Part 9

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.

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

=Case 21.= Martha Bartlett, a woman of 40 years, was brought to the Psychopathic Hospital aphasic, or at least unable to talk distinctly enough to be understood, or even to give name and address. The police had found her wandering aimlessly about the streets. Although she was well-dressed, she was mud-bespattered and apparently had not changed her garments for several days. It shortly developed that the patient, although unable to express herself either in words or by writing, could understand everything that was said to her and could indicate by the monosyllables _yes_ or _no_ whether she agreed or disagreed with statements made. It was thus determined that she was pretty well oriented. She was able to understand both speech and printed words. Although she approximated more than is at all common a pure type of _motor aphasia_, it appeared that there was a slight involvement on the sensory side, especially in the sphere of visual imagery.

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

=CONDITIONS IN WHICH SPEECH DEFECT IS FOUND=

NEUROSYPHILIS HYPOGLOSSAL PARALYSIS FACIAL PALSY PARALYSIS OF PALATE (POST-DIPTHERITIC) BULBAR PALSY PSEUDOBULBAR PALSY MYOPATHY—FACIO-SCAPULO-HUMERAL TYPE OF LANDOUZY AND DEJERINE MYASTHENIA GRAVIS FRIEDREICH’S ATAXIA LARYNGEAL TABES ALCOHOLIC INTOXICATION POST HEMIPLEGIC LENTICULAR DISEASE BILATERAL ATHETOSIS MULTIPLE SCLEROSIS DEAF MUTISM PARALYSIS AGITANS CHOREA STAMMERING TICS HYSTERICAL APHONIA

CHART 17

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

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

On the whole, it would seem best to consider the case of Mrs. Bartlett to be one of CEREBRAL ARTERIOSCLEROSIS OF SYPHILITIC ORIGIN, and a case in which there is no evidence of meningitis or meningoencephalitis.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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