Neurosyphilis

Part 28

Chapter 283,668 wordsPublic domain

=Mentally=, there was a degree of depression and worry hardly out of keeping with the general situation. Despite the preservation of memory, Mr. Stone failed to do rather simple arithmetical calculations; this was the more remarkable as in his business he had to handle figures a great deal and had been doing so until recently. There was a slight tremor in his writing, as well as a certain difficulty in enunciating test phrases. Insomnia, irritability, and a feeling of nervousness and of being tired out, completed the picture.

A suggestion for diagnosis would be classically offered by the Argyll-Robertson pupils. Should not a patient with the Argyll-Robertson pupils have either tabes or paresis? However, in favor of tabes, besides the pupil, are to be counted merely the troubles with the eyes. In the direction of paresis we have to consider speech defect, to say nothing of less definite symptoms such as insomnia and increased irritability.

We are inclined to think, however, that the disease in this case is meningovascular. This diagnosis is suggested by the cranial nerve palsies and by the headache. Headache is much more rarely a phenomenon in the paretic type of neurosyphilis than in the meningovascular type.

In point of fact, the spinal fluid phenomena bore out the diagnosis of MENINGOVASCULAR NEUROSYPHILIS inasmuch as the globulin, albumin, cellular content, gold sol, and W. R.’s were all weakly positive.

1. How far can we regard the cardiorenal defects as syphilitic? Perhaps we may do so on the general principle of parsimony in scientific interpretation.

The diagnostic lumbar puncture led to an extremely severe exacerbation of the pains on the left side of the head. In fact, these pains could not be held in check by the exhibition of pyramidon. Mr. Stone regarded the pain as due to the lumbar puncture. However, there was no improvement in the pain in the prone position,—a feature characteristic of lumbar puncture pains. Upon administration of salvarsan, this local pain rapidly disappeared. In fact, there was a startling improvement; the ocular palsies disappeared in a few weeks, although these palsies had been present for several months before the administration of salvarsan. The blood pressure was reduced; the urine became negative. Perhaps the most startling feature of all (although of this we are not sure) was that the patient states he was accepted by a life insurance company although he had been twice refused previously.

=Note= in this case the 30–year interval between infection and generalized neurosyphilitic involvement. Note also the amenability of the process despite this duration. We are perhaps entitled also to note that a neurological examination careful enough to detect an Argyll-Robertson pupil should have been made by a number of examiners long before the particular crisis which we have sketched. It is also permissible to note that the rhinological work should not have been carried out independently of all other medical work.

2. What are the untoward results of lumbar puncture? It is true that there is always a possibility of setting up a septic meningitis by lumbar puncture, but this is a very remote possibility and with any reasonable care it is not to be considered. Lumbar puncture also has a considerable danger in cases of increased intracranial pressure. In cases of brain tumor where the tumor is located in the posterior fossa, sudden death may occur from withdrawal of spinal fluid. This is supposed to be due to the medulla being pressed down into the foramen magnum and causing paralysis of respiration. Therefore lumbar puncture should never be performed except with the greatest caution in a case in which brain tumor is suspected.

However, aside from these remote serious consequences which play very little rôle in the ordinary procedure of lumbar puncture, certain unpleasant symptoms do frequently arise. These symptoms are chiefly headache and nausea, but, however, may go as far as vomiting. These symptoms occur almost entirely in the cases in which there is no abnormal condition producing increased spinal fluid pressure. Such unpleasant symptoms may last as long as four or five days; as a rule, however, last only for a period of a day or two.

3. What is the treatment of discomfort following lumbar puncture? It is a rule well worth observing that the patient after lumbar puncture should remain flat on his back without a pillow for 24 hours in order to avoid any unpleasant symptoms. If any symptoms do occur, it will be almost certainly when the patient arises, and in nearly every instance they will be overcome if the patient again assumes the prone position. Raising the foot of the bed so as to lower the head also helps. Veronal or bromides may be given but as a rule are not very satisfactory.

4. How permanent is the improvement obtained in the case of Mr. Stone likely to be? As a matter of fact, the patient discontinued treatment as soon as he felt well again, but after two months the pain returned to be again quickly dispelled by salvarsan. This improvement must be considered as only temporary. Under continued treatment there may be no further relapse. There is, however, evidence that much damage has been done to the body by the spirochetes, much of which is irreparable. It is even possible that further disintegration might occur even while undergoing treatment. Still treatment offers much in such a case and is to be highly recommended.

=In DIFFUSE NEUROSYPHILIS, rendering the spinal fluid negative by treatment may mean neither cure nor disappearance of symptoms.=

=Case 107.= Greta Meyer, a widow, 51 years of age, came voluntarily to the hospital, seeking medical aid for a marked depression. She was also suffering from a right hemiplegia. It appeared, according to Mrs. Meyer, that she was married at 16, and lived with her husband until 29, whereupon she left him on account of his alcoholism, his abuse of her, and the discovery through his physician that he was suffering from venereal disease. She had had two healthy children and there never had been miscarriages or stillbirths. Six years after the separation, namely at 35 years of age, and 16 years before resort to the Psychopathic Hospital, Mrs. Meyer developed certain red areas on her hand, and learned at a hospital that these were due to syphilis. She kept up treatment for these lesions for a year, until she seemed perfectly well.

She had, in fact, remained perfectly well for some 14 years, until at 49, a small tumor had appeared on the right side of the forehead, near the hair line. This tumor was firm and not sore. Medical treatment reduced it, leaving, however, a depression in the bone. One day, about a month after the appearance of the tumor, the patient lay down for a nap, and upon awaking found she could only with difficulty move her right arm and leg. Her face was not affected; she was not in pain; and there was no disorder of speech. In a few days she got much better and she had been improving for some time past through the administration of further medicine.

However, since the onset of the hemiplegia Mrs. Meyer had been very despondent. There had been ups and downs but she had rarely felt well. The depression was a mild one and in point of fact may perhaps be regarded as non-psychopathic, since at her age with her disability, there might well be a degree of sadness and unhappiness concerning the future. =Mentally=, there was no other disorder of note, and in particular no disorder of memory.

=METHODS OF TREATMENT=

I. BY MOUTH. 1. MERCURY 2. IODIDES 3. ARSENIC

II. INTRAMUSCULAR INJECTIONS 1. MERCURY 2. SALVARSAN, NEOSALVARSAN, OTHER ARSENIC PREPARATIONS 3. SODIUM NUCLEINATE 4. ANTIMONY

III. INTRAVENOUS 1. MERCURY 2. MERCURIALIZED SERUM 3. SALVARSAN, NEOSALVARSAN, ARSENIC 4. IODIDES

IV. SPINAL INTRADURAL 1. SALVARSANIZED SERUM (IN VIVO—SWIFT-ELLIS) 2. SALVARSANIZED SERUM (IN VITRO—MARINESCO-OGILVIE) 3. MERCURIALIZED SERUM (BYRNES)

V. CEREBRAL SUBDURAL AND INTRAVENTRICULAR 1. SALVARSANIZED SERUM (IN VIVO) 2. SALVARSANIZED SERUM (IN VITRO) 3. MERCURIALIZED SERUM

CHART 26

=Physically=, the patient showed a right-sided hemiplegia with excessive right knee-jerk, but without Babinski or other abnormal reflex phenomena. The extraocular movements were somewhat restricted in range but there was neither strabismus nor nystagmus.

The question arose whether the hemiplegia was of hemorrhagic or thrombotic origin. After all, at 51 years, hemiplegia is rather unlikely to be of a non-syphilitic arteriosclerotic origin; moreover, we had a clear history of syphilis. The serum W. R. proved positive as well as the spinal fluid W. R. The finding of 77 cells per cmm., excess albumin, and positive globulin test, taken in connection with the entire picture seems to warrant a diagnosis of CEREBROSPINAL SYPHILIS. If we proceed on statistical grounds, it might be regarded as more probable that the hemiplegia is THROMBOTIC in origin rather than hemorrhagic. It appears that syphilitic cerebral thrombosis rather characteristically occurs without preliminary symptoms, despite the fact that many cases do show headache, dizziness, and restlessness as prodromal symptoms.

1. What is the treatment indicated in the case of Mrs. Meyer?

It would appear that little or nothing can be done for the hemiplegia unless the claims of Franz with respect to reëstablishment of a degree of function in certain hemiplegics are substantiated. However, the indication of meningitic process as shown by the spinal fluid, suggests that the case is not a purely vascular one but may be regarded as meningovascular. (Possibly, also, we should regard the left frontal depression and scar as indicative of a non-parenchymatous and non-vascular process.) Accordingly, antisyphilitic treatment should be theoretically of some value.

In point of fact, the patient was given injections of mercury salicylate, mercury by mouth, and potassium iodid. Her psychopathic depression under this treatment, supported by proper hygiene and rest, diminished. However, six months later, the patient slipped on a wet floor and fell. Though the impact seemed hardly sufficient to cause a fracture, the pelvis was somewhat severely fractured. Very probably there was a syphilitic rarefaction of the bone. Six months later the patient’s depression was still in evidence, though somewhat less than upon admission. The blood serum remained positive but the spinal fluid had become entirely negative, both in respect to the W. R. and in respect to the other findings.

2. How may one explain the continuance of the depression after the spinal fluid had become entirely negative under treatment? It may be that while the active process had been stopped, as seems probable from the negative spinal fluid, that a permanent destruction of brain tissue may account for the depression. We recognize this readily in instances of vascular disturbance where (as also in this case) the active process being stopped, a residual defect remains.

3. Should treatment have been discontinued on reduction of the gumma? It cannot be too often emphasized that the disappearance of symptoms in cases of syphilis can not be considered as evidence of cure. The neurologist and psychiatrist see only too often cases of neurosyphilis occurring in patients who have been declared cured at some time previous because the symptoms then present had cleared up and remain in abeyance for years.

=Contrary to various warnings, arteriosclerosis by no means absolutely contraindicates intensive salvarsan therapy.=

=Case 108.= Victor Friedberg, 42 years of age, gave the following history. He acquired syphilis at 22 years. He had “adequate” medical treatment for two years with inunctions of mercury and mercury by mouth and potassium iodid. The only secondary symptoms were skin lesions of the legs; these disappeared upon treatment. Married, Friedberg has one child, apparently normal. There had been no miscarriages or stillbirths.

At about 34 years, there began to be shooting pains in the legs, occurring at first about once in three months, but later much more frequently. These pains were severe, lightning in character, lasting several days at a time, at which period his head would feel heavy; but there were no disturbances, crises, or difficulty in locomotion.

At 36 years of age, Friedberg waked up with pain one night, and found he was unable to move his left leg or hand, and he felt his mouth drawn to the left. Upon trying to get out of bed, he fell to the floor. In five hours, however, he was entirely recovered, able to get up and walk about, and to use his left arm quite normally. He went to sleep, but upon waking up after an hour, discovered that his left side was again paralyzed. After two weeks in a hospital, he was able to walk with a crutch. The arm remained helpless for about a year. Both arm and leg improved slowly for two years, after which time his condition had remained stationary. For four years past, there had been no more pain, but at 42—about two years before admission—the pains returned in his legs, back, and side. At that time he received four injections of salvarsan, mercury tablets, and potassium iodid. Three weeks before admission to the hospital, Friedberg again began having headaches, very much worse than formerly. At first these headaches were frontal, then occipital, and there was a feeling as if something were growling inside of the head. There was a feeling of pressure in front on the head and at the base of the nose.

=Physically=, Friedberg appeared somewhat older than his assigned age. There was a degree of general peripheral arteriosclerosis, but in general the physical examination was negative. _Neurologically_, there was a left hemiplegia with appropriate increase of the reflexes on that side, spasticity, Babinski reflex, and an Oppenheim; the pupils reacted properly; there was no Romberg reaction.

_Mentally_, Friedberg was entirely negative.

The W. R. of the blood serum was doubtful, as was that of the spinal fluid. There were but two cells per cmm. and there was neither globulin nor excess albumin in the spinal fluid.

The =differential diagnosis= might lie between cerebral hemorrhage and syphilitic thrombosis. Thrombosis is much more common as a result of syphilis than is hemorrhage. The occurrence of the thrombosis during sleep without premonitory symptoms is also characteristic in syphilis. Possibly there was a low-grade spinal meningitis at the bottom of the lancinating pains. Whether the headache is an arteriosclerotic effect or due to a meningitis not shown in the cerebrospinal fluid is doubtful. However, the absence of inflammatory products in the cerebrospinal fluid rather indicates that the headache is of arteriosclerotic origin. Autopsies, however, warn us that we may have a localized meningitis in various parts of the cranial cavity without the determination of any inflammatory products in the spinal fluid.

1. How shall we explain the doubtful (slightly positive) W. R. in the spinal fluid if the case is one of VASCULAR BRAIN SYPHILIS? The finding is not unusual in these cases. The W. R. producing body is recognized to be of a separate nature from the globulin and albumin bodies, and is probably also separate from the gold sol reaction producing bodies.

Treatment: The theory of treatment is that any spirochetes that may be still active in the body should be destroyed. Accordingly, although salvarsan can certainly have no effect in reproducing nerve tissue, it nevertheless seems indicated. It is frequently stated, however, that salvarsan is dangerous in cases of this group. We have not found this statement correct. In this case, there was a symptomatic improvement, as far as pain and discomfort went, under salvarsan and iodids.

2. What precautions should be taken in intensive salvarsan treatment of syphilitic arteriosclerosis? Treatment should be begun with very small doses of salvarsan, that is, about 0.1 of a gram and then the amount slowly increased. The injection should be given slowly so as not to put too great a load upon the cardiovascular system.

3. What rôle does the mental attitude of the patient play in a case like that of Friedberg? It was quite evident that Friedberg was neurotic and that he had a syphilophobia. Consequently some of the symptomatic improvement may have been more results of assurances offered by the physician and knowledge that he was being treated, than results of salvarsan. In some cases mental anguish suffered by the patient is of more importance than the actual symptoms of the disease and this point must be always borne in mind in handling syphilitic patients.

=Symptoms of intracranial pressure cured by antisyphilitic treatment.=

=Case 109.= Mrs. Annie Rivers, a housewife 36 years of age, sought advice and treatment for severe convulsions which she had had during a period of several weeks. She left the hospital before being properly examined, and had several more convulsions, after which she was brought back in a state of marked confusion. The confusion shortly disappeared almost completely, and a good history was obtained.

It appears that the patient led a normal life and had had six children, the last of whom was born about four months before her coming to the hospital. The first symptoms appeared about a month after the birth of the child, when, one afternoon, Mrs. Rivers suddenly fell unconscious while ironing. She remained unconscious for nearly three hours. During this attack there were no convulsive movements or tongue-biting; and after the spell, she felt neither lame nor sore, but merely tired. This was Mrs. Rivers’ statement; but her daughter stated that the patient really did have convulsive movements. A week later came a second convulsion, followed by daze and stupor. This second attack lasted two hours.

About a week before entrance, the patient had remained in bed on account of dull grinding pain in the left side of the head, below the ear, and upon this day the patient vomited twice. In addition to the dull grinding pain, there were pains referred to the ear itself and to the left side of the head, especially over the left eye; there were no pains on the right side of the head. The next day the patient was better, but the day thereafter again remained in bed. The only other symptoms were cold feelings at times and bright spots in the field of vision.

No =mental symptoms= were observed in Mrs. Rivers except a bit of depression after her hasty retreat from the hospital the first time. Upon her second admission, however, after a week or ten days’ residence, apathy developed together with considerable amnesia for the same facts she had quite readily remembered a few days previously. Along with the apathy and amnesia developed considerable headache; and there were attacks of vomiting.

=UNTOWARD SYMPTOMS OF THERAPEUTIC AGENTS=

=A. SALVARSAN=

CYANOSIS MALAISE RAPID PULSE PERSPIRATION RESPIRATORY DIFFICULTIES FEVER NAUSEA, VOMITING, DIARRHOEA DERMATOSES EDEMA KIDNEY IRRITATION LIVER IRRITATION INTENSIFICATION OF SYMPTOMS COLLAPSE

=B. MERCURY=

SALIVATION FETID BREATH EXCESS FLOW OF SALIVA TENDERNESS OF TEETH—LOOSENING AND FALLING OUT SPONGY GUMS—EROSION METALLIC TASTE NECROSIS OF BONES OF JAW SORENESS OF PARETIC AND MAXILLARY GLANDS SWELLING AND EROSION OF TONGUE AND MUCOUS MEMBRANES GASTRO-INTESTINAL SYMPTOMS ANEMIA PAIN IN JOINTS NEPHRITIS

=C. IODINE=

SKIN LESIONS METALLIC TASTE SALIVATION CORYZA URTICARIA (EVEN TO GRADE OF ANGIONEUROTIC EDEMA) PAINS CONSTIPATION INVOLVEMENT OF JOINTS FEVER SOFTENING AND BLEEDING OF GUMS EROSION OF MUCOUS MEMBRANES GASTRO-INTESTINAL SYMPTOMS ANOREXIA WEAKNESS

CHART 27

On the =physical= side, it is interesting to note that the ophthalmoscopic examination upon Mrs. Rivers’ first admission to the hospital was entirely negative, whereas a week later, pronounced difficulty with vision appeared so that in a few days she was able to make out only very large type. The fundi now showed hazy and indistinct disc outlines, with small yellowish areas of fatty degeneration above the disc, reduction of arterial calibre, and dilated and somewhat tortuous veins (no projection of papillæ), so that the ophthalmological diagnosis was chronic neuritis.

The physical examination otherwise was mostly negative. The skin presented irregular areas covered with silvery scales over the arms and chest, back, abdomen, and legs (the patient had had psoriasis several years before). Both pupils reacted to light and distance, though the right was slightly larger than the left and somewhat irregular. There was a slight tremor of the tongue and extended fingers. The reflexes were active, especially the knee-jerks; no abdominal reflexes could be obtained. The serum W. R. was positive, but the spinal fluid W. R. was negative. The spinal fluid showed but 3 cells per cmm., but there was a positive globulin test and an excess of albumin.

=Diagnosis=: After the symptoms had fully developed, it became clear from the optic neuritis, headaches, and vomiting that a condition of intracranial pressure existed. In view of the positive serum W. R., it is natural to conceive that the agent producing the intracranial pressure was a gumma.

It is, of course, possible that a marked degree of meningitis might be so localized as to produce the same symptoms. The diagnostician would crave a pleocytosis of the spinal fluid if a diagnosis of meningitis is to be made; and there was no such pleocytosis. On the whole, we do not feel that it is possible to make a diagnosis either of MENINGITIS or of GUMMA.

=Treatment=: Treatment, however, caused a disappearance of all symptoms. The treatment consisted of but one injection of 0.3 gram of salvarsan, followed by a few injections of mercury; whereupon Mrs. Rivers became much brighter, recovered her vision, lost her headaches, ceased to have convulsions or vomiting spells.

1. Is salvarsan contraindicated in cases with involvement of the optic or auditory nerves? Such a contraindication exists according to prevailing opinion. In this particular case, a hemorrhagic retinitis occurred after the injection of salvarsan, but this retinitis disappeared along with the other symptoms. On the whole we believe that in many cases of optic or auditory nerve involvement salvarsan should be used. However, one should never lose sight of the possibility of untoward results and should advise such treatment only when other treatment seems inefficient.

=TABETIC NEUROSYPHILIS (“tabes dorsalis”) may show very marked improvement as a result of intraspinous therapy.=