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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.

Although 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 to 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 pupillary and aphasic symptoms. In the presence of Argyll-Robertson pupil or even a slight speech defect, the diagnosis of neurasthenia must certainly be made with caution if at all. Kraepelin remarks: The sudden occurrence of neurasthenic disorders in a male of middle age without any evident cause therefor is always suspicious. Yet it must be emphasized that a complaint of occasional dizziness, slight speech defect, tremor of tongue, and a moderate increase of tendon reflexes do not possess any marked diagnostic significance. Clear insight and understanding of the nature of the disease phenomena, a persistent search for recovery, reasonableness in conversation, progressive improvement under appropriate treatment, speak for neurasthenia.

Joffroy and Mignot differentiate what they call preparetic neurasthenia from other neurasthenic states, not only on the basis of its etiology but on the basis of its symptoms. They also call attention to the fact that neurasthenia, being a pure neurosis, develops either on a manifestly hereditary basis or upon some physical injury, weakening disease, or moral shock. The pure neurotic suffers a great deal more than the patient who is destined to become a victim of paresis. The character change in neurasthenia does not amount to that entire transformation of personality (even to the performance of criminal acts) that we find in paretic neurosyphilis; at the most, the neurasthenic shows minor emotional disturbances and a certain pathological egoism. The psychotherapeutic test also rather readily dissipates many of the neurotic, hypochondriacal fears and feelings. Although both pure neurasthenia and the paretic pseudoneurasthenia are characterized by sexual weakness, the sexual anæsthesia of the preparetic is practically always preceded by a stage of sexual over-excitement. These finer clinical indications, however, fade into insignificance beside the data that can and should be obtained from laboratory tests.

5. How exceptional is such a case as that of Harrison? We have in our experience seen many patients with a similar course and configuration of symptoms, although the majority of these cases in a community advanced enough to provide easy access to a Wassermann laboratory are now diagnosticated far earlier than was the case of Harrison.

6. What attitude shall we take toward so-called syphilophobia? It seems to us that resort to a serum W. R. is indicated, both from the standpoint of the community and still more importantly from the standpoint of the patient. We are even inclined to suggest for a case of persistent syphilophobia, when the serum W. R. has proved negative, a lumbar puncture. Syphilophobia must be considered, not as a syphilitic psychosis, but as a phobia to be classified among the psychoneuroses. It becomes a difficult question to decide at times whether a patient who has had syphilis, has had a considerable course of treatment and shows the symptoms of a syphilophobiac should be further treated for syphilis or merely for his phobia. We have seen recently such a patient who gave a certain history of syphilis and who was greatly disturbed lest he should be developing paresis. This fear bothered him greatly. Examination showed irregular pupils, but no other signs of syphilis. The W. R. in blood and spinal fluid was negative as were the other spinal fluid tests. It was considered wise to treat him only for his phobia and under this treatment he was given some relief.

PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like MANIC-DEPRESSIVE PSYCHOSIS.

Case 10. The mental picture in Lyman Agnew, an architect, 58 years of age, was wholly characteristic of manic-depressive psychosis. In the first place, there had been (at 55) a previous attack of depression, lasting a few months, from which Agnew had completely recovered. He had remained entirely well up to four months before consultation. (Manic-depressive psychosis is, at least in a majority of cases, hereditary. There had been mental disorder in one maternal cousin, and mental impairment in the patient’s mother some time before her death from cerebral hemorrhage. There was no other report of mental disease in the family.)

It appears that in the interval between attacks, Agnew had been working very hard and had been fairly successful in paying off a mortgage on his house. A marked elation, somewhat natural, followed this success and continued to an abnormal degree. Agnew labored under considerable excitement, was over-fussy, and at times showed a flight of ideas. His mania or hypomania gradually diminished and depression set in, in which depression he arrived for consultation. He had marked ideas of self-accusation, was emotionally unstable, wept much, and showed a characteristic retardation of activities and unrest.

Physically, there was no neurological disorder. The patient appeared rather under-nourished. The heart borders lay 2 cm. to the right and at 11½ cm. to the left of the mid-sternal line. The aortic second sound was very loud. There was a moderate radial arteriosclerosis. Systolic blood pressure was 210, diastolic 155.

The high blood pressure suggested nephritis, possibly of arteriosclerotic origin, but urine examination and blood-nitrogen tests yielded no evidence of kidney disease. Moreover, it is our experience that a manic-depressive psychosis in persons past middle life is not infrequently complicated by high blood pressure. In point of fact, some authors insist upon a relation between manic-depressive psychosis and the arteriosclerosis which rather frequently sets in in this disease.

Routine examination of the blood serum, however, yielded a positive W. R. Following the approved rule of making an examination of the spinal fluid in all mental cases having a positive serum W. R., we proceeded to lumbar puncture. The fluid was clear and contained 35 cells per cmm., the albumin was in excess, and there was a positive globulin reaction. The gold sol reaction was of the “paretic” type; the W. R. was strongly positive.

On this basis, it seems worth while to consider the diagnosis of General Paresis or that of some form of non-paretic neurosyphilis. The former is the diagnosis which we prefer.

1. What is the classical differential diagnosis between manic-depressive psychosis and neurosyphilis? The laboratory tests have naturally supplanted the older purely clinical methods of differential diagnosis. The difficulties lodge, in the first instance, in depressive states. It would appear to be impossible on purely clinical grounds in certain cases to tell the depression of neurosyphilis from the depression of manic-depressive psychosis, since the slightly greater interest in the outer world taken by manic-depressive patients and their greater responsiveness to diagnostic threats (suggestion that patient is to be pinched or cut) are of no special value in the individual case. Identical considerations hold for the maniacal phases of manic-depressive psychosis, for these maniacal phases may even develop delusions (Kraepelin) of precisely the same nature as the characteristic expansive delusions of the excited paretic.

2. If the clinical symptoms are insufficient in differential diagnosis, are not the pupillary signs and the speech defect of greater value? They are of value if present, but as in the case of Agnew, the victim of neurosyphilis may show no pupillary or speech disorder. Instances are familiar, also, in which the pupillary and speech signs are absent in very advanced cases of non-paretic or even of paretic neurosyphilis.

3. Would not a circular course or recurrence of attacks be decisive for manic-depressive psychosis? Paretic neurosyphilis sometimes exhibits the same circular or recurrent course. We conclude that neither the clinical symptoms, the classical pupillary and speech signs, nor the ups and downs of a particular disease, are at all decisive as between manic-depressive psychosis and paretic neurosyphilis. Resort must be had to laboratory tests.

4. What is the significance of the high blood pressure in paretic neurosyphilis? Work from our laboratory (Southard and Canavan) has shown plasma cells in the kidneys in 17 out of 30 paretics (56%), and in 16 of these 17 paretics with renal plasmocytosis, the plasma cells were found in the periglomerular region. What the relation of these findings may be to heightened blood pressure is as yet unknown. The severe syphilitic involvement of the aorta so characteristic in paretic neurosyphilis, as in other forms, may possibly have a bearing on blood pressure.

A POSITIVE SERUM WASSERMANN REACTION associated with mental symptoms (even with grandiosity) does NOT prove the EXISTENCE OF PARETIC NEUROSYPHILIS (“general paresis”).

Case 11. Juliette Lachine came to a general hospital with pain in the right upper quadrant of the abdomen, wherein was found an enlarged liver. This liver was regarded as syphilitic on the ground that the patient had a positive serum W. R. and that her two elder children were clearly suffering from congenital syphilis. The liver mass was promptly reduced by antisyphilitic treatment of the classical sort. When, however, the patient was given an injection of salvarsan, she shortly began to develop marked mental symptoms, whereupon she was removed to the Psychopathic Hospital.

The mental picture at the Psychopathic Hospital was as follows: Lack of orientation for time, marked distractibility of attention, with a certain jumping from one subject to another, delusions of a religious nature, claims of wonderful powers possessed by the patient, moods variable, though as a rule of a euphoric and elated nature, with laughing and singing. The activity seemed to be of a mental rather than a peripheral nature. The patient did not regard herself as mentally abnormal. The liver was still 4 cm. below the costal margin in the nipple line. We found the W. R. to be positive in the serum but negative in the spinal fluid. In fact, the spinal fluid was entirely negative.

So far as we are aware the picture presented by this case is one of Manic-Depressive Psychosis. We regard the disease as merely complicating the syphilis, although it is entirely possible that some visceral condition incidental to the syphilis might be proved (in a higher stage of psychiatric science) to have produced the mania.

In any event, the patient quite recovered from her mental symptoms in a month. She was then able to tell us of a previous attack of depression some 12 years previously, namely, at the age of 26. It appears that she had at that time been committed to a hospital for the insane.

1. In this case, in which the diagnosis of manic-depressive psychosis and not paretic neurosyphilis was made, are we sure that the symptoms that we term manic-depressive psychosis were not actually produced by syphilotoxins? In other words, in the absence of spinal fluid signs of inflammation or chemical change, might it not be possible for generalized syphilis outside the nervous system to produce manic-depressive symptoms? There is so far in the literature no experimental or other evidence of syphilotoxins. The existence of products and substances permitting the W. R. and the gold sol reaction is not of course evidence of syphilotoxins. Although there is no evidence of soluble syphilotoxins, it is thought that in the so-called Järisch-Herxheimer reaction (the intensification of clinical symptoms after salvarsan injection) effects may be due to the liberation of products from the killed bodies of spirochetes. Such endotoxins are not here in question.

2. Is visceral syphilis, such as gumma of the liver, able to produce characteristic syphilitic reactions in the spinal fluid? We have had an autopsied case in which there was a “paretic” gold sol reaction of the fluid (though without other signs). The autopsy showed gummata of the liver. However, the finer anatomy of the nervous system showed a mild but definite meningo-encephalitic process, which was doubtless responsible for the gold sol reaction.

3. What is the value of grandiose ideas? Ballet distinguishes two groups of grandiose ideas: (a) ideas of self-satisfaction, including ideas concerning extraordinary capacity, strength, power, and wealth on the part of the patient; and (b) ideas of ambition; the latter being of a more exact, constant, uniform and systematizing nature. The more vague and less systematized ideas of self-satisfaction rest in a phase of contentedness and optimism; the more definite ideas of pride and ambition are responsible for striking transformations of personality. General paresis shows, according to Ballet, these ideas of self-satisfaction in their most developed form. A certain variability, absurdity, incoherence, and contradictoriness characterize these ideas and the patient has little or no insight into their nature. When such ideas occur at the outset of the disease, they naturally may be of medicolegal interest. Cotard explains these ideas of megalomania on the part of paretics on the ground that they are essentially motor or will disorders and rest upon a sort of hyperbulia, exhibiting itself in exuberant activity. Régis has thought that the delusional generosity and liberality of the paretic, and his willingness to lend his wealth and talents to social progress, is helpful for diagnosis when contrasted with the more personal egoism of the victim of manic-depressive psychosis. The self-satisfaction of the manic-depressive patient often does not reach a delusional stage, but remains a mere feeling of pathological well-being or euphoria. The maniacal patient may compare himself with some great man but he does not identify himself with him. It must be remembered that these ideas of self-satisfaction occur also in alcoholism, but according to Ballet they occur only in the dementing phase of chronic alcoholism, and have no special diagnostic value. They may be a clinical stumbling-block for a time in the cases of alcoholic pseudoparesis. As for the ideas of ambition in which the patients believe themselves to be princes, emperors, divine messengers, and the like, these are less characteristic of paretic neurosyphilis than of delusional psychoses of a non-syphilitic nature. At all events, such ideas if definite, of long-standing, and systematized by the patient to form a thorough-going portion of his life, are not characteristic of neurosyphilis. The victim of paretic neurosyphilis can as a rule be persuaded out of his delusions, at least for the time being. These distinctions, it must be added, are hardly of value in the early cases of any of the psychoses in question, and cannot be made as a rule in either private or psychopathic hospital practice. Typical examples of grandiosity, although not so frequent as might be thought from textbooks, are always on display in institutions for the chronic insane.

PARETIC NEUROSYPHILIS (“general paresis”) may look precisely like DEMENTIA PRAECOX. Autopsy.

Case 12. Henry Phillips remains a striking case in the memory of those who knew him and his medical findings. Phillips came to the hospital voluntarily at 42 years of age from the bank where he worked as a clerk; he came at the suggestion of his employer. It seems that he had been annoying his associates because he had fallen into a habit of continually scratching himself. Phillips was entirely sure that he was the victim of what he called the “Scotch itch,” and explained off-hand that this itch had been put upon him by the Free Masons as a matter of revenge because he would not join their order. He said once, for example: “At times I feel like raising Hell; then I get a psychic intimation; and then I get to using a foot-rule on my back and to slapping my face.” He explained this psychic intimation as coming from the order of Scottish Rites. Another example of talk is as follows: “My father is a fighting man; that is part of it. They mean to throw me down. I am through now trying for membership in the Free Masons. They have good cause, they must fight. They do not want me for some personal matters. I can go just so far in agreeing and seconding their advances, but in the end it fails. I have no strength nor endurance.”

Aside from these delusions, there was little abnormality to be found, though his recollection for minor events of the immediate present was inaccurate. He was rather abnormally impulsive, gesticulating a good deal while talking, and was of the appearance that the laity call “nervous.” It appears that he had always been peculiar, subject to violent fits of temper, in which fits he might throw things at other members of the family. He always had pronounced likes and dislikes which he never concealed. He had never had friends, had always been secretive; and he was often termed a great student. For some five years he had been studying Japanese from time to time, associating himself with a Japanese.

It never does to jump at the diagnosis dementia praecox. However, the picture seemed characteristic enough for the paranoid form of this disease. Physically, Phillips had no particular abnormality; the knee-jerks were a little lively, and the pupils reacted a little sluggishly. However, the routine W. R. of the serum proved to be positive. Examination of the spinal fluid was resorted to,—as in all cases with a positive serum W. R.—and it also proved to be positive and strongly so; the globulin and albumin were increased, and there was a pleocytosis. A diagnosis of neurosyphilis was hardly avoidable. Phillips later admitted a chancre, which he claimed was located on the mucous membrane of the cheek and acquired by using the same utensils as his Japanese friend, which friend, he stated, had active syphilis.

Antisyphilitic treatment of considerable intensiveness was begun, with intravenous injections of salvarsan and intraspinous injections of salvarsanized serum, but the patient grew steadily worse. His mental symptoms became more marked, although not especially characteristic of general paresis. Neurologically, he did develop signs more suggestive of general paresis, and 18 months later died.

The autopsy showed features of General Paresis. It is not necessary to enter into the question of the details of histological correlation at this time.

1. What conclusion can be drawn from lively knee-jerks? Lively knee-jerks are of very little significance. Not only certain neurosyphilitics but also a variety of neurotic persons, victims of dementia praecox and hysteria, are very prone to have active tendon reflexes. Of course, extreme degrees of exaggeration are of importance, and especially an association of the hyperreflexia with the Babinski reaction, the Gordon, or Oppenheim reflexes, ankle clonus, and the like.

2. Is there any special or differentiating factor in an extragenital chancre as against a genital chancre? Probably this question should be answered in the negative. Some have claimed that chancres draining by lymphatic channels of the head are more likely to lead to cerebral syphilis. This idea cannot be said to be established.

3. Is there any significance in the story, if true, that Phillips acquired his syphilis from a Mongolian? It seems to be fairly well established that syphilis of the nervous system is extremely rare in China and Japan, whereas bone syphilis is very frequent there. It has been held that this has to do (a) with strains of spirochetes, (b) with the state of civilization, or (c) with the degree of “syphilization.” Apparently when a race is first infected with syphilis the lesions are chiefly of the cutaneous and osseous systems; only in later generations the vascular and nervous systems suffer. However, involvement of the nervous systems of Mongolians resident in this country is no rarity, a point possibly in favor of the theory of special strains affecting the nervous system as prevalent in western countries. Little or nothing is known as to the effect of transmission from one race to another, as from Mongolian to Caucasian in Phillips’ story.

NEUROSYPHILIS is NOT to be entirely ruled out by a negative serum Wassermann Reaction; for the fluid Wassermann Reaction may be positive.

Case 13. William Twist is a case of note in the matter of the so-called preparetic period (the idea of Charles L. Dana which was scoffed at when first proposed by him in 1910). The patient, a very successful traveling salesman, 35 years of age, was admitted to the Psychopathic Hospital showing a typical picture of general paresis.

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.

The 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.

3. 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.

Station in syphilitic hemiplegia. Syphilitic pigmentation of skin.

It 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.

The 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, autopsies 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.