Neurosyphilis

II. THE SYSTEMATIC DIAGNOSIS OF THE MAIN FORMS OF NEUROSYPHILIS

Reading edition

PARETIC NEUROSYPHILIS (“general paresis”) sometimes persistently receives the diagnosis NEURASTHENIA simply through omission to apply approved diagnostic methods.

Case 9. Greeley Harrison, a man of 46, certainly looked like a neurasthenic. He wanted aid for nervous indigestion of years’ standing, headache, insomnia, nervousness, failing memory, and deafness. He volunteered, in fact, that he had neurasthenia, and that he had been treated for this by hypophosphites.

During the practically negative physical examination, Harrison complained of headache and throbbing in the head, and during examination of the abdomen felt much nauseated and proceeded to vomit rather persistently. There were hemorrhoids.

Neurological examination showed that the left pupil was smaller than the right, was irregular, failed to react consensually, and reacted very slowly to direct light. For the rest, however, the neurological examination was negative. On account of the nausea and vomiting, special examination of the gastric contents was made, but nothing abnormal was found.

Mentally, it was rather striking that the patient’s memory was quite inaccurate both for remote and for recent events. His school knowledge was very meagre. As for delusions, the only approximation thereto was the patient’s continually dwelling upon his bodily symptoms. Emotionally, he varied between depression and a sanguine attitude.

64Although there was no symptom directly suggesting syphilis in the Harrison case, the slightly abnormal pupillary reactions and the amnesia warranted the suspicion of syphilis. The blood and spinal fluid both proved positive to the W. R.; the gold sol reaction was of the “paretic” type; there were 18 cells per cmm.; there was considerable globulin, and an excess of albumin. On the whole, therefore, we felt entitled to make the diagnosis General Paresis. Why should not a careful observer have considered syphilis seriously? Yet in our experience such cases are frequently diagnosticated neurasthenia, thus entailing dangerous delay in treatment (in this case, five years’ delay).

Going over the history of the case with still greater detail, we learned that for a number of years past, there had been symptoms of a neurological nature. For instance, five years before, at the age of 41, the patient had been apparently overcome when working near a stove, and went upstairs talking incoherently, but recovered shortly. Thereafter, such spells occurred almost every month; later, more frequently; still later, the attacks were associated with unconsciousness and amnesia. Occasionally preceding the attack there would be twitching of the mouth, jerking of the arms, and incoherent talk. Throughout these last five years, in point of fact, the patient had been unable to do regular work, had been given to much complaining, and had been far less efficient than formerly. In short, it would seem that, with the improved technique now in the possession of medical science for the diagnosis of general paresis, cases like that of Harrison will be diagnosticated earlier and earlier.

1. How typical is the insidious onset of symptoms in the case of Harrison? The onset of symptoms in neurosyphilis is ordinarily considered to be sudden, and this statement is generally true despite the fact that after the diagnosis is established a number of mild prodromal symptoms can be remembered by the relatives. However, some cases, of which Harrison is an example, have an exceedingly insidious onset without sudden access of striking symptoms. Joffroy and Mignot remark that with the improvement of clinical methods, the course of paretic neurosyphilis must now be stated 65to take some six or seven years for completion. In point of fact, there were early episodic symptoms (seizures almost monthly) which should not have escaped medical attention. They did escape medical attention, however, and Harrison was wont to say “Why wasn’t I told that my disease was syphilis five years ago?”

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 66pupillary 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.

676. 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 69in 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.

703. 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 72previous 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, 73and 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 75termed 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 76this 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.

Thus, mentally, the patient showed elation, grandiosity (millions of dollars to give away), intellectual weakness, disorder of memory, lack of judgment, rambling talk, speech defect, omission of letters in writing and spelling.

Neurologically, there was tremor of the lips, slight irregularity of the pupils, which however reacted well, and lively knee-jerks.

Mr. Twist had sought advice at our out-patient department in his thirty-third year. The records show that at that time he was somewhat depressed, and his speech was even then, according to his own statement, stammering. However, we found the W. R. at that time to be negative in the blood serum. It appeared that his mother had died of consumption; his father was said to have committed suicide. A brother had once recovered from an attack of depression, presumably an attack of manic-depressive psychosis. Accordingly, we thought at the time that the case was probably one of manic-depressive psychosis. Moreover, our routine serum W. R. failed to indicate any syphilitic process. As for the so-called stammering of speech, this appeared to be a matter of the patient’s own recollection rather than of our observation. In any event, the patient had gone into the country and appears to have entirely recovered; falling, again, however, into mental difficulties after a short period, and finally arriving at the hospital in the above-mentioned classical condition.

78The W. R. in the blood serum proved again negative. The test was repeated a number of times; also, after salvarsan had been given. The salvarsan did not act provocatively, and the blood serum has remained consistently negative.

In cases of syphilis the W. R. is at times negative. Swift claims that in such cases an injection of salvarsan will often produce a positive W. R. if the blood is tested on several days following the injection.

The spinal fluid, however, did show a positive W. R. as well as a gold sol reaction of a “paretic” type. There were at the first examination 194 cells per cmm., there was a moderate excess of albumin, and a positive globulin test. In short, there was no question of any other diagnosis than General Paresis.

1. How can the negative W. R. of the blood serum be explained? It is difficult or impossible to explain this. Figures differ as to the percentage of cases of general paresis with negative blood serum; perhaps 3 to 5% of these cases yield a negative serum W. R.

It is important to note the long preparetic period: at least a year and a half. Could our diagnostic methods be sharpened a trifle, such cases as these could be obtained early in this preparetic period and it might then be safe to promise good therapeutic results.

2. What is the nature of the preparesis of Dana? When Dana’s brief paper on preparesis was written, there was of course hardly any idea that cases of paretic neurosyphilis could be cured or would recover, except possibly vanishingly few curiosa about which there would always rage a diagnostic question. Accordingly, Dana, having found certain cases that seemed to him to have early signs of paresis but had apparently been cured by treatment, proposed to call them cases of preparesis. His idea was that he would thereby not offend those who held that general paresis was theoretically a fatal disease. With modern work and the display of more and more atypical cases of neurosyphilis, and the observation of relatively numerous cures or remissions under treatment, the designation of preparesis for a separate entity, or even for a sub-form of neurosyphilis, becomes superfluous.

793. What is the percentage of cases of paretic neurosyphilis that show a negative serum W. R.? Among the best figures are those of Müller, who found that of 386 examples of paretic neurosyphilis, 379 showed all reactions positive, or 98.5%.

4. What is the meaning and value of the so-called provocative salvarsan injection? In practice, there may be a series of negative W. R.’s in the blood serum before a positive reaction is finally obtained, owing to technical difficulties or biological peculiarities. Where intensive work is being done upon the neurosyphilis problem, it is beyond question desirable to make the W. R. test upon at least three separate samples of blood drawn at intervals, for the second or third test may prove positive. This situation makes the interpretation of the so-called provocative salvarsan injection exceedingly doubtful; that is, the reaction might have been positive on repetition without the injection of salvarsan. The present case, as above stated, failed to yield a serum W. R. even after repeated tests and the “provocative.”

5. What is the significance of the irregular pupils in this group? Paretic neurosyphilis shows inequality of the pupils in a high per cent of cases. Irregularity of outline of the pupils is commonly thought to be an important sign and to suggest neurosyphilis. It is true that many cases of pupillary irregularity are syphilitic, but the sign is of little or no differential value since congenital malformations and relics of old injuries and adhesions may produce effects identical with those of neurosyphilis.

DIFFUSE (that is, meningovasculoparenchymatous^[5]) NEUROSYPHILIS is typically associated with six positive tests (serum Wassermann reaction, fluid Wassermann reaction, spinal fluid gold sol reaction, pleocytosis, positive globulin, excessive albumin); but one or more, and frequently several, of these tests are likely to run mild as compared with the tests in PARETIC NEUROSYPHILIS (“general paresis”). The clinical course of the diffuse (and especially the meningovascular) cases is likely to be protracted, with a good prognosis as to life (barring fatal vascular insults).

Case 14. We shall present the case of John Jackson, a surveyor, 31 years of age, suffering from a left hemiplegia, with this in mind: To exhibit difficulties in diagnosis in the presence of an embarrassment of symptomatic riches.

The patient arrived at the hospital, in the first place, because he had been threatening a woman who lived next door to him. He believed that this neighbor had been talking about him and circulating reports against him. Excited by these ideas, he had threatened to cut her throat.

Now the occurrence of hemiplegia in adult life before the approach of senium is always suspicious of syphilis, and this suspicion we naturally entertained from the beginning. However, there was upon the scalp a crooked linear furrow about six inches long, running from the vertex to the right parietal eminence. Another furrow about an inch long was present upon the forehead. These furrows appeared to be of a bony nature and were not tender. There was evidence of an old decompression operation on the right side of the head; there were also large scars on both sides of the neck, evidently the result of old operations; and there were numerous palpable glands—the largest about the size of a lima bean—all firm and not tender.

Illustration from Neurosyphilis: Modern systematic diagnosis and treatment presented in one hundred and thirty-seven case histories

Station in syphilitic hemiplegia. Syphilitic pigmentation of skin.

81It seems that at the age of eight, according to the patient’s mother, Jackson had received a head injury and had remained unconscious for three weeks. Upon recovery, he had to relearn both to walk and to talk; however, he was able to begin school where he left off. He became more nervous and irritable after the accident than previously. Nothing further had developed until, at about 25 years of age, a tubercle was discovered in his eye (the right pupil was smaller than the left, reacting more slowly; right iris bound down by adhesions, with white opacity of anterior chamber). For two years, 25 to 27, the patient was under medical treatment for tuberculosis, and at the conclusion of this period numerous glands were removed from the neck and diagnosticated tuberculous. However, the neck did not heal and he carried bandages upon it for two years.

At 28, the patient’s mother described the occurrence of a slight shock, with head retraction, for a minute or two, and inability to speak. Thereafter there had been five or six similar attacks, less severe, and without loss of speech. The attacks were never accompanied by convulsive movements. Then occurred a paralytic stroke, leaving the patient with a left hemiplegia, which had somewhat improved. Mentally, the patient had gone down hill, becoming less alert and more apathetic, and to some extent amnestic. One had to consider, accordingly, the somewhat doubtful possibility of post-traumatic and post-operative conditions, and the question of tuberculosis (possibly errors in diagnosis; the lungs showed no evidence of tuberculosis).

Physically, the signs of a left hemiplegia were appropriate. Spasticity on the left side was found; there were Babinski, Gordon, Oppenheim reflexes and ankle clonus on the left side (all absent on the right). Speech defect was present. Mentally, aside from the delusions noted at the beginning of our analysis, a striking feature was the patient’s childishness. While reciting delusions, the patient was overactive and evinced a somewhat childish interest. Arithmetically, Jackson had preserved a fair ability but his apathy and lack of interest interfered with tests, and possibly also with the exercise of memory. As above noted, we were compelled to maintain the suspicion of syphilis throughout despite the attractive hypotheses of traumatic and post-decompressive effects and cerebral tuberculosis. A history of the acquisition of syphilis an unknown number of years before admission entered to strengthen the suspicion of the syphilitic nature of the mental symptoms.

TYPICAL LABORATORY FINDINGS IN NEUROSYPHILIS (Nonne, 1915)

Diagnosis W. R., Blood Serum W. R. 0.22 cc. Blood Serum Spinal Fluid, 1.0 cc. Phase I, Globulin Pleocytosis

PARESIS OR TABOPARESIS POSITIVE IN ALMOST 100% POSITIVE, 85–90% POSITIVE, 100% POSITIVE, 95–100% POSITIVE, ABOUT 95%

TABES (not combined with paresis) POSITIVE, 60–70% POSITIVE, 20% POSITIVE, 100% POSITIVE, 90–95% POSITIVE, 90%

CEREBROSPINAL SYPHILIS POSITIVE, 70–80% POSITIVE, 20–30% POSITIVE ALMOST ALWAYS POSITIVE almost always; NEGATIVE only EXCEPTIONALLY POSITIVE ALMOST ALWAYS

Chart 8

Syphilitic thrombosis. Contours of brain preserved.

83The W. R. proved positive in blood and spinal fluid. The gold sol reaction was of the syphilitic type; 37 cells were found per cmm.; there was a slight amount of globulin and a slight excess of albumin.

We made a diagnosis of Cerebrospinal Syphilis rather than general paresis on account of, first, the slow course of the disease; second, the vascular type of the cerebral insult, hardly typical of paresis; and third, the mild spinal fluid reaction. Treatment will hardly cure the hemiplegia, at least so far as restoration of cerebral tissues lost in the insult is concerned. We were perhaps entitled to consider that, as in the cases of Petrofski (17), O’Neil (19), Robinson (45), the meningitic process could be arrested. Unfortunately, our treatment of 20 injections of salvarsan over a period of 10 weeks, followed by a number of months of bi-weekly injections of mercury salicylate, proved incapable of making any change in the mental and physical picture or in the laboratory findings.

1. Can we explain the apparently poor reaction to treatment of the cerebrospinal syphilis in the case of Jackson by supposing a more deep-seated involvement than the meningovascular involvement indicated by the hemiplegia and the signs in the fluid? Autopsied cases in our experience show focal parenchymatous involvements that have not caused obvious clinical symptoms at any time during the course of the disease. These symptomatically silent lesions may have been present.

2. What is the comparative prognostic value of seizures in paretic neurosyphilis and in such a meningovascular case as that of Jackson? Paretic seizures are often and indeed characteristically recovered from. Moreover, 84autopsies in paretic neurosyphilis characteristically show no gross focal destructive lesions to correspond with the seizures. The paretic seizures are apparently more irritative than paralytic. However, the seizures of the meningovascular group of neurosyphilis are also, though less commonly, recovered from, so that the differential diagnosis on the basis of the outcome of seizures is not safe. Rarely paretic neurosyphilis itself also develops seizures from which no recovery is made.

3. What is the relation of neuropathic heredity to neurosyphilis? The family history of John Jackson is undoubtedly poor, since his father died of diabetes and a paternal uncle was insane; and on the mother’s side, the grandmother died of tuberculosis and an aunt died insane. This general question was more interesting in the days before the syphilitic nature of general paresis and of allied diseases was known. However, we may still hold perhaps that not only syphilis but also various intoxications, especially alcoholism, do flourish upon a neuropathic soil. This question, like that of Krafft-Ebing’s celebrated claim of the relation between syphilization and civilization, needs revision in the light of more extensive applications of the W. R. in larger and larger groups of persons under various community conditions.

The SIX TESTS (serum Wassermann reaction, fluid Wassermann reaction, pleocytosis, gold sol reaction, globulin, excess albumin) are likely to run STRONGER in PARETIC NEUROSYPHILIS (“general paresis”) than in DIFFUSE (especially meningovascular) NEUROSYPHILIS; in particular, the gold sol reaction is likely to prove “paretic” rather than “syphilitic.” The clinical course of paretic neurosyphilis (“general paresis”) is likely to terminate in death within a few years.

Case 15. Pietro Martiro was a well developed and nourished man, 30 years of age, who had been doing erratic things and acting peculiarly for a few weeks before entering the hospital. In the hospital, Martiro proved to be very excitable and given to violence. He had marked delusions of grandeur, saying he was worth many millions of dollars, was the greatest singer in the world, the greatest athlete in the world, and the like.

Physically, there was no disorder except overactivity of some reflexes. The diagnosis of General Paresis offered no difficulties, and it was confirmed by the laboratory tests (positive serum and fluid W. R., “paretic” gold sol reaction, 42 cells per cmm., an excess of albumin, and a positive globulin test).

Treatment: The perfect physique of this case and the extremely brief clinical duration (a few weeks) would naturally suggest a probably favorable outcome. However, cases with marked delusions of grandeur have very frequently proved to be cases with extensive brain tissue loss as shown in certain studies with Danvers material.

In any event, the treatment in this case proved unavailing. Enormous doses of salvarsan, twice a week, aided by mercury and potassium iodid, were given. Although other cases had been helped by such intensive treatment, Martiro went steadily downhill, nor was there the slightest diminution in the intensity of any of the spinal fluid reactions. After 50 injections of salvarsan over a period of 30 weeks without improvement, treatment was discontinued. A few months later, the patient died.

PARETIC NEUROSYPHILIS (GENERAL PARESIS)

PHYSICAL SYMPTOMS

EARLY HEADACHE

VISUAL DISORDER

HYPALGESIA

ADIADOCHOKINESIS

ATAXIA

NASOLABIAL FLATTENING

VOCAL CHANGE

SPEECH DISORDER

WRITING DISORDER

LOSS OF MANUAL DEXTERITY

PUPILLARY CHANGES

REFLEX CHANGES

SEIZURES

LATE: PARALYSIS, CONTRACTURE

Chart 9

PARETIC NEUROSYPHILIS (GENERAL PARESIS)

MENTAL SYMPTOMS

INTAKE IMPAIRED

CONSCIOUSNESS CLOUDED

FATIGUABILITY INCREASED

HALLUCINOSIS RARE

AMNESIA—RECENT! CHRONOLOGY AND STORAGE IMPAIRED. FABULATION

OVER-SUGGESTIBILITY

JUDGMENT IMPAIRED

FANTASTIC DELUSIONS

INSIGHT INTO ILLNESS NIL

EARLY IRRITABILITY OR HEBETUDE

QUICK SHIFTING EMOTION

CHARACTER CHANGE

CONDUCT SLUMP

Chart 10

881. What is the duration of paretic neurosyphilis (“general paresis”)? If we omit the doubtful, early, and prodromal stages and count the beginning of the disease with the occurrence of definite symptoms, we find (Kraepelin) that almost half the patients with pronounced paretic signs die within the first two years of their disease. Kraepelin’s observations upon 244 cases are as follows:

Year: 1 2 3 4 5 6 7 8 9 10 14

Cases: 51 63 52 41 22 4 5 2 2 1 1

The average duration of the disease in months has been calculated as varying from 24 to 32 months. Juvenile paresis runs a slower and more insidious course. The duration of paresis, according to many observers, diminishes with the increasing age of the patient. It is now held that a combination of tabes with paresis does not prolong the duration of the paresis. As noted above in the discussion of Case Harrison (9), our conceptions of the characteristic duration of paretic neurosyphilis must alter with the increase of our knowledge due to the early application of laboratory tests.

2. What is the significance of the term general paresis? The case of Martiro is, of course, a good instance to show that the term is sometimes a misnomer. The characteristic generalized motor incapacity denoted by the term general paresis is shown in patients in the institutions for the chronic insane in their last few months of life. The term paresis is perhaps to be preferred to the term paralysis because the paralysis is not complete but partial; but perhaps the best reason is that the word paresis is a shorter word. When the mental side is to be emphasized, the term paralytic dementia is employed. In this book we have used the term paretic neurosyphilis to mean a more precise statement of the etiology of general paresis (general paralysis, paralytic dementia). The lay term, softening of the brain, like the terms metasyphilis and parasyphilis is in the present phase of our knowledge to be eschewed.

Euphoria in paretic neurosyphilis (“general paresis”). The head, arms and trunk were shaking with mirth; hence, the indistinct outlines of the photograph.

893. If this fatal case be typical of general paresis (for more favorable results, see Part V), what is the toll of deaths from this disease in the community at large? A striking statement may be quoted from Dr. Thomas W. Salmon’s “Analysis of General Paralysis as a Public Health Problem:”

“With the information in our possession at the present time, we are able to state that not fewer than 1000 persons in whom general paralysis is recognized die in New York State every year. Let us compare this with the lives lost from some other important preventable diseases. It means that one in nine of the 6909 men who died between the ages of 40 and 60 in New York last year died from recognized general paralysis and that one in thirty of the 5299 women who died in the same age-period died from this disease.

“The number of deaths from general paralysis in New York last year about equalled the number of deaths from typhoid fever. The following table gives the number of deaths due to the ten most important specific infectious diseases. Of course, deaths in measles, typhoid fever and scarlet fever will be found also under the names of some of the complications of these diseases, but it should be remembered that these primary diseases are not invariably fatal as general paralysis is. Many of the patients with measles who died from bronchopneumonia would have recovered but for this complication, while the paretics with bronchopneumonia would have died even if this complication had not arisen. No attempt is being made to compare the prevalence of general paralysis with that of other diseases—we are trying only to estimate its share in the mortality.

“1. Tuberculosis (all forms) 16,133

2. Pneumonia 9,302

3. Bronchopneumonia 7,217

4. Diphtheria and croup 1,854

5. Influenza 1,381

6. Measles 1,071

7. Typhoid Fever 1,018

General paralysis (recognized) 1,000

8. Scarlet fever 837

9. Whooping cough 818

10. Syphilis 782”

PARETIC NEUROSYPHILIS (GENERAL PARESIS)

CHARACTERISTICS

AMNESIA

QUICK SHIFTING EMOTIONS

CHARACTER CHANGE

CONDUCT SLUMP

NERVOUS DISORDERS

SPEECH DISORDERS

PUPILLARY CHANGES

REFLEX CHANGES

SEIZURES

CEREBROSPINAL FLUID PICTURE

Chart 11

SYPHILITIC PSYCHOSES

SYPHILITIC NEURASTHENIA

GUMMA

SYPHILITIC PSEUDOPARESIS

APOPLECTIC CEREBRAL SYPHILIS

SYPHILITIC EPILEPSY

SYPHILITIC PARANOIA

TABETIC PSYCHOSIS

HEREDITARY

PARESIS

Kraepelin, 1910

Chart 12

TABOPARETIC NEUROSYPHILIS (“taboparesis”) is CLINICALLY a combination of the symptoms of TABES DORSALIS and those of GENERAL PARESIS. The COURSE of TABOPARESIS is likely to be from a characteristic tabes dorsalis (often of years’ standing) to a characteristic general paresis; the ultimate paretic picture is likely to retain various characteristics of tabes. The LABORATORY TESTS in the paretic phase are characteristic of ordinary (non-tabetic) general paresis. The PROGNOSIS, after the paretic phase has arrived, is apt to be that of general paresis.

Case 16. Joseph Sullivan, a waiter, 50 years of age, sought assistance at the Psychopathic Hospital voluntarily. His complaint of severe and lancinating pains in the legs, difficulty with his gait, and a feeling of constriction about the waist, was forthwith suggestive of tabes dorsalis. He was a rather poorly nourished, white-haired man, with a drooping of the left side of the face. The pupils reacted sluggishly to light, the right somewhat better than the left. A marked Romberg reaction could be demonstrated. Ataxia in walking was marked. There was some incoördination of the hands, considerable tremor, and writing was poorly performed. The ankle-jerks and knee-jerks were absent. On the whole, the diagnosis of Tabes Dorsalis was clear enough.

The most appealing situation was mental. Sullivan was exceedingly apprehensive about his condition on the ground that it was growing progressively worse; if it was to get worse, Sullivan feared he would commit suicide. From his own account, he had become irritable, quick-tempered, and often unreasonable. As usual in these cases, the question arose whether the depression was psychopathic or natural.

TABETIC SYMPTOMS AND SIGNS IN ORDER OF THEIR FREQUENCY

ANALYSIS OF 250 CASES

PER CENT

1. ROMBERG SIGN 96.4

2. ABSENT KNEE-JERKS 90.0

3. LANCINATING PAINS 88.4

4. STAGGERING GAIT 87.2

5. ARGYLL-ROBERTSON PUPIL 80.0

6. ATAXIA IN UPPER EXTREMITIES 68.2

7. SPHINCTER DISTURBANCES 67.6

8. SENSORY DISTURBANCES 58.2

9. VISUAL DISTURBANCES 43.6

10. PARESTHESIA AND NUMBNESS OF FEET AND LOWER EXTREMITIES 42.8

11. GIRDLE SENSE 31.2

12. PTOSIS OF EYE-LIDS 23.2

13. PARESTHESIA OR NUMBNESS IN HANDS OR UPPER EXTREMITIES 13.6

14. STRABISMUS 12.0

15. VISCERAL CRISES 12.0

16. LOSS OF SEXUAL DESIRE 11.5

17. CHARCOT JOINTS 9.2

18. VERTIGO 4.0

19. MAL PERFORANS 3.2

20. PAIN IN JOINTS 2.8

21. RECTAL TENESMUS 2.8

22. MENTAL DEGENERATION (other than paresis) 2.4

23. HEMIPLEGIA 2.4

24. VESICAL TENESMUS 2.0

25. DIFFICULTY IN ARTICULATION 2.0

26. DEAFNESS 1.2

27. ANOSMIA 0.8

Baldwin Lucke.

Chart 13

94While in the hospital things shortly came to a crisis. In the midst of a fit of depression, Sullivan attempted suicide by beating his head against the wall. Whether this attempt could be regarded psychopathic, however, remained in question. Sullivan had been drinking very heavily although he had stopped about six weeks before admission, fearing that the alcohol was causing a development of symptoms. The remedy was almost worse than the disease because he then became more nervous, lost his appetite, and had a marked insomnia.

According to the patient’s own history, he had had several attacks of gonorrhœa and a syphilitic infection at the age of 19; that is, some 31 years before admission to the hospital. However, the first neurological symptoms of which the patient was aware came about 27 or 28 years after infection, namely, 3 or 4 years before admission, when facial paralysis developed. At that time, he had suddenly felt a peculiar sensation in the throat and became unable to swallow for a time. His voice remained hoarse and low for some time, and his face began to droop. The lancinating pains and the ataxia also dated back several years.

1. How shall we evaluate the mental symptoms? The prognosis of tabes dorsalis is relatively good so far as life is concerned, and it might even be possible for Sullivan by training to remain capable of being a waiter. The manual incoördination was not marked, and possibly the manual tremor was in part due to alcohol. Accordingly, the mental symptoms, such as emotional lability and memory defect, were in the foreground of attention. In point of fact, the laboratory examinations showed positive W. R. in the serum and the spinal fluid, which latter also contained 60 cells per cmm., positive globulin, and an excess of albumin. The Diagnosis made was that of Taboparesis, meaning thereby a tabes associated with appropriate symptoms of a mental nature.

2. How shall the term taboparesis be used? Some use the term, as we feel erroneously, for instances of general paresis which happen to show crural areflexia (absence of knee-jerks). We feel that the best usage of the term is for instances in which well-defined symptoms of tabes (as well as of paresis) are present, namely, characteristic ataxia, lightning pains, and the like. If the 95term is used more loosely, as above mentioned, then practically every case of general paresis might perhaps be termed taboparesis, since almost every case of paresis does show involvement of the cord as well as of the cerebrum. Such involvement may lead to hyperreflexia, hyporeflexia, or areflexia according to the localization of the process. In true taboparesis, in which there is a commingling of the features of tabes with those of paresis, we should find the posterior roots of the spinal cord affected. The spinal lesions of paresis itself are more apt to be intraspinal; that is, confined to the nervous system within the pial investment.

3. Bearing in mind that Sullivan was a waiter, what shall be said about the infectivity of these cases? It is counted as a rule as negative, since there are no open spirochete-bearing lesions. The longer the period since infection the less, as a rule, is the chance of contagion in syphilis; and as tabes and paresis occur fairly late in the disease, the infectiousness at this stage is practically negligible.

4. Of what differential value is the insight shown by Sullivan into the nature of his symptoms? Kraepelin remarks that a genuine insight into the nature of the disease does not as a rule occur in paresis. At the beginning of the disease, there may sometimes be a correct understanding of the nature of the disease and of its probable outcome; but the presence or absence of insight into the fact of mental disease is by no means a differential sign of practical value.

5. What is to be said of the occurrence of depression and excited states in paretic neurosyphilis? A variety of classifications of sub-forms of paretic neurosyphilis have been propounded. Kraepelin, for example, deals with four: the demented, depressive, expansive, and agitated forms, but remarks that the division is merely convenient for exposition. The institutional intake does not accurately represent the distribution of cases. Under psychopathic hospital conditions with the relatively easy resort to such institutions, the number of quiet cases increases; under the less advanced conditions in Heidelberg, Kraepelin took in 53% demented paretics as against 56% at Munich (73% women) under the easier conditions of admission. The admissions of demented paretics varied from 37 to 56%. The variations depend much upon the facility with which the cases can be brought to institutions. Where admission 96is beset with various legal restrictions, the quiet and demented cases are more apt to be treated for long periods at home. The depressive type of paretic neurosyphilis forms a much smaller group, according to Kraepelin, as only about 12% of his Heidelberg admissions were of this type, and still fewer of his Munich admissions. Other authors give percentages as high as 16 and 19. The so-called expansive group is larger, Kraepelin finding 30% of his Heidelberg cases to be of this group, and 21 to 22% of his Munich cases. The rarest sub-form of paretic neurosyphilis is the agitated form: 6% of Kraepelin’s Heidelberg admissions; 14% among males and 5% among females in his Munich admissions, where the diagnosis of agitated paresis was entered on somewhat broader lines. French authors (Sérieux and Ducaste) have enlarged the number of sub-forms of paretic neurosyphilis as follows: Expansive 27%; sensory 24%; demented 24%; persecutory 3%; depressive 2%; circular 7%; hypochondriacal 7%; and maniacal 6%.

DIFFUSE (meningovasculoparenchymatous) NEUROSYPHILIS may look precisely like PARETIC NEUROSYPHILIS (“general paresis”) at certain periods of clinical and laboratory examination.

Case 17. The police found Gregorian Petrofski crouching on his knees on a Boston sidewalk, attempting to take pickets off a fence. Petrofski knew little English; he said that he had slept in Poland the night before. He did not appear to be alcoholic.

When he was examined, through an interpreter, he told how he had been in America two days, and in Boston two years; that he was at the present time in Poland, and that his brother had brought him to the hospital and left him there.

The physical examination showed Petrofski to be well developed and nourished. His pupils were somewhat dilated and reacted somewhat slowly to light and accommodation. Neurologically, there was nothing else abnormal found upon systematic examination although, through lack of coöperation, sensory and coördination tests proved difficult if not impossible. There was a large ulcer on the under surface of the glans penis, with several small smooth scars on the upper surface. There was a purulent discharge from the external meatus. There were exostoses of both tibiae.

The initial diagnosis had to consider uremia and diabetes, which could be easily excluded on examination. Alcoholism was excluded through absence of alcohol on the breath. There remained such diagnoses as epilepsy, some post-traumatic condition, or meningitis, to say nothing of the hypothesis of syphilis raised by the tibial exostoses and the lesions of the penis. The hypothesis of trauma was given up, as well as epilepsy and meningitis upon the data of the lumbar puncture. The spinal fluid proved to be clear but with enormous amounts of globulin and albumin, 80 cells per cmm., a “paretic” gold sol reaction, and a positive spinal fluid 98W. R. (the serum W. R. was also positive). Accordingly, it was clear that the case was one of neurosyphilis.

Treatment was instituted with injections of mercury salicylate, a grain and a half twice a week, and potassium iodid. After some weeks, diarrhoea and salivation with marked symptoms of mercury poisoning set in; the treatment was suspended, but later re-instituted. In a few weeks Petrofski was apparently quite well, the spinal fluid tests had all become negative, as had the serum W. R.

Petrofski now began to pick up a good deal of English, and gave a consistent narrative of his past life, although the period just prior to and during his early stay in the hospital has remained blank. Without further treatment Petrofski has remained well for over a year.

1. Does the “paretic” gold sol reaction mean general paresis? In connection with this general question, a brief summary of the significance of the gold sol reaction in this group may be made. (1) Fluids from cases of general paresis in the vast majority of cases will give a strong and fairly characteristic reaction, especially if more than one sample is tested. (2) Very rarely general paresis fluid will give a reaction weaker than the characteristic one. (3) Fluids from cases of syphilitic involvement of the central nervous system other than general paresis often give a weaker reaction than the paretic, but in a fairly high percentage of cases give the same reaction as the paretics. (4) Non-syphilitic cases may give the same reaction as the paretics; these cases are usually chronic inflammatory conditions of the central nervous system. (5) When a syphilitic fluid does not give the strong “paretic reaction” it is presumptive evidence that the case is not general paresis, and this test offers a very valuable differential diagnostic aid between general paresis, tabes, and cerebrospinal syphilis. (6) The term “syphilitic zone” is a misnomer, as non-syphilitic as well as syphilitic cases give reactions in this zone, but no fluid of a case with syphilitic central nervous system disease has given a reaction out of this zone, so that the finding may be used negatively; and any fluid giving a reaction outside of this zone may be considered non-syphilitic. (7) Mild reactions may occur without any evident significance, while a reaction of no greater strength may mean marked inflammatory reaction. (8) Tuberculous meningitis, brain tumor, and purulent meningitis fluids characteristically, though not invariably, give reactions in higher dilutions than syphilitic fluids. (9) The unsupplemented gold sol test is insufficient evidence on which to make any diagnosis, but used in conjunction with the W. R., chemical and cytological examinations, it offers much information, aiding in the differential diagnosis of general paresis, cerebrospinal syphilis, tabes dorsalis, brain tumor, tuberculous meningitis, and purulent meningitis. (10) We believe that no cerebrospinal fluid examination is complete for clinical purposes without the gold sol test.

FREQUENT SYMPTOMS IN DIFFUSE AND VASCULAR NEUROSYPHILIS

(“CEREBRAL” AND “CEREBROSPINAL SYPHILIS”)

PUPILLARY DISORDER

HEADACHE

VERTIGO

INSOMNIA

DROWSINESS

CHANGE IN DISPOSITION

Irritability Slow thinking

SEIZURES

PARALYSES

Permanent Transient

APHASIA

HEMIANOPSIA

SENSORY DISTURBANCES

GASTRIC CRISES

SPHINCTER DISTURBANCES

INTRACRANIAL PRESSURE SYMPTOMS

POLYURIA, POLYDIPSIA, GLYCOSURIA

MÉNIÈRE’S SYNDROME

NYSTAGMUS

Chart 14

100See Appendix B for technical details.

2. What is the relation of the tibial exostosis to neurosyphilis? The syphilographers have always stressed the tibial lesions in the diagnosis of syphilis. Although not so much attention has been paid to these and kindred osseous lesions in neurosyphilis, yet we have frequently found such lesions and they afford an important auxiliary means of diagnosis.

101

A POSITIVE SERUM Wassermann reaction with a NEGATIVE FLUID Wassermann Reaction may be found in NEUROSYPHILIS, particularly in VASCULAR NEUROSYPHILIS: the remaining signs in the fluid, although frequently positive, may even be negative.

Case 18. Frederick Wescott was a promoter, an elderly looking man of 60 years. His health had been failing for 18 months. There had been shortness of breath, dizziness, a tired feeling, inability to “get the words he wanted,” and forgetfulness of names. About eight weeks before examination, Wescott had had a convulsion, following which he had been unable to express himself at all well. This convulsion was not accompanied by loss of consciousness. Besides a marked motor aphasia, there was agraphia.

Physically, Wescott showed arteriosclerosis and a blood pressure of 135 systolic, but, except very lively knee-jerks, no other reflex disorders or anomalies were discovered. In particular, the pupils reacted fairly well.

There was, perhaps, no special reason to implicate syphilis in the case, yet Wescott gave a history of syphilis at 35 years. The W. R. of the blood serum proved positive; that of the spinal fluid was negative, and the albumin was but slightly increased; there was a very slight amount of globulin, and there were 16 cells per cmm. in the fluid. The gold sol reaction suggested syphilis.

We felt entitled to make a diagnosis of Syphilitic Cerebral Arteriosclerosis, regarding the convulsion or seizure eight weeks before as due to a vascular insult. The laboratory picture in the spinal fluid in Wescott’s case seems to be rather characteristic of this group of syphilitic arteriosclerotics.

1. What is the reason for the negative spinal fluid W. R.? The theory would be that the syphilitic lesion is localized in the vascular system and that the parenchyma 102is only secondarily, if at all, involved. The W. R. producing bodies are accordingly not found in the fluid.

2. How frequently are several of the spinal fluid tests negative, while others are positive? Whereas, clinically speaking, the five tests in the spinal fluid (W. R., globulin reaction, excess albumin, pleocytosis, and gold sol reaction) are each indicative of a pathological condition in the central nervous system, yet a specially intensive study of the distribution of these tests has shown that they are prone to occur independently. Consequently, we must concede that they do not all represent the same inflammatory products and chemical conditions. The W. R. producing bodies, the gold sol reaction producing bodies, as well as the globulins and albumins, have been proved to be separate. Special work has also shown that these tests disappear under treatment at different rates. There is, unfortunately, no doubt that the rate and intensity, presence or absence, and the order of disappearance of these tests in either treated or untreated cases, do not at all parallel the clinical conditions of the patients.

3. What is the prognosis in vascular neurosyphilis, such as in the case of Wescott? The prognosis is identical with that of cerebral arteriosclerosis in general, that is to say, bad, but with frequent periods of improvement. In the neurosyphilitic type of arterial disease thromboid formation is frequent. Where the lesion is chiefly perivascular infiltration, rather than disintegration of the vessel wall, improvement may very well occur as a result of treatment. Wescott showed slight improvement under treatment. He has already lived two years since his first convulsion, and three and a half years since the onset of symptoms.

103

DIFFUSE NEUROSYPHILIS (so-called “cerebrospinal syphilis”) is often marked by SEIZURES.

Case 19. Agnes O’Neil, an unmarried woman of 28 years, was first examined five weeks after the initial symptoms. It appears that she had had certain seizures, with unconsciousness and twitching of the limbs (otherwise not well described), followed by confusion of mind and sometimes by a weakness of the left side and a difficulty in speaking. Headache had been almost constant, as well as pains in the arms and legs.

Physically, both in general and neurologically, there were no signs or symptoms; mentally, we could discover no symptoms. Syphilis was denied, although possible exposure to syphilis was admitted.

The diagnosis of some form of organic brain disease was clear with the picture of convulsions followed by slight aphasia with headaches and limb pains. With onset at 28, the most frequent cause for such epileptiform seizures is certainly syphilis. Examination of the blood and spinal fluid showed a positive W. R., in both. The albumin was also somewhat increased. The clinical picture suggested a fairly generalized meningitic involvement.

The prognosis in such cases of generalized meningitic involvement is in general good, and this principle was illustrated in the O’Neil case, in which the symptoms soon disappeared under intensive antisyphilitic treatment. In fact the spinal fluid W. R. became negative in the course of four weeks. The blood serum W. R., however, has remained positive despite eight months of active treatment.

104

CONDITIONS IN WHICH CONVULSIONS OCCUR

NEUROSYPHILIS

HYSTERIA

EPILEPSY MAJOR (Grand Mal)

EPILEPSY MINOR (Petit Mal)

DEMENTIA PRAECOX

TOXIC CONDITIONS:

Asphyxia, Uremia, Alcohol, Absinthe, Lead, Mercury, etc.

ORGANIC BRAIN LESIONS

Apoplexy, Meningitis, Intracranial Growths

STOKES-ADAMS DISEASE

MALINGERING

DISSEMINATED SCLEROSIS

Chart 15

1051. Are certain cases of syphilitic epilepsy really cases of Jacksonian epilepsy? As a matter of nomenclature, Jacksonian cortical epilepsy is usually the result of a focal and circumscribed irritative lesion in the cortex. Gumma, local syphilitic meningitis, and syphilitic vascular lesions, as well as scars consequent upon the latter, are among the causes of Jacksonian epilepsy, along with such other focal lesions as trauma, tumor abscess, tubercle, and the like. Even non-syphilitic Jacksonian epilepsy has been observed from time to time in cases of diffuse intracranial pressure. Jacksonian attacks also have been found in so-called genuine epilepsy. Accordingly, we must not conclude from the occurrence of Jacksonian convulsions, even though in a proved syphilitic case, that the convulsions in question are surely due to a focal lesion, for they may be due to diffuse syphilitic lesions.

2. What is the significance of aphasia in Agnes O’Neil? Aphasia is not a characteristic symptom in ordinary Jacksonian epilepsy, but the aphasia is another sign of focal lesion and forms an added argument against the diagnosis of genuine or idiopathic epilepsy. See also discussion of aphasia in paretic neurosyphilis under Case Levenson (22).

3. What is the behavior of the serum W. R. and the spinal fluid W. R. under systematic treatment? Sometimes, as in this case, the serum W. R. remains positive and the fluid W. R. becomes negative; but in other equally well-defined cases, the reverse holds true, and the serum W. R. reaction becomes negative whereas the spinal fluid reaction remains positive. The obvious conclusion is that we cannot always be sure even by faithful tests of either the serum or the fluid alone, whether the treatment has succeeded in abolishing the laboratory signs.

4. Can this case be regarded as one of cure? Not by the definition adopted in this book or by the syphilographers who take into account not only the nervous system but the body which contains it. To be sure, the spinal fluid of Agnes O’Neil is now entirely negative and she is clinically free from symptoms; yet from the broad standpoint of syphilis therapy in general, this patient is not cured, as is evidenced by the positive serum W. R.

106

PARETIC NEUROSYPHILIS (“general paresis”) is often marked by SEIZURES.

Case 20. Lester Crane, a plumber, 37 years of age, came to the hospital with a slow and defective speech. Moreover, there seemed to be some mental disorder since his answers to questions were not always relevant. It appeared that he was seeing bugs on the wall.

Physically, Crane was a well developed and nourished man, with overactive knee-jerks and a Babinski reaction on the left side.

It developed that there was an impairment in hearing. The pupils reacted well both to light and to distance. The patient was very restless and smiled in a silly fashion. His memory was decidedly defective in all spheres, and he was very slow in the intake of ideas.

The plumber’s wife said that, at about the age of 23 or 24, he had a spell of confusion lasting two or three days, with peculiar conduct, unintelligible talk, and a good deal of weeping. The medical diagnosis at that time took into account the fact that Crane was a plumber and was “lead encephalopathy.”

However, according to his wife, Crane had acquired chancre at about 26 years, was treated mercurially for about three years and declared well. He had remained well up to about 18 months before entrance, when, without previous warning, the patient had a convulsion with the continuous movements for about half an hour. He was semi-conscious for about 18 hours and vomited continuously. There was amnesia for the whole affair on regaining consciousness. In a week’s time, Crane was entirely well. But six weeks later there was another convulsion. Upon removal to a hospital, the diagnosis of general paresis was made, and the patient was given the Swift-Ellis intraspinous treatment. This seemed to be very successful, and the patient discontinued treatment after 14 weeks (during which time there had 107been seven treatments) on the ground that he was entirely well.

However, after discontinuing treatment, there was another convulsion in about a month, and further convulsions occurred once a month. For six months, however, the patient took no treatment, but finally returned to the hospital and was given mercury. This treatment appeared to suspend convulsions again for three months, but at the expiration of six months, the patient had three convulsions in one day, and several more during the following days. After the last of these convulsions, there had been numbness on the right side of the body and considerable headache.

The diagnosis of Paretic Neurosyphilis (“general paresis”) is borne out by the laboratory tests. The W. R. of the blood serum was, to be sure, negative, but the W. R. of the spinal fluid was positive, and there was a “paretic” type of gold reaction, together with other laboratory signs.

The case well demonstrates that group of paretic cases in which convulsions periodically occur, leaving the patient worse after each convulsion. Treatment with salvarsan was instituted, and mercury and iodid was given by mouth. During the period of eight months which have now elapsed since the beginning of this treatment, there have been no convulsions; there has been a great improvement in the memory, the hearing has improved, the W. R. in the spinal fluid is much less intense, the gold sol test has become negative, and the other tests are all less intense.

The patient, however, has not been entirely well, for in place of the generalized convulsions, he has had minor seizures, beginning as a rule with a tingling sensation in the right hand, extending up the arm, down the trunk and leg, and through the right side of the face, with a bitter sensation on the right half of the tongue. The patient maintains that this sensation is absolutely confined to the right half of the body (in this connection we may recall case Morton (1), in which there was also a hemiplegia together with other apparently hysterical symptoms at several times during the long course of a disease with abundant structural correlations). During these minor seizures, the patient is unable to talk, although he does not lose consciousness and is entirely aware of everything going on about him. These attacks have of late been growing somewhat less frequent.

108

LOSS OF DEEP REFLEXES

NEUROSYPHILIS

NEURITIS

(alcohol, diabetes, diphtheria, lead, arsenic, tubercle, cachexia, etc.)

Peripheral nerves sensory or motor

PERIPHERAL NERVE PALSIES

TEMPORARILY FROM COMPRESSION BY TOURNIQUET

FRIEDREICH’S ATAXIA

SUBACUTE COMBINED DEGENERATION OF POSTERIOR AND LATERAL COLUMNS

Posterior column disease

FOCAL LESION IN GRAY MATTER OF CORD

INFANTILE PARALYSIS (ACUTE ANTERIOR POLIOMYELITIS)

PROGRESSIVE MUSCULAR ATROPHY

(chronic anterior poliomyelitis)

Anterior cornua of cord

AMYOTROPHIC LATERAL SCLEROSIS

SYRINGOMYELIA

THROMBOSIS OF ANTERIOR SPINAL ARTERY

LANDRY’S PARALYSIS

Anterior cornua and peripheral motor nerves

MYOPATHIES

(pseudohypertrophic and atrophic types)

MuscLe itself

AMYOTONIA CONGENITA

FAMILY PERIODIC PARALYSIS

(during attacks)

INCREASED INTRACRANIAL PRESSURE

(especially hydrocephalus and tumors of posterior fossa)

PNEUMONIA

IMMEDIATELY AFTER ATTACK OF MAJOR EPILEPSY

(post-epileptic coma)

TOXIC COMA

(uremia, morphine, etc.)

DURING SPINAL ANESTHESIA

COMPLETE TRANSVERSE LESION OF CORD

Purves Stuart

Chart 16

1091. What is the cause of the negative serum W. R.? It is claimed that 3 to 5% of all cases of general paresis yield a negative blood serum. In this particular case, there had been considerable treatment, including some Swift-Ellis treatment, so that it may be that this treatment had reduced a formerly positive blood serum W. R. to a negative one.

2. What is the nature of the typical seizures of general paresis? The most frequent seizures are epileptiform and bear a general resemblance to cortical epilepsy; but more rarely these seizures resemble the ordinary epileptic attack or consist of a violent general shaking of the whole body. A variety of initial minor disorders usher in the attacks: the temperature is often increased. The attacks are over after one or at most after a few hours. Kraepelin speaks of one that lasted 14 days. Sometimes a status paralyticus develops, suggestive of the status epilepticus. Another rarer form of characteristic seizure is the apoplectiform, which can hardly be told from an ordinary stroke, and may be followed by the usual post-apoplectic phenomena. A good many of the strokes leading to sudden death in middle life are probably cases of neurosyphilis although often set down as early arteriosclerosis of a non-syphilitic nature. Besides the epileptiform and apoplectiform seizures, there are certain seizures of a less definite and complete nature, ranging from simple fainting spells, dizzy spells and petit mal attacks, to various special forms of irritative muscular contractions and temporary speech disorders. Sometimes these attacks occur with complete preservation of consciousness. Transient paresthesias, visual field defects, and especially attacks of vomiting, which, according to Kraepelin, may precede paresis by years (of course in this connection gastric crises of tabes must be thought of), may be counted as sensory seizures.

3. What is the proportion of paretic cases developing seizures? Figures vary from 30 to 90%. According to Kraepelin, seizures occurred in 30 to 40% of his cases at Heidelberg; he was of the impression that treatment in bed had reduced the number of seizures. 65% of 110paretics admitted to Munich (under very free conditions of admission) were determined to have shown seizures before their admission to the hospital. Seizures are said to be somewhat more frequent in men than in women. These paretic seizures are not due to either hemorrhages or vascular plugging—at least in the vast majority of cases—and must be ascribed to the effects of microscopic injuries.

4. What is the effect of seizures upon the future course of paretic neurosyphilis? The current idea as expressed, for example, by Mercier, is that “immediately after each crisis the patient is much worse than he was before it, and thereafter there is some improvement, but he never improves up to the point at which he was before the occurrence of the crisis.” That is, “The course of the disease is one of sudden plunges, each deeper than the last, each followed by a gradual recovery that is less complete than the recovery from the previous plunge.”

5. During what period of the disease are seizures most common? Late in the disease many cases have convulsions, even though there were none for the first year or two. In other cases the convulsion is the first indication of paresis.

111

DIFFUSE (non-paretic) NEUROSYPHILIS (“cerebrospinal syphilis”) is often marked by APHASIA.

Case 21. Martha Bartlett, a woman of 40 years, was brought to the Psychopathic Hospital aphasic, or at least unable to talk distinctly enough to be understood, or even to give name and address. The police had found her wandering aimlessly about the streets. Although she was well-dressed, she was mud-bespattered and apparently had not changed her garments for several days. It shortly developed that the patient, although unable to express herself either in words or by writing, could understand everything that was said to her and could indicate by the monosyllables yes or no whether she agreed or disagreed with statements made. It was thus determined that she was pretty well oriented. She was able to understand both speech and printed words. Although she approximated more than is at all common a pure type of motor aphasia, it appeared that there was a slight involvement on the sensory side, especially in the sphere of visual imagery.

Neurologically, the patient showed moderate strabismus, slight deviation of the tongue to the right, and considerable tremor on protrusion of the tongue. The right side of the palate hung lower than the left. The ankle and arm reflexes were possibly more active on the left side, and the left grasp was somewhat better than the right. Both knee-jerks were active, but again the reflex on the left side was more active than the right. No other abnormalities of reflex were determined. There was no Rombergism but the gait was somewhat ataxic. For the rest, the physical examination was normal. The blood pressure was 120 systolic, 85 diastolic.

112

CONDITIONS IN WHICH SPEECH DEFECT IS FOUND

NEUROSYPHILIS

HYPOGLOSSAL PARALYSIS

FACIAL PALSY

PARALYSIS OF PALATE (Post-Diptheritic)

BULBAR PALSY

PSEUDOBULBAR PALSY

MYOPATHY—FACIO-SCAPULO-HUMERAL TYPE OF LANDOUZY AND DEJERINE

MYASTHENIA GRAVIS

FRIEDREICH’S ATAXIA

LARYNGEAL TABES

ALCOHOLIC INTOXICATION

POST HEMIPLEGIC

LENTICULAR DISEASE

BILATERAL ATHETOSIS

MULTIPLE SCLEROSIS

DEAF MUTISM

PARALYSIS AGITANS

CHOREA

STAMMERING

TICS

HYSTERICAL APHONIA

Chart 17

113The ready suspicion was that the case was one of apoplexy of slight degree with post-apoplectic phenomena. Upon investigation, this suspicion was confirmed since it appeared that Mrs. B. had been apparently quite well until about six months before admission, when without particular warning she began to act strangely and promptly fell into a series of convulsions. These convulsions would begin with twitchings of the face, and then spread throughout the body. There would be a period of unconsciousness for two or three hours. It is not certain how many of these convulsive seizures the patient had. At all events she is reported to have recovered therefrom completely, remaining well for three months; whereupon, suddenly, while visiting a friend, she suffered a paralysis of the left side of the body. She remained dazed and had hospital treatment for about a week. Ever since this left-sided paralysis, the aphasic condition above described has persisted.

Such a phenomenon has often been dismissed in the past as due to an early arteriosclerosis, but most neurologists and internists of today would look beyond the diagnosis of mere arteriosclerosis and consider syphilis. The only suggestive feature in the case, aside from the post-apoplectic reflex disorder and spastic phenomena, is the irregularity and diminished light reaction of the pupils. Our suspicions were confirmed by the positive serum W. R. The W. R. of the spinal fluid proved, however, to be negative. There was a moderately strong gold sol reaction of the syphilitic type. There was a slight excess of albumin, and there was an exceedingly slight amount of globulin. There was but one cell per cmm.

On the whole, it would seem best to consider the case of Mrs. Bartlett to be one of Cerebral Arteriosclerosis of Syphilitic Origin, and a case in which there is no evidence of meningitis or meningoencephalitis.

1. What is the explanation of the negative spinal fluid W. R.? It may be that none of the W. R. producing bodies have gone over into the spinal fluid. It has been shown by the work of Weston that the W. R. producing body is not identical with the bodies responsible for the other tests in cerebrospinal syphilis. Moreover, it has been clearly shown that these several tests of the spinal fluid do not run at all parallel with one another. Especially is it true that the chemical tests do not correspond at all with the degree or nature of the pleocytosis. On the whole, when involvement of the nervous 114system is entirely vascular, it is not only theoretically proper but also practically common, to find a spinal fluid negative to several tests.

2. Omitting consideration of the syphilitic gold sol of this case, what conclusion could be drawn from the albumin and globulin findings? It would not be warrantable to assume syphilis since it is a common finding after cerebral hemorrhage due to non-syphilitic arteriosclerosis to find excess albumin and also globulin in the spinal fluid. Occasionally, also, pleocytosis occurs in cases of cerebral hemorrhage even when the hypothesis of an active meningitis can be excluded. We may recall in this connection the pleocytosis in so-called meningitis sympathica of certain brain tumors. (See also the case of Milton Safsky (48), a case of brain tumor in which there was an excess of albumin, a large quantity of globulin, and a pleocytosis of 146 cells per cmm.)

3. What can be expected from treatment in these cases of vascular cerebral syphilis? The condition offers very little opportunity for therapeutic results. However, antisyphilitic therapy is indicated to prevent if possible further progress of the lesions. Since the lesions are, however, vascular, and since it must remain a question how far these vascular lesions are due directly to spirochetal action, and since in any event it may be difficult to reach the spirochetes thus active, perhaps it is best to place most reliance on potassium iodid. In any event, potassium iodid should be given. Salvarsan and mercury are also indicated. It is common to warn against administration of large doses of salvarsan in this type of case on the ground that further vascular ruptures may be produced. (See Friedberg, 108.)

4. If we conclude that the aphasia of the Bartlett case is due to vascular disease, can we conclude a relation between this vascular disease and vascular tension? It is not safe to draw such a conclusion. The Bartlett case itself showed low blood pressure. To be sure, some cases of neurosyphilis show high blood pressure from which one draws the à la mode clinical conclusion to the effect that the kidneys are probably involved in the arteriosclerosis; but other cases do not show a high blood pressure but may in fact show a low blood pressure. The vascular disease doubtless responsible for the aphasia in the Bartlett case is probably not at all an effect of blood pressure conditions, but is, on the contrary, an effect of local syphilitic vascular lesions.

115

PARETIC NEUROSYPHILIS (“general paresis”) is often marked by APHASIA.

Case 22. Meyer Levenson, a traveling salesman of 36 years, had for the last two or three years been undergoing a change of disposition, quite interfering with his work. He had begun to take unreasonable aversions to people, had become irritable and emotionally depressed, and often fell to weeping without cause.

About nine months before hospital observation, it seems that a trunk-cover had fallen on Levenson’s head, and there is some question as to whether he did not have a convulsion at that time. However, a month later he had a definite seizure, followed by speech disorder, a slight paralysis, and a staggering gait. Four weeks later, however, he had gotten over these post-convulsive difficulties and had gone back to work.

At his work, he became tired easily, his gait and speech did not seem entirely normal, and there was a considerable memory disorder. After five more months, another attack of a convulsive nature, with twitching of hands and face and tongue-biting occurred, and the attending unconsciousness remained for two days. Again improvement followed, though without ability to return to work. Four (?) months later there were several severe convulsions and Levenson would remain unconscious for a day or two at a time. Restlessness, irritability, and irrational talking followed.

Physically, the patient was fairly well developed and nourished; blood pressure 168 systolic, 68 diastolic; pupils reacted very sluggishly to light. There was a marked motor aphasia, which the patient recognized as a speech difficulty. On the whole, however, Levenson was very euphoric and was entirely sure that he was improving and would surely get well.

Shortly after entrance, Levenson had a severe convulsion, with unconsciousness. The movements were mainly on the right side of the body, and there was a post-convulsive weakness 116of the right side for several days, followed by a slow recovery of strength.

The course of the disease—convulsions followed by improvement—is very characteristic of a paretic onset. The laboratory findings were in all respects confirmatory. It was rather striking that a permanent motor aphasia followed the convulsions in this case, since the seizures of paresis do not in the vast majority of cases leave permanent paralyses. The course of the disease continued to show convulsions, which would in each instance leave him at a lower terrace of capacity than had been before shown. The patient died four years after the onset of symptoms of a general asthenia. With the exception of the permanent motor aphasia, this case might be regarded as a fairly typical one of general paresis.

1. What is the general nature of speech disorder in paretic neurosyphilis? Speech disorder is, along with the pupillary changes, one of the most important clinical symptoms in paretic neurosyphilis. There are aphasic and articulatory disturbances. The aphasia that accompanies paretic seizures is of a transient nature as a rule. A case with such long-standing motor aphasia as shown by Levenson is not common. Paraphasia, with incorrect naming of objects, may last longer. The so-called “sticking” phenomenon is often observed.

Word-deafness is said to be rarer but is difficult to test on account of the patient’s dementia. Agrammatism (incapacity to form correct sentences) is sometimes observed. But the most characteristic disorder is in the syllabic composition of words. Syllables are left out (“medaltricity” for medical electricity), or fused (“exity”), or doubled (“electricicity”). Besides the central speech disorders of which the above are examples, there are disorders in articulation, which at first occur as a consequence of paretic seizures or in states of excitement, but later become permanent. These are divided into paretic and ataxic disturbances.

2. What is the structural basis of these forms of aphasia? It is believed that they are due to microscopic changes, not to coarse destructive lesions.

BROOKLINE, MASS.

BROOKLINE, MASS. Mss. of Levenson, case 22. Paretic neurosyphilis. Tremor, misspelling. Metathesis of letters (Bk, not Br) omission of letters (Book).

God save the Commonwealth of Massachusetts Mss. of Safsky, case 48, brain tumor. Tremor not marked. Misspelling, omission of letters. Wrong letters (h in hweth).

Mss. of Halleck, case 31, cervical tabes. No brain disorder. Pen-holding and bearing on difficulties. Crowding of phrases result of ataxia.

Mss. of Collins, case 61, paretic neurosyphilis. One misspelling (-chussetts); not psychopathic? Characteristic tremor.

117

REMISSIONS of identical appearance occur in PARETIC and in DIFFUSE (non-paretic meningovascular) NEUROSYPHILIS.

Case 23. Thomas Donovan, a merchant 44 years of age, acquired syphilis according to his own story at the age of 31, and he was at that time treated at a well-known watering-place with mercurial injections. Later he continued treatment under his family physician, and at 34 was pronounced cured. However, four years later—that is seven years after his initial infection and in his 38th year—he had his blood examined and it proved positive. He was accordingly treated by salvarsan and his W. R. became negative. The story did not end there, however, for at 43, mental symptoms appeared of the nature of depression and a diagnosis of paresis was made. He was released from the institution against advice at that time, and without treatment, made a partial recovery.

A sudden outburst of violence brought Mr. Donovan to the Psychopathic Hospital; he was very surly, combative, and difficult to manage, standing 6′ 2″, and weighing 210 pounds. He was oriented only fairly well and his surliness was streaked with humor. He facetiously said that the Psychopathic Hospital was the largest hospital in the country, and that it was, in fact, a horse hospital; that he had come because he liked the surroundings, not to make money; that he was the healthiest man in the world, never having been sick; that the Psychopathic Hospital was a club, for which you have to get somebody to propose your name. There was amnesia and no knowledge of current events. He regarded the food as poisoned, refused to eat, and was very irritable and untidy.

Physically, there were few abnormalities, but the pupils failed to react either to light or accommodation, and the knee-jerks and ankle-jerks were absent. There was a slight Rombergism. There was a marked speech defect to test phrases. Both serum and spinal fluid W. R.’s were positive; the fluid showed 41 cells per cmm., there were large amounts of globulin and albumin, and the gold sol reaction was of the “paretic” type.

118

ATAXIA OR INCOÖRDINATION

NEUROSYPHILIS

LESION OF PERIPHERAL SENSORY NERVES

DIVISION OF POSTERIOR ROOTS

TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COLUMNS

SUBACUTE COMBINED DEGENERATION

VESTIBULAR ATAXIA

FRIEDREICH’S ATAXIA

FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS

THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY

MARIE’S HEREDITARY CEREBELLAR ATAXIA

LESIONS OF CEREBELLUM, TUMORS, ETC.

WRITERS’ CRAMP

PREHEMIPLEGIA

MULTIPLE SCLEROSIS

PSEUDO-SCLEROSIS

HYSTERIA

Chart 18

119

CONDITIONS IN WHICH VERTIGO IS FOUND

NEUROSYPHILIS

HEAD TRAUMA

CEREBRAL ANEMIA AND HYPEREMIA

MENOPAUSE

ARTERIOSCLEROSIS

RENAL DISEASE

CEREBRAL HEMORRHAGE AND THROMBOSIS

INTRACRANIAL TUMORS

MULTIPLE SCLEROSIS

EPILEPSY (Aura)

TOXIC CONDITIONS:

alcohol, tobacco, constipation

PSYCHONEUROSIS

OCULAR DISTURBANCES

EAR DISEASE

MÉNIÈRE’S DISEASE

MIGRAINE

Chart 19

120Salvarsanized serum was injected intraventricularly through a trephine opening in the right frontal region. Injections were made through the corpus callosum into the third ventricle. There was progressive symptomatic improvement after each of four injections. In fact, after the fourth injection the patient was allowed to leave the hospital despite the fact that there was only a slight improvement in the spinal fluid findings. The speech defect had entirely disappeared. (Speech defect, according to many authorities, including Kraepelin, is of very grave diagnostic significance.) His memory returned. Mr. Donovan is now able to handle figures rather extraordinarily well. He now has a good insight into his delusions and tells stories about them with great humor.

1. What is the definition of a remission in general paresis? Remissions form a foil to seizures; just as seizures mark a sudden advance in the severity of the disease or may even lead to death; so remissions may cause a sudden cessation of both mental and nervous phenomena in the disease. Whereas the seizures occur most often, according to Kraepelin, in the demented types of paresis, the remissions occur in all cases except in the terminal phase. Kraepelin quotes Hoppe as observing pronounced remissions of long duration in 17% of male and 15% of female paretics. Gaupp observed marked improvement in less than 10%, and very marked improvement indeed in only 1% of his cases. Kraepelin states that such improvements are most frequent in agitated and especially in expansive forms of paresis, and that they are rarer and less complete in the depressive and demented forms. Sometimes the improvement occurs over night, although the full extent of the remission becomes complete only gradually, perhaps in the course of months. The sensorium clears, the disorientation disappears, the delusions retreat, and the former delusions are treated as dreams and imaginations. There is often a good deal of persistent uncertainty as to events during the height of the disease. 121The nervous disorders are far more obstinate than the mental. Still, both speech and writing may often greatly improve.

Cotton in New Jersey found, among 127 cases of paresis diagnosticated by modern methods during seven years, that remissions occurred in but five, or about 4%, lasting from a half to three years.

2. Does a remission ever amount to a cure? The classical case quoted in this connection is one observed by Tuczek. This case developed a picture of paresis in 1876, at the age of 36; and a remission, or cessation, of symptoms, occurred in 1878; but in 1883, at 43 years, the patient developed a tabes without any trace of mental disorder, which tabes gradually advanced. By the middle of 1898, when the patient was 58, certain symptoms of excitement and confusion occurred, which led to death with dementia, 22 years after the beginning of the disease. Nissl pronounced the cortex to be undoubtedly the characteristic cortex of a paretic. This observation seems to indicate that a clinical remission tantamount to a clinical recovery may occur without the death of the spirochetes engaged. This observation is to be held in mind in connection with all therapeutic work with neurosyphilis.

Nonne states that during his clinical experience of 19 years he had followed 10 cases of paresis with apparent recovery; but of these ten cases, four had to be thrown out by Nonne because the apparent recoveries turned out to be only long and almost complete remissions, finally issuing in characteristic dementia. Of the remaining six cases, perhaps two should hardly be counted as paretic and Nonne rather preferred to term them cases of syphilitic dementia in the sense of a non-paretic cerebral syphilis. At the end, therefore, of his review of observations, Nonne found himself with four cases of true recovery from paresis.

Spielmeyer holds that there is no theoretical reason why paresis might not be cured, since all the different changes that have been described in the disease can be halted, and many of them can be repaired. In particular, he reminds us that the acute infiltrative process, the neuroglia reaction, and the phagocytic action of the large mononuclear cells are distinctly removable processes. (See discussion below under Section V, for apparent cures and remissions occasionally secured under treatment.)

122

REMISSIONS of identical appearance occur in PARETIC (“general paresis”) and in DIFFUSE (non-paretic) NEUROSYPHILIS.

Case 24. Michael O’Donnell, a laborer of 48 years, came home, one day, at 5:30, complaining of severe headache. His wife told him he should lie down and, taking him by the arm, tried to help him to the bed. At this moment, O’Donnell lost control of both left arm and left leg, and fell, unable to move but with consciousness preserved. The wife noted that the left side of his face was drawn up and that he drooled. He was at once carried to a general hospital, remaining there for about three weeks, talking at random in a delirious manner and tied in bed. Two intraspinous injections of salvarsan were given, and O’Donnell showed considerable improvement and went home.

However, upon his return from the hospital, he became very wilful, would not remain in bed, and on one occasion actually took the mattress from the bed, carried it to another room, and then returned to his own room and slept upon the springs. He became irritable and emotional, insisted upon going to the hospital, did not go there but upon returning home insisted that he had been there. That night, O’Donnell left the house only partly dressed.

It appears that O’Donnell had been excessively alcoholic, but that before August 15, when he sustained the left-sided hemiplegia above mentioned, there had been no symptoms except that in February he had once been very dizzy. It appears that there had been another dizzy spell, three nights before the paralysis, accompanied by a fall and unconsciousness for about 15 minutes.

123

TRANSIENT OR FLEETING PARALYSES

NEUROSYPHILIS

MYASTHENIA GRAVIS

MYOTONIA CONGENITA (THOMSEN’S DISEASE)

PARAMYOTONIA CONGENITA

MYOTONIA ATROPHICA

INTERMITTENT CLAUDICATION

OCCUPATION NEUROSES

FAMILY PERIODIC PARALYSES

ETANY

EPILEPSY MINOR

HYSTERIA

MULTIPLE SCLEROSIS

APOPLEXY

CEREBRAL THROMBOSIS

Chart 20

124O’Donnell was brought to the Psychopathic Hospital some six weeks after the paralysis, complaining merely of a slight headache and desirous of treatment. There were no mental symptoms of any sort. Physically, O’Donnell was in general not abnormal (there was a slight pre-systolic murmur and a blood pressure of 190 mm. systolic). The pupils were slightly irregular, the left larger than the right; both reacted sluggishly. Both ears were moderately deaf; the tendon reflexes of the left arm and leg were somewhat more lively than those on the right. The systematic neurological examination otherwise revealed no abnormalities. The urine was negative. The serum W. R. was positive but the spinal fluid reaction was negative. There were but 2 cells per cmm., and there was a very slight trace of albumin.

1. How shall we account for O’Donnell’s transient paralysis? We might invoke brain tumor, alcoholic pseudoparesis, or some form of neurosyphilis. The diagnosis of brain tumor seems quite untenable in view of the absence of premonitory symptoms and in the absence of intracranial pressure. As for alcoholic pseudoparesis it is true that the patient was excessively alcoholic.

However, against these two diagnoses and in favor of the diagnosis of Neurosyphilis, are the positive serum W. R. and the pupillary reactions (although these are short of the true Argyll-Robertson phenomenon). Dizziness with retention of consciousness and associated with the paralyses mentioned suggests rather a subcortical than a cortical lesion. We are inclined to regard this lesion as probably Thrombotic, and to place it possibly in the region of the internal capsule. We are inclined to regard the phenomenon as purely vascular and as not in this case associated with an encephalitis. We are, however, not entirely satisfied with the diagnosis.

2. What shall be said as to treatment? A full-blown left-sided hemiplegia may be produced even when the thrombotic lesion is itself exceedingly small. It is common to explain this on the basis that there is an area of collateral edema about the small necrotic, thrombotic, or hemorrhagic area responsible for the lesion. In short, numerous neurones are functionally rather than structurally affected, or at all events capable of early restitution of function.

3. What is the prognosis in such cases? It appears that now and again patients run for several years without further trouble, both with and without treatment. We are inclined, however, to advocate treatment rather than absence of treatment for a variety of reasons. 125In the first place, vascular lesions may at any time become associated with meningitic lesions, and treatment by salvarsan may perhaps be counted on to head off this process; secondly, the treatment with iodids may possibly aid in the resolution of a local thrombotic process.

Notes in this chapter

Note 1 · Proof of marked parenchymatous lesions must hang on post mortem data; the inference here as to the presence of parenchymatous lesions is a c