Part 15
1. How shall we explain such a symptom as the transient diplopia? This diplopia is probably an example of a neurorecidive, but it will be observed that it occurred without salvarsan therapy. See discussion above under the case of Bennett (34), where the general result of the neurorecidive inquiry launched by Ehrlich early in the history of salvarsan therapy showed that precisely similar phenomena had always occurred in neurosyphilis, whether under treatment or not. The anatomical and histopathological explanation of such phenomena is, of course, doubtful, but a review of the findings in the case of Alice Morton (1) will show how many apparently serious symptoms in neurosyphilitics are actually irritative or at least due to lesions which are entirely recoverable. We may suppose, first, a local proliferation of spirochetes; second, a local over-formation of toxic substances, directly or indirectly the product of spirochetosis; thirdly, a local exudation; fourthly, a local proliferation; fifthly, a combination of these phenomena, any or all of which may be regarded as but transient. We have sometimes found at autopsy very little exudate except in small areas; sometimes not more than a few mm. or cm. in superficial extent. Note, for example, the small areas of lymphocytosis demonstrable in but two foci in the case of Alice Morton, the paradigm placed at the beginning of this book.
=NEUROSYPHILIS(?) in the SECONDARY STAGE of syphilis. HYSTERICAL symptoms. Diagnosis?=
=Case 46.= Alice Caperson was a colored girl of 18 years. She had acquired syphilis five months before admission to the hospital, and the secondary symptoms of this syphilis had just disappeared before admission.
Very shortly after acquiring syphilis, the young negress began to act peculiarly. She describes herself as having a sort of nightmare, both when asleep and also when awake. For instance, she saw her dead grandmother. It appeared at first like a seraph; then it came nearer to her and seemed to fill out; and then was dressed precisely as her grandmother had been. This seraph appeared as though trying to tell her something, but she could not make out what the something was. The vision had appeared on two or three occasions.
Our examination detected little beyond instability and irritability of mood with some depression. The patient readily fell to weeping. She soon made friends in the wards, however, and got on well. =Physical examination= was entirely negative but the W. R. of the blood serum was positive. The W. R. of the spinal fluid was negative, as was the gold sol reaction; there was an excess of albumin and a positive globulin test; there were seven cells per cmm.
The psychiatric diagnosis of a case like that of Alice Caperson would waver between hysteria and dementia praecox. However, as for dementia praecox there are hardly any typical symptoms. There is insight into the hallucinations, which are hypnagogic. There are, however, no hysterical stigmata.
The spinal fluid reaction is typical of the secondary stage of syphilis. It is commonly said that in every case of syphilis the nervous system is involved at some period, if only to the degree shown in the present case. However, such involvement tends to disappear both with and without antisyphilitic treatment, just as do the secondary skin symptoms. So far as syphilis is concerned, the prognosis under radical treatment is as good as usual. We are inclined to regard the case as one of the HYSTERICAL or PSYCHOPATHIC group and inasmuch as cases occurring in the developmental stage of a patient’s life are of fairly good general prognosis, we are inclined to regard the prognosis in this particular case as good under proper therapy and hygiene.
1. What is the relation of neuroses to syphilis? Neurasthenia, chorea, hysteria, and epilepsy are often grouped (for example, by Nonne) as neuroses bearing at times important relations to neurosyphilis. (For the relations of neurasthenia, chorea, and epilepsy, see cases of Greeley Harrison (9), Margaret Green (72), and David Borofski (49), respectively.) As for the hysteria shown in Caperson, Charcot enumerated syphilis among _agents provocateurs_ of hysteria along with alcohol, lead, arsenic, and the like. Fournier has also considered the problem. It is clearly necessary to show that before infection there were no hysterical symptoms, and that the hysteria developed during the operation of the syphilitic process, and it is probably necessary to show that the symptoms will clear up under antisyphilitic treatment, if we are to concede the existence of a syphilitic hysteria.
2. What are the evidences of neurosyphilis in the secondary and primary stages of syphilis? As above stated, the findings in Caperson are typical enough. Wile and Stokes at first stated that 60 to 70% of the secondary syphilitics show changes in the spinal fluid; in a further article they maintain that probably every case shows such changes and that clinical symptoms of neurosyphilis of the secondary period can probably be determined. They claim that it is probable also that the same holds for primary syphilis itself. The importance of these claims lodges partly in the relation of these early signs of neurosyphilis to the whole question of latency and to the question of _paresis sine paresi_. For a discussion of _paresis sine paresi_ see cases Lawlor (25), Vogel (52).
=Differential diagnosis between NEUROSYPHILIS and MANIC-DEPRESSIVE PSYCHOSIS.[9]=
=Case 47.= As in other instances (compare Martha Bartlett (21) and Annie Monks (85)) so also in the case of Ethel Hunter, a woman 61 years of age, there was no initial suspicion of neurosyphilis. Mrs. Hunter was brought to the hospital stuporous as a result of an overdose of paraldehyd. The paraldehyd had been administered by a physician to combat insomnia and agitation. As soon as Mrs. H. had recovered from the drug stupor, this agitation appeared once more, and it was clear that she was suffering from marked depression. There was tremendous worry over the sickness of a woman with whom the patient lived. The patient was very self-accusatory, blaming herself for many things that had happened in the household. Besides her agitation, depression, self-accusations, and insomnia, the patient showed a good deal of the symptom frequently termed “retardation”—a kind of lagging of all mental processes found, according to Kraepelin, in manic-depressive psychosis.
Accordingly, the diagnosis of manic-depressive psychosis might well have been rendered. The fact that the psychosis so far as known began in the involution period was not against the diagnosis since the so-called involution-melancholia of this period is at least in a certain fraction of cases nothing more or less than a form of manic-depressive psychosis. However, the =physical examination= made the diagnosis of manic-depressive psychosis a little doubtful. There was a superficial thickening of the arteries (blood pressure: systolic, 170; diastolic, 104), which thickening would not in itself be against the diagnosis of manic-depressive psychosis. (In point of fact, arteriosclerosis is rather common late in this disease and previous attacks could not be excluded on the basis of available history.) The contracted pupils were irregular and both reacted sluggishly to light, although better to accommodation; the right pupil was larger than the left. The arm reflexes were pretty active. The left knee-jerk could not be obtained, nor was the right knee-jerk more than very sluggish. The Achilles reflexes could not be obtained. Although there was not a positive Romberg sign, there was a considerable swaying in Romberg position. There was no speech defect. The other reflexes showed nothing abnormal. On the whole, we had to conclude that, although Mrs. Hunter might be an instance of manic-depressive psychosis, still there was much of neurological interest in the case.
This conclusion was emphasized when the W. R. of the blood serum was found to be positive. The spinal fluid W. R. was also positive, and the gold sol index was of the “paretic” type. There were 74 cells to the cmm. Globulin stood at ++++, and albumin at ++++.
This case, therefore, again illustrates, as well the protean nature of GENERAL PARESIS (the diagnosis rendered), as the doubtful value of making a psychiatric diagnosis without due consideration of the physical examination and laboratory findings. How easy might it have been, at least some years ago, to consider that this patient of 61 years had suffered a slight shock at some previous time (left knee-jerk absent), but was as a matter of fact a case of manic-depressive psychosis with a vascular complication!
Note: We must again duly insist that the merely sluggish light reactions of the pupils in such a case as this do not especially point to general paresis. The literature seems to establish that sluggishness of light reaction precedes the classical Argyll-Robertson pupil. Yet it does not do to say that, if the Argyll-Robertson pupil pretty conclusively points to neurosyphilis (for exceptions see cases Falvey (55), Murphy (60)), then a sluggish pupillary reaction to light looks in the same direction. Sluggishness may precede stiffness in many, or perhaps all, cases, but sluggishness of pupils is a frequent phenomenon outside the syphilitic group of cases.
1. What part is played by emotional shock and psychic causes in the starting up of general paresis? The answer to this question cannot be definite. That a paretic process can be started up after trauma is admitted on all sides; but we here suppose actual physical or chemical brain disturbance permitting increased spirochetosis or inflammatory reaction. In the case of psychic shock, or what might be called _psychogenic general paresis_, our best resort will be to the indirect effects of hormone action, or of vasomotor and other autonomic disturbances produced directly or indirectly by emotion. We are clearly here dealing with material too speculative to be of practical service at this time.
2. Was the depressive drug therapy in the case of Hunter justifiable? The paraldehyd had been administered by a physician apparently on purely symptomatic grounds to combat the insomnia and agitation of this woman of 61 years. With all due acknowledgment of the difficulties of private practice, we must insist that when ordinary measures in the relief of insomnia and agitation are insufficient to curb these conditions, then a positive danger ensues with the larger doses. As a rule, with these larger doses and with the withdrawal of sensory stimulation, the patients relapse into a stupor of grave moment. We need only recall the situation in delirium tremens where adequately depressive drugs often tend to kill the patient.
=Case for diagnosis. Errors in the diagnosis of NEUROSYPHILIS are possible even when abundant clinical and laboratory data are available.=
=Case 48.= The first error chosen for demonstration is that in the case of the machinist, Milton Safsky.
Safsky, about 8 months before his entrance to the hospital in the 42d year of his life, had begun to lose strength, to grow thin and pale, and to suffer from an extreme and continuous thirst. He was said to have drunk as much as 6½ gal. in a day, and passed appropriately large quantities of urine. After a time, his management at a general hospital became difficult, as Safsky became confused, cried “hysterically,” and was at times very noisy. He sustained a marked memory loss, seemed to show visual hallucinations, and complained of headache, both frontal and occipital, and of pain about the eyes. Sometimes the patient was very euphoric and expressed what seemed to be delusions of grandeur, saying he was wealthy and owned many machine shops.
Some symptoms, e.g., polydipsia and polyuria amounting to a diabetes insipidus, associated with headache and arrested attention, suggested possibly a new growth in the pituitary region. The mental symptoms might naturally be supposed to be due to some infiltration or pressure effect of intracranial growth. After admission to the Psychopathic Hospital, the patient was found difficult to arouse, although he could eventually be aroused. His orientation proved to be as poor as his memory. From time to time, the patient became a bit more intelligent and able to execute requests.
The =physical examination= was in general almost entirely negative. =Neurologically=, the pupils were markedly contracted and reacted slowly to light, though they were otherwise normal. The deep reflexes were all somewhat lively, though equal. The umbilical and cremasteric reflexes in particular were present. Systematic examination revealed no other reflex disorder, nor any disturbance of sensation. There was a coarse tremor of the extended hands. There were no phenomena of importance in the visual fields.
As against the diagnosis of growth, pituitary or extrapituitary (diabetes insipidus and headache), a hypothesis of neurosyphilis had to be considered. Not only were the contracted, slowly-reacting pupils and the active deep reflexes suggestive, but the euphoria with grandiose ideas looked entirely consistent. As for the polyuria, one had to think of the so-called syphilitic polyuria of the textbooks, which is regarded as a more or less characteristic result of syphilitic involvement of the _basis cerebri_. Moreover, the W. R. in the spinal fluid proved to be slightly positive; 146 cells per cmm. were found therein; there was a large quantity of globulin, and a very marked increase in albumin. These observations seemed to be exceedingly suggestive of a cerebral syphilis.
However, as the case progressed, the diagnostic situation changed. The W. R. upon a second puncture fluid proved negative. After some weeks, characteristic symptoms of intracranial pressure developed; the diagnosis of BRAIN TUMOR had to be taken as established, and there is no doubt of its correctness.
1. What is the explanation of the weakly positive W. R. in Safsky’s spinal fluid? An explanation is not easy to find. Possibly we may regard the reaction as an example of error in technique. It is even possible that it may have been produced by exudative products in the spinal fluid.
2. What precautions may be taken against an error in diagnosis such as was first made through the positive spinal fluid Wassermann in the case of Safsky? First, repetition of the W. R.; secondly, it is very unusual to find a weakly positive W. R. in a case with such marked excess of albumin and such very marked increase of globulin as was shown by this case.
3. How can we explain the inflammatory products in the puncture fluid? Superficial brain tumors are frequently associated with a so-called _meningitis sympathica_. The products of such meningitis are exhibited: _viz._, globulin, albumin, and pleocytosis, exactly as shown in Safsky.
=Can PARETIC NEUROSYPHILIS (“general paresis”) appear clinically EARLY (e.g., two years) after the initial syphilitic infection?=
=Case 49.= David Borofski, a street car conductor, 27 years of age, suddenly had a convulsion while at work in his car. For four months Borofski continued to have rather numerous convulsions, was finally compelled to discontinue work, and resorted to the Psychopathic Hospital. It appears from his own story that, about two years before, he had had a chancre, for which he had been treated at a general hospital syphilis clinic, and of which he was told he was cured. With a progressive loss of memory and with convulsions, Borofski became much concerned about himself, and was finally persuaded by his fellow-workers to come to the Psychopathic Hospital.
The convulsions were described as follows: The patient gives a short cry, has convulsive movements for about ten minutes, remains unconscious for perhaps half an hour, and wakes with headache, dizziness, and a feverish appearance. Sometimes the attacks were more severe, with frothing at the mouth, biting of lips, and loss of sphincter control. There were also slight attacks, occurring almost every day, without loss of consciousness; these latter attacks consisted of dizziness, inability to speak for a few seconds, and some arm twitching.
=Physically=, Borofski was well developed and nourished, with a blood pressure of 160. The only abnormal phenomena =neurologically= were absent knee-jerks and ankle-jerks, sluggish pupillary reactions, and slight tremor of the hands.
=Mentally=, despite suggestive complaint of amnesia, the memory was found to be fairly good but knowledge of current events and school knowledge was poor. The simplest problems in arithmetic Borofski gave up.
The first diagnosis in such a case would naturally be epilepsy. However, when an epileptic or epileptiform attack occurs for the first time in adult life, the chances are probably against an idiopathic epilepsy. (This is not a universal rule but will serve.) Borofski himself, moreover, gave a history of syphilis. And the very nature of the attacks, with arm twitching and without loss of consciousness, would not readily fit into the frame of the idiopathic group. The absence of certain reflexes and the sluggish pupils are naturally also suggestive of syphilis, although not convincing.
The W. R. of the serum proved positive, as did that of the spinal fluid. The gold sol reaction was characteristically “paretic”; there was an excess of albumin and a positive globulin, and there were 15 cells per cmm. There could be little or no doubt of the diagnosis of some form of neurosyphilis. The laboratory picture was consistent either with general paresis or with cerebrospinal syphilis. So far as we are aware in the present stage of knowledge, the two conditions can hardly be differentiated unless we choose to rely on therapeutics. However, it is exceedingly rare for general paresis to occur only two years after the original infection. If we can trust this statistical fact, we shall perhaps be wiser to term the case of Borofski one of DIFFUSE CEREBROSPINAL SYPHILIS, and not one of paresis.
=Treatment=: Borofski was put on antisyphilitic treatment consisting of 0.6 gram of salvarsan twice a week and potassium iodid, together with intramuscular injections of mercury salicylate. The convulsions then ceased. After four months Borofski returned to work, and he has remained at work for a year. He has never regained his former health.
Fifteen months after beginning of treatment the laboratory tests were again made (there had been more than 60 injections of salvarsan), and the cell count and gold sol reactions were found to be negative. Globulin and albumin were also in smaller amounts than in the original examination. However, the W. R. of the serum and the spinal fluid remained positive.
Head and Fearnsides state that cases of cerebrospinal syphilis should return negative spinal fluid tests after six months of treatment. Upon this criterion of Head and Fearnsides, Borofski would not be a case of cerebrospinal syphilis; but it is probably impossible to separate various forms of neurosyphilis into categories on any such grounds.
1. Shall case David Borofski be regarded as one of paretic neurosyphilis (“general paresis”)? He has returned to work and has remained at work, though without regaining his former health. In any event, however, he does not offer the typical picture of inevitable decline and death presented by the typical case of Pietro Martiro (15) presented in our discussion of systematic diagnosis. However, we could not upon laboratory grounds, or even upon the ground of clinical observation, distinguish Borofski from Martiro; Borofski has greatly improved; Martiro is dead. Borofski developed his obvious neurosyphilis only two years after the original infection. The conservative syphilographer might, accordingly, reply that David Borofski is not a typical case of paretic neurosyphilis (“general paresis”) either in the length of the incubation period for his neurosyphilitic symptoms, or in his outcome.
2. What is the cause of such convulsions as those developed by David Borofski? Evidence from clear cases of general paresis with convulsions leads to the hypothesis that such convulsions as those developed by Borofski are not necessarily based upon frank destructive lesions such as would be produced by the plugging of terminal arteries. They may well be produced through the activities of minor lesions, only demonstrable by microscopic methods, either through properly disposed cell losses or by the pressure of exudate, or even by endotoxins or other substances derived from the bodies of dead or living spirochetes.
3. Aside from the well-known syphilitic epilepsy due to meningitis, is there a non-meningitic epilepsy (such a disease as Fournier formerly described under the term parasyphilitic epilepsy)? We dismiss from discussion the so-called symptomatic epilepsies which are the result of a gross organic disease of the brain substance or its membranes, and which do not differ so far as we are aware from organic epilepsy produced by other gross lesions of an identical size and structure. These symptomatic epilepsies may be partial, or even may present the appearance of generalized epilepsy. We may also leave out of account those epileptic pictures which are produced in general paresis itself, and which may be viewed as nothing but partial phenomena of general paresis. The kind of so-called “parasyphilitic” epilepsy that Fournier described is a kind of epilepsy that cannot be distinguished from genuine epilepsy, in which the sole disease-phenomenon throughout a long period of time consists of epileptic convulsions. It appears that these “parasyphilitic” imitations of genuine epilepsy occur in individuals with a very long post-infective “incubation period,” but that there are some cases in which the epilepsy appears, on the contrary, in the very earliest stages of syphilis. The attacks are a little less common than those of idiopathic epilepsy; they have the same apparently causeless beginning; are associated with complete amnesia; and are followed by characteristic dazed states. The patient’s intelligence, however, suffers little. Now and then a case reacts well to antisyphilitic treatment energetically pushed. (Spontaneous long remissions in non-syphilitic epilepsy must be remembered.) Petit mal attacks occur sometimes between the more severe attacks. In short, it would appear that there is a group of syphilitic epilepsies in which the brain shows no gross structural lesions, which accordingly do not exhibit any Jacksonian appearances, and which last a comparatively long time without changing their character, and often without being especially altered for the better by any form of antisyphilitic treatment. This condition is sometimes known as a post-syphilitic epileptic neurosis. Nonne had been able to collect up to 1902 some 12 cases from his own service.