Part 6
2. Is there such a disease as syphilitic neurasthenia? According to Kraepelin, syphilitic neurasthenia has been described as occurring shortly after infection and in the first stages of syphilis. There are milder and severer forms; the milder forms show discomfort, difficulty in thinking, irritability, insomnia, cephalic pressure, indefinite variable, uncomfortable sensations, and pains. The severer cases acquire anxiety, more pronounced emotional disorder, dizziness, disorder of consciousness, difficulty in finding the right word, transient palsies, pronounced sensory disorders, nausea, and increase of temperature. Kraepelin is in doubt whether there is any definite clinical picture of this sort, and whether there is any causal relation between the syphilitic infection and such symptoms as those described. If the effect of knowledge concerning infection is a merely psychic effect, then it is improper to term the neurasthenia in question a syphilitic neurasthenia. For the relation of hysteria to the acquisition of syphilis, see below the case of Alice Caperson (46). In point of fact, modern work has shown even in the primary and secondary stages of general syphilis more or less pronounced neurosyphilitic phenomena in the shape of the so-called meningitic irritation of French authors. (Besides the case of Caperson (46), see the case of Fitzgerald and the discussions under these cases.)
3. What is the relation of the early symptoms of this case to the so-called preparesis of Dana? The case might well have been an example of Dana’s preparesis. For a discussion of this, see Case of William Twist (13).
4. What is the classical differential diagnosis between paretic neurosyphilis and neurasthenia? The testing of the blood by the W. R. is unconditionally necessary. If the W. R. is negative, the diagnosis of paretic neurosyphilis is extremely improbable. (It must be borne in mind that a number of cases of paretic neurosyphilis have been shown to have a negative W. R. in the serum, and receive a proper diagnosis only after spinal fluid examination.) Next to the serum W. R. stand the pupillary and aphasic symptoms. In the presence of Argyll-Robertson pupil or even a slight speech defect, the diagnosis of neurasthenia must certainly be made with caution if at all. Kraepelin remarks: The sudden occurrence of neurasthenic disorders in a male of middle age without any evident cause therefor is always suspicious. Yet it must be emphasized that a complaint of occasional dizziness, slight speech defect, tremor of tongue, and a moderate increase of tendon reflexes do not possess any marked diagnostic significance. Clear insight and understanding of the nature of the disease phenomena, a persistent search for recovery, reasonableness in conversation, progressive improvement under appropriate treatment, speak for neurasthenia.
Joffroy and Mignot differentiate what they call preparetic neurasthenia from other neurasthenic states, not only on the basis of its etiology but on the basis of its symptoms. They also call attention to the fact that neurasthenia, being a pure neurosis, develops either on a manifestly hereditary basis or upon some physical injury, weakening disease, or moral shock. The pure neurotic suffers a great deal more than the patient who is destined to become a victim of paresis. The character change in neurasthenia does not amount to that entire transformation of personality (even to the performance of criminal acts) that we find in paretic neurosyphilis; at the most, the neurasthenic shows minor emotional disturbances and a certain pathological egoism. The psychotherapeutic test also rather readily dissipates many of the neurotic, hypochondriacal fears and feelings. Although both pure neurasthenia and the paretic pseudoneurasthenia are characterized by sexual weakness, the sexual anæsthesia of the preparetic is practically always preceded by a stage of sexual over-excitement. These finer clinical indications, however, fade into insignificance beside the data that can and should be obtained from laboratory tests.
5. How exceptional is such a case as that of Harrison? We have in our experience seen many patients with a similar course and configuration of symptoms, although the majority of these cases in a community advanced enough to provide easy access to a Wassermann laboratory are now diagnosticated far earlier than was the case of Harrison.
6. What attitude shall we take toward so-called syphilophobia? It seems to us that resort to a serum W. R. is indicated, both from the standpoint of the community and still more importantly from the standpoint of the patient. We are even inclined to suggest for a case of persistent syphilophobia, when the serum W. R. has proved negative, a lumbar puncture. Syphilophobia must be considered, not as a syphilitic psychosis, but as a phobia to be classified among the psychoneuroses. It becomes a difficult question to decide at times whether a patient who has had syphilis, has had a considerable course of treatment and shows the symptoms of a syphilophobiac should be further treated for syphilis or merely for his phobia. We have seen recently such a patient who gave a certain history of syphilis and who was greatly disturbed lest he should be developing paresis. This fear bothered him greatly. Examination showed irregular pupils, but no other signs of syphilis. The W. R. in blood and spinal fluid was negative as were the other spinal fluid tests. It was considered wise to treat him only for his phobia and under this treatment he was given some relief.
=PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like MANIC-DEPRESSIVE PSYCHOSIS.=
=Case 10.= The mental picture in Lyman Agnew, an architect, 58 years of age, was wholly characteristic of manic-depressive psychosis. In the first place, there had been (at 55) a previous attack of depression, lasting a few months, from which Agnew had completely recovered. He had remained entirely well up to four months before consultation. (Manic-depressive psychosis is, at least in a majority of cases, hereditary. There had been mental disorder in one maternal cousin, and mental impairment in the patient’s mother some time before her death from cerebral hemorrhage. There was no other report of mental disease in the family.)
It appears that in the interval between attacks, Agnew had been working very hard and had been fairly successful in paying off a mortgage on his house. A marked elation, somewhat natural, followed this success and continued to an abnormal degree. Agnew labored under considerable excitement, was over-fussy, and at times showed a flight of ideas. His mania or hypomania gradually diminished and depression set in, in which depression he arrived for consultation. He had marked ideas of self-accusation, was emotionally unstable, wept much, and showed a characteristic retardation of activities and unrest.
=Physically=, there was no neurological disorder. The patient appeared rather under-nourished. The heart borders lay 2 cm. to the right and at 11½ cm. to the left of the mid-sternal line. The aortic second sound was very loud. There was a moderate radial arteriosclerosis. Systolic blood pressure was 210, diastolic 155.
The high blood pressure suggested nephritis, possibly of arteriosclerotic origin, but urine examination and blood-nitrogen tests yielded no evidence of kidney disease. Moreover, it is our experience that a manic-depressive psychosis in persons past middle life is not infrequently complicated by high blood pressure. In point of fact, some authors insist upon a relation between manic-depressive psychosis and the arteriosclerosis which rather frequently sets in in this disease.
Routine examination of the blood serum, however, yielded a positive W. R. Following the approved rule of making an examination of the spinal fluid in all mental cases having a positive serum W. R., we proceeded to lumbar puncture. The fluid was clear and contained 35 cells per cmm., the albumin was in excess, and there was a positive globulin reaction. The gold sol reaction was of the “paretic” type; the W. R. was strongly positive.
On this basis, it seems worth while to consider the diagnosis of GENERAL PARESIS or that of some form of non-paretic neurosyphilis. The former is the diagnosis which we prefer.
1. What is the classical differential diagnosis between manic-depressive psychosis and neurosyphilis? The laboratory tests have naturally supplanted the older purely clinical methods of differential diagnosis. The difficulties lodge, in the first instance, in depressive states. It would appear to be impossible on purely clinical grounds in certain cases to tell the depression of neurosyphilis from the depression of manic-depressive psychosis, since the slightly greater interest in the outer world taken by manic-depressive patients and their greater responsiveness to diagnostic threats (suggestion that patient is to be pinched or cut) are of no special value in the individual case. Identical considerations hold for the maniacal phases of manic-depressive psychosis, for these maniacal phases may even develop delusions (Kraepelin) of precisely the same nature as the characteristic expansive delusions of the excited paretic.
2. If the clinical symptoms are insufficient in differential diagnosis, are not the pupillary signs and the speech defect of greater value? They are of value if present, but as in the case of Agnew, the victim of neurosyphilis may show no pupillary or speech disorder. Instances are familiar, also, in which the pupillary and speech signs are absent in very advanced cases of non-paretic or even of paretic neurosyphilis.
3. Would not a circular course or recurrence of attacks be decisive for manic-depressive psychosis? Paretic neurosyphilis sometimes exhibits the same circular or recurrent course. We conclude that neither the clinical symptoms, the classical pupillary and speech signs, nor the ups and downs of a particular disease, are at all decisive as between manic-depressive psychosis and paretic neurosyphilis. Resort must be had to laboratory tests.
4. What is the significance of the high blood pressure in paretic neurosyphilis? Work from our laboratory (Southard and Canavan) has shown plasma cells in the kidneys in 17 out of 30 paretics (56%), and in 16 of these 17 paretics with renal plasmocytosis, the plasma cells were found in the periglomerular region. What the relation of these findings may be to heightened blood pressure is as yet unknown. The severe syphilitic involvement of the aorta so characteristic in paretic neurosyphilis, as in other forms, may possibly have a bearing on blood pressure.
=A POSITIVE SERUM WASSERMANN REACTION associated with mental symptoms (even with grandiosity) does NOT prove the EXISTENCE OF PARETIC NEUROSYPHILIS (“general paresis”).=
=Case 11.= Juliette Lachine came to a general hospital with pain in the right upper quadrant of the abdomen, wherein was found an enlarged liver. This liver was regarded as syphilitic on the ground that the patient had a positive serum W. R. and that her two elder children were clearly suffering from congenital syphilis. The liver mass was promptly reduced by antisyphilitic treatment of the classical sort. When, however, the patient was given an injection of salvarsan, she shortly began to develop marked mental symptoms, whereupon she was removed to the Psychopathic Hospital.
The =mental picture= at the Psychopathic Hospital was as follows: Lack of orientation for time, marked distractibility of attention, with a certain jumping from one subject to another, delusions of a religious nature, claims of wonderful powers possessed by the patient, moods variable, though as a rule of a euphoric and elated nature, with laughing and singing. The activity seemed to be of a mental rather than a peripheral nature. The patient did not regard herself as mentally abnormal. The liver was still 4 cm. below the costal margin in the nipple line. We found the W. R. to be positive in the serum but negative in the spinal fluid. In fact, the spinal fluid was entirely negative.
So far as we are aware the picture presented by this case is one of MANIC-DEPRESSIVE PSYCHOSIS. We regard the disease as merely complicating the syphilis, although it is entirely possible that some visceral condition incidental to the syphilis might be proved (in a higher stage of psychiatric science) to have produced the mania.
In any event, the patient quite recovered from her mental symptoms in a month. She was then able to tell us of a previous attack of depression some 12 years previously, namely, at the age of 26. It appears that she had at that time been committed to a hospital for the insane.
1. In this case, in which the diagnosis of manic-depressive psychosis and not paretic neurosyphilis was made, are we sure that the symptoms that we term manic-depressive psychosis were not actually produced by syphilotoxins? In other words, in the absence of spinal fluid signs of inflammation or chemical change, might it not be possible for generalized syphilis outside the nervous system to produce manic-depressive symptoms? There is so far in the literature no experimental or other evidence of syphilotoxins. The existence of products and substances permitting the W. R. and the gold sol reaction is not of course evidence of syphilotoxins. Although there is no evidence of soluble syphilotoxins, it is thought that in the so-called Järisch-Herxheimer reaction (the intensification of clinical symptoms after salvarsan injection) effects may be due to the liberation of products from the killed bodies of spirochetes. Such endotoxins are not here in question.
2. Is visceral syphilis, such as gumma of the liver, able to produce characteristic syphilitic reactions in the spinal fluid? We have had an autopsied case in which there was a “paretic” gold sol reaction of the fluid (though without other signs). The autopsy showed gummata of the liver. However, the finer anatomy of the nervous system showed a mild but definite meningo-encephalitic process, which was doubtless responsible for the gold sol reaction.
3. What is the value of grandiose ideas? Ballet distinguishes two groups of grandiose ideas: (_a_) ideas of self-satisfaction, including ideas concerning extraordinary capacity, strength, power, and wealth on the part of the patient; and (_b_) ideas of ambition; the latter being of a more exact, constant, uniform and systematizing nature. The more vague and less systematized ideas of self-satisfaction rest in a phase of contentedness and optimism; the more definite ideas of pride and ambition are responsible for striking transformations of personality. General paresis shows, according to Ballet, these ideas of self-satisfaction in their most developed form. A certain variability, absurdity, incoherence, and contradictoriness characterize these ideas and the patient has little or no insight into their nature. When such ideas occur at the outset of the disease, they naturally may be of medicolegal interest. Cotard explains these ideas of megalomania on the part of paretics on the ground that they are essentially motor or will disorders and rest upon a sort of hyperbulia, exhibiting itself in exuberant activity. Régis has thought that the delusional generosity and liberality of the paretic, and his willingness to lend his wealth and talents to social progress, is helpful for diagnosis when contrasted with the more personal egoism of the victim of manic-depressive psychosis. The self-satisfaction of the manic-depressive patient often does not reach a delusional stage, but remains a mere feeling of pathological well-being or euphoria. The maniacal patient may compare himself with some great man but he does not identify himself with him. It must be remembered that these ideas of self-satisfaction occur also in alcoholism, but according to Ballet they occur only in the dementing phase of chronic alcoholism, and have no special diagnostic value. They may be a clinical stumbling-block for a time in the cases of alcoholic pseudoparesis. As for the ideas of ambition in which the patients believe themselves to be princes, emperors, divine messengers, and the like, these are less characteristic of paretic neurosyphilis than of delusional psychoses of a non-syphilitic nature. At all events, such ideas if definite, of long-standing, and systematized by the patient to form a thorough-going portion of his life, are not characteristic of neurosyphilis. The victim of paretic neurosyphilis can as a rule be persuaded out of his delusions, at least for the time being. These distinctions, it must be added, are hardly of value in the early cases of any of the psychoses in question, and cannot be made as a rule in either private or psychopathic hospital practice. Typical examples of grandiosity, although not so frequent as might be thought from textbooks, are always on display in institutions for the chronic insane.
=PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like DEMENTIA PRAECOX. Autopsy.=
=Case 12.= Henry Phillips remains a striking case in the memory of those who knew him and his medical findings. Phillips came to the hospital voluntarily at 42 years of age from the bank where he worked as a clerk; he came at the suggestion of his employer. It seems that he had been annoying his associates because he had fallen into a habit of continually scratching himself. Phillips was entirely sure that he was the victim of what he called the “Scotch itch,” and explained off-hand that this itch had been put upon him by the Free Masons as a matter of revenge because he would not join their order. He said once, for example: “At times I feel like raising Hell; then I get a psychic intimation; and then I get to using a foot-rule on my back and to slapping my face.” He explained this psychic intimation as coming from the order of Scottish Rites. Another example of talk is as follows: “My father is a fighting man; that is part of it. They mean to throw me down. I am through now trying for membership in the Free Masons. They have good cause, they must fight. They do not want me for some personal matters. I can go just so far in agreeing and seconding their advances, but in the end it fails. I have no strength nor endurance.”
Aside from these delusions, there was little abnormality to be found, though his recollection for minor events of the immediate present was inaccurate. He was rather abnormally impulsive, gesticulating a good deal while talking, and was of the appearance that the laity call “nervous.” It appears that he had always been peculiar, subject to violent fits of temper, in which fits he might throw things at other members of the family. He always had pronounced likes and dislikes which he never concealed. He had never had friends, had always been secretive; and he was often termed a great student. For some five years he had been studying Japanese from time to time, associating himself with a Japanese.
It never does to jump at the diagnosis dementia praecox. However, the picture seemed characteristic enough for the paranoid form of this disease. Physically, Phillips had no particular abnormality; the knee-jerks were a little lively, and the pupils reacted a little sluggishly. However, the routine W. R. of the serum proved to be positive. Examination of the spinal fluid was resorted to,—as in all cases with a positive serum W. R.—and it also proved to be positive and strongly so; the globulin and albumin were increased, and there was a pleocytosis. A diagnosis of neurosyphilis was hardly avoidable. Phillips later admitted a chancre, which he claimed was located on the mucous membrane of the cheek and acquired by using the same utensils as his Japanese friend, which friend, he stated, had active syphilis.
Antisyphilitic treatment of considerable intensiveness was begun, with intravenous injections of salvarsan and intraspinous injections of salvarsanized serum, but the patient grew steadily worse. His mental symptoms became more marked, although not especially characteristic of general paresis. =Neurologically,= he did develop signs more suggestive of general paresis, and 18 months later died.
The =autopsy= showed features of GENERAL PARESIS. It is not necessary to enter into the question of the details of histological correlation at this time.
1. What conclusion can be drawn from lively knee-jerks? Lively knee-jerks are of very little significance. Not only certain neurosyphilitics but also a variety of neurotic persons, victims of dementia praecox and hysteria, are very prone to have active tendon reflexes. Of course, extreme degrees of exaggeration are of importance, and especially an association of the hyperreflexia with the Babinski reaction, the Gordon, or Oppenheim reflexes, ankle clonus, and the like.
2. Is there any special or differentiating factor in an extragenital chancre as against a genital chancre? Probably this question should be answered in the negative. Some have claimed that chancres draining by lymphatic channels of the head are more likely to lead to cerebral syphilis. This idea cannot be said to be established.
3. Is there any significance in the story, if true, that Phillips acquired his syphilis from a Mongolian? It seems to be fairly well established that syphilis of the nervous system is extremely rare in China and Japan, whereas bone syphilis is very frequent there. It has been held that this has to do (_a_) with strains of spirochetes, (_b_) with the state of civilization, or (_c_) with the degree of “syphilization.” Apparently when a race is first infected with syphilis the lesions are chiefly of the cutaneous and osseous systems; only in later generations the vascular and nervous systems suffer. However, involvement of the nervous systems of Mongolians resident in this country is no rarity, a point possibly in favor of the theory of special strains affecting the nervous system as prevalent in western countries. Little or nothing is known as to the effect of transmission from one race to another, as from Mongolian to Caucasian in Phillips’ story.
=NEUROSYPHILIS is NOT to be entirely ruled out by a negative serum Wassermann Reaction; for the fluid Wassermann Reaction may be positive.=
=Case 13.= William Twist is a case of note in the matter of the so-called preparetic period (the idea of Charles L. Dana which was scoffed at when first proposed by him in 1910). The patient, a very successful traveling salesman, 35 years of age, was admitted to the Psychopathic Hospital showing a typical picture of general paresis.