Читать книгу Neurosyphilis - Elmer Ernest Southard - Страница 11

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

1. 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.

3. 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

While 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 term 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 is 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 W. 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

See 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.

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 is 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.

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.

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

1. 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.

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 been 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.

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

1. 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 paretics 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.