Miss O’Brien remarked, “I must think all this nonsense, turning against God.”
The patient frequently attitudinized and would remain in an apparently catatonic condition for many minutes. For the most part, she was resistive and mute and non-coöperative as to examination. From time to time, she made impulsive suicidal attempts. So far as a somewhat inadequate physical examination was concerned, nothing abnormal could be made out; in particular, the pupils reacted normally to light and were otherwise normal. The routine W. R. of the blood serum, however, returned positive, and in accordance with the policy of the Psychopathic Hospital, the patient was subjected to a lumbar puncture. The lumbar puncture yielded a positive W. R., 109 cells per cmm., a positive globulin and a considerable excess of albumin, and an exceedingly mild gold reaction—syphilitic type.
Ten days after admission, the patient had a convulsion. She never regained consciousness, continued to have convulsions for a few hours, and died, apparently from paralysis of respiration. The heart continued to beat for a short period after respiration ceased. The autopsy was consistent with the diagnosis which had been rendered after the surprising results of the W. R. in the blood and the laboratory findings in the spinal fluid had been learned. There was a generalized encephalitis with congestion of all the smaller cerebral vessels and petechial areas in the meninges and upon the cortical surfaces. We regard the case as one of syphilitic encephalitis.
The brain weighed 1265 grams, indicating a loss of 79 grams by Tigges’ formula (8 times the body length in centimetres). The pia mater was, in the gross, quite normal within the cranium; nor were any cells found in a smear from this pia mater; but the pia mater over the spinal cord was visibly edematous, and a smear from the spinal pia mater showed great numbers of lymphocytes and especially of plasma cells—a finding which was confirmed in stained section, by which a remarkable display of plasma cells was found plastered somewhat generally over the entire pia mater of certain segments. The brain substance was softer than normal, but displayed no differences of consistence. The stripping of the pia mater of the temporal lobes on both sides yielded the so-called “decortication” (that is, the adhesion of small bits of brain substance to the pia mater). The optic nerves were somewhat thinner than normal. No other gross lesions of the brain were found.
The dura mater, although dense and injected, was not otherwise abnormal. There was an early visible sclerosis of the middle meningeal arteries, more marked on the left side.
The cause of death, so far as the autopsy revealed it, was bronchial pneumonia. There was a diffuse nephritis.
1. Are the hallucinations in the case of O’Brien characteristic? Hallucinations are regarded as playing a minor rôle in general paresis. In fact, earlier workers sometimes denied that hallucinations occurred at all, and this denial has been made once more of late by Plaut,[15] but Kraepelin quotes Obersteiner as observing hallucinations in 10%, and regards that figure as approximately corresponding with his own experience. Junius and Arndt are cited as finding 17% of their cases hallucinated. Auditory hallucinations are somewhat more frequent than those of vision (alcoholic psychosis must be considered). The visual hallucinations of paresis are thought by Kraepelin to be related with atrophy of the optic nerves, and he states that they occur by preference in patients having such atrophy. Hallucinations though not common are more frequent in non-paretic neurosyphilis than in paretic neurosyphilis.
2. What was the cause of death in Margaret O’Brien? The autopsy, as above stated, indicated pneumonia. In point of fact, this patient developed convulsions and ceased respiration, the heart continuing to beat for some time after respiration had ceased. It may be that the death should be counted as one of neurosyphilitic seizure.
Tonsillar abscess associated with neurosyphilis (Lues Maligna?).
Case 69. Frank Mason, 49 years, a rectifier of spirits, was admitted to the Psychopathic Hospital in a tremulous, mentally confused, depressed, and unhappy state. He was particularly concerned because he could not give an accurate account of his past life and because he found that he was continually contradicting himself.
Superficial examination shortly discovered the pupils to be much contracted, irregular, and non-reactive either to light or distance. Although these pupils showed more than the Argyll-Robertson phenomenon, yet the suspicion of syphilis was important.
Throat examination showed a large area of ulceration involving the whole of the right tonsil and extending even to the left side of the median line so that the whole of the faucial pillar was involved. In the midst of this ulcerative area was a mass of purulent necrotic tissue, about which the edges of the ulcer stood out sharply. There was, however, very little acute reaction about the margin of the area.
The association of pupillary changes (especially stiffness to light), what looked like tonsillar gumma, and mental disorder (including memory disturbance) heightened the impression of syphilis.
However, the remainder of the examination was not especially confirmatory of the diagnosis. The man was well developed and obese, with a slightly enlarged heart, with sounds of poor quality and the aortic second sound accentuated. The systolic blood pressure was 130; the diastolic, 90. There was no disorder of reflexes except that the arm reflexes were very lively.
After a time, a few facts concerning the patient’s life became available. Although a rectifier of spirits, Mason could not be found to have over-indulged in alcohol. It appears that some five months before his admission to the hospital, a wisdom tooth had been extracted. About four months before admission, the ulceration of the faucial pillar had begun, and this ulceration was immediately laid to infection from the wisdom tooth cavity. Mason then had to discontinue work and a depression followed. But the account of this depression led us to think that he was a victim more of natural sadness than psychopathic depression. There was much worry and insomnia. To meet the insomnia, large amounts of hypnotics were administered. The sequence of these hypnotics was a tremendous disturbance and continual crying out by the patient. In fact, Mason became so excited that he was removed to the Psychopathic Hospital for temporary care in the condition above mentioned.
We naturally awaited the outcome of the serum W. R. The return was negative. However, the typical position of the ulcerative lesion and the non-reacting pupils,—to say nothing of the mental symptoms and the associated tremors, with incoördination (this incoördination was non-characteristic and apparently due largely to the tremor),—led to lumbar puncture.
The spinal fluid yielded a weakly positive W. R. There was a slight positive albumin, the globulin test was slightly positive, there were 14 cells per cmm., and the gold sol reaction was of the syphilitic type. We were, then, probably entitled to conclude that syphilis was active not only in the body at large but also in the nervous system. Looking back upon the case, we considered that large doses of morphine and hyoscyamus might well have produced the marked mental confusion and possibly the tremors that characterized Mason on his arrival at the hospital.
Improvement followed after a few days of rest; the confusion disappeared and the tremors diminished; the pupils returned to their normal size and reaction; depression persisted, and the patient was very properly much concerned about the tonsillar lesion. However, further improvement did not take place under antisyphilitic treatment and patient died after several weeks from what was believed to be an embolus from the tonsil.
1. What was the true interpretation of Frank Mason’s pupillary changes? They were probably due to the opiates, despite the fact that, taken in association with the gummatous lesion of the faucial pillar, we had regarded them as possibly syphilitic.
2. How shall the negative serum W. R. be explained? Such a reaction is consistent with the diagnosis gumma. It is, however, a little surprising that with active neurosyphilis and a relatively active non-nervous syphilitic lesion like that in this case, the serum W. R. should have been negative. Possibly a repetition of the test at various times would have shown a positive serum W. R. In any event, the fluid reaction was positive.
3. Could the tonsillar ulceration be due to dental infection? The chances are against this on account of the interval (2 months) between extraction of the wisdom tooth and the ulceration, which itself seems to be of a tertiary syphilitic nature. In point of fact, the patient admitted a syphilitic infection 21 years previously namely, at 28 years of age. At that time he took large quantities of mercury and potassium iodid by mouth.
4. Relation of the case of Frank Mason to the so-called lues maligna? The case closely resembled the cases reported by Bly. Frank Mason showed great destruction of tissue, toxemia, failure to react to antisyphilitic treatment. In both of Bly’s cases, the tonsil was the starting point of the illness; and in both cases there was a trauma of the tonsil or peri-tonsillar structures (tonsillectomy and application of caustic). In our case there not only had been extraction of a wisdom tooth, but the tonsil had been cauterized.
Neurosyphilis versus multiple sclerosis.
Case 70. Annie Kelly is a young Irish woman, 21 years of age, who was perfectly well until three months before her admission to the Psychopathic Hospital, when suddenly one evening she became very dizzy. This was followed by a chill and vomiting. The next day she had a sore throat but was able to be about and do her work. The dizziness, however, continued and she began to feel rather queer. Gradually it became difficult for her to walk on account of staggering.
A little later she noticed a weakness of the left side, involving face, arm, and leg; then she began to find it difficult to talk. Finally the right leg became weak, making walking practically impossible. All these symptoms grew worse and the dizziness increased. At times her vision would be blurred; there were somewhat frequent attacks of diplopia. Finally she had to take to her bed, and at last she lost control of her sphincters.
At no time did she suffer any pain. She was taken to a hospital, and after a time improved somewhat; but she was told she had a brain tumor and had better be in a large city, where she could have surgical aid if this became necessary; consequently, she was brought from Montana to Boston.
On admission to the hospital, the examination disclosed no important symptoms outside of the nervous and locomotor systems. She was unable to walk unless assisted. The pupils were large but reacted well to both light and accommodation, were equal in size, and regular. Slight nystagmus was present; there was no ptosis or strabismus; vision in the left eye was poor. The other cranial nerves showed no involvement. The tendon reflexes were all present and very lively; Babinski, Gordon, and Oppenheim signs were present on either side. The ataxia was marked, especially of the lower arms, and she had some difficulty in the alignment of the fingers. The sense of position of the limbs was very poor. There was some tremor, which was not of the intention type. The writing showed some incoördination. The speech showed nothing abnormal. Mental examination disclosed nothing of note objectively, but patient stated she could not think so clearly as she could formerly.
The diagnosis would seem to lie between brain tumor,—which had been suggested to the patient by her physician,—multiple sclerosis, and neurosyphilis. The numerous neurological symptoms without any definite evidence of intracranial pressure were sufficient to rule out for the moment the consideration of brain tumor. The syndrome of multiple sclerosis is not complete, but the race, age, and onset, with the increasing and decreasing intensity of symptoms are very suggestive of this diagnosis. The symptoms, of course, are all consistent with neurosyphilis also, and while the patient denied any knowledge of syphilitic involvement, the examination of the blood and spinal fluid was made. The W. R. was negative in both the blood serum and spinal fluid. Further examination of the spinal fluid showed presence of globulin and an increase in the albumin content, 43 cells per cmm. and a “paretic” type of gold sol reaction. With the negative W. R. of both blood serum and spinal fluid, and with so much in favor of Multiple Sclerosis, this diagnosis was made.
1. What is the relation of multiple sclerosis to syphilis? There is no definite relationship between multiple sclerosis and syphilis,—that is, multiple sclerosis is not a syphilitic disease; but the complete syndrome of multiple sclerosis is often given by a syphilitic involvement of the central nervous system (see case Lauder, 71).
2. Is the spinal fluid finding in this case consistent with multiple sclerosis? According to Nonne, about 19% of the cases of multiple sclerosis show globulin and pleocytosis in the spinal fluid. As a rule, the number of cells ranges between 10 and 20 per cmm. and the globulin is not present in large amounts. In this case, the amount of globulin, which was given as 2+, is only a moderate amount,—less than is usually found in cases of general paresis. There are not very many cases of multiple sclerosis in the literature in which a gold sol reaction has been performed, but in the majority of those tested, the reaction is reported as mild. However, cases of multiple sclerosis giving a typical paretic curve have been described by a number of observers, among whom may be mentioned Kaplan and Solomon.
3. How frequently is it necessary to make a differential diagnosis between multiple sclerosis and neurosyphilis? Before the days of the W. R. this differentiation was much more difficult than at present. But we, however, still have to face a not very rare difficulty in separating the two conditions. Syphilis is prone to cause small localized lesions in the nervous system. The changes in the patient’s condition, with improvements and regressions are equally characteristic of both diseases. How closely the symptomatology of neurosyphilis may simulate that of typical multiple sclerosis is shown in the next case (Lauder, 71). When the sclerotic area of multiple sclerosis occurs in appropriate parts of the cerebrum, symptoms of mental disturbances will occur. In its histological picture multiple sclerosis is at times highly suggestive of syphilis, even showing mononucleosis and meningitis.
Optic atrophy; nystagmus; spasticity; intention tremor. Diagnosis: ?
Case 71. James Lauder began to lose his eyesight at 32 years, and was shortly determined to be suffering from primary optic atrophy. In the course of a year, he had become completely blind. No mental symptoms had developed.
Physically, Lauder was in very good condition. Neurologically, there was a complete optic atrophy with paralysis of the internal rectus muscle, marked nystagmus, and absent pupillary reactions. All the tendon reflexes were exceedingly lively, though the right arm reflexes were more lively than the left, and the left leg reflexes more lively than the right. There was an ankle clonus on both sides. The abdominal and cremasteric reflexes were lively. There was a slight intention tremor. There was, however, no ataxia and no speech defect.
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.