Part 18
There were local variations in the severity of the meningitis. The sulci showed the most marked infiltration. The slighter degrees of exudation were made up largely of lymphocytes with a few plasma cells, occasionally large mononuclear cells, and rarely a polymorphonuclear leukocyte. Where the exudation was more extensive, the large mononuclear cells became more common and the polymorphonuclear leukocytes increased in number. The large mononuclear cells were often phagocytic, containing from one to six leukocytes. The exudate was always most abundant about the blood vessels. The plasma cells were always most numerous in the adventitia of the veins, here greatly outnumbering the leukocytes. The polymorphonuclear leukocytes were relatively infrequent except where there were necrotic areas, which areas were usually continuous with an infiltration of a vessel wall.
As to vascular changes, the media was not often involved, nor was the adventitia so often affected as the intima. Such lesions as appeared in the intima and adventitia were infiltrative rather than proliferative. The elastica of the blood vessels proved to show but slight changes.
A characteristic change was the endarteritis,—of a focal nature with a few large mononuclear and lymphocytic cells pushing the intima inward at the edge of a lesion. In the more marked portion of the focal process, the thickness of the intima was greatly increased by proliferation. Great numbers of large mononuclear cells could be seen between the intima and the elastica. About these cells and interlacing among the other elements of the proliferating tissue was an excess of connective tissue fibres.
The meningeal veins were more often diseased than the arteries; there was adventitial infiltration with lymphoid and plasma cells; sometimes the vein walls had become necrotic and infiltrated with polymorphonuclear leukocytes.
It will be remembered that the left Sylvian artery was grossly thickened, and microscopic section of this vessel showed a partial thrombosis.
The brain showed diffuse and focal changes. The _diffuse_ process was one of nerve cell degeneration and proliferative changes in the neuroglia and blood vessels, and no section of the many examined proved to be free from such changes, although in the majority of instances, these diffuse changes were slight. The cortical layers showed more of these diffuse changes than did the white substance. Barrett considered that the glial cell changes were more delicate indicators of the cortical changes than the nerve cell changes. He found rod cells, satellitosis, superficial gliosis, and a large gamut of changes in the neuroglia. There were two rather characteristic nerve cell changes: a shrinkage change going on to almost complete destruction, and a type of cell swelling, also apparently proceeding to complete destruction.
Among _focal_ changes, there were four main types: Areas of encephalitis, having the general appearance of granulation tissue, areas of simple necrosis or softening, apparently directly related to vascular changes near by, hemorrhages, and certain foci regarded as gummatous.
Save for pial infiltration and a few vascular changes, there was very little change in the medulla and spinal cord. There was a hypertrophic gliosis of the margin of the medulla and cord throughout, and a focal lesion of well marked gliosis at one point in the bulb. There were no fibre degenerations in the medulla or cord, nor were there any coarse fibre degenerations in the cortex itself except in one locus, the left gyrus rectus. This case is of peculiar value in showing to what extent lesions may proceed in a period of six to eight months after primary infection.
Of course the case is in one sense entirely atypical. The lesions were not confined to the nervous system. Aside from the maculo-papular eruption and ecchymosis of the skin, there was a diffuse hemorrhage of the inner half of the conjunctiva of the left eyeball, a small hemorrhagic focus in the mitral valve, a caseous nodule, one cm. in diameter, in the apex of the left lung whose tuberculous or syphilitic nature is left in doubt; a broad milk-colored patch of thickening of the capsule of the liver. It is to be noted that there were no gross lesions of the aorta.
=On the classical assumption that PARETIC NEUROSYPHILIS (“general paresis”) is a fatal disease, is there a disease PSEUDOPARETIC NEUROSYPHILIS (“pseudoparesis”) which may recover or pursue a long course like that of a case of diffuse neurosyphilis (“cerebrospinal syphilis”)?=
=Case 58.= Peter Burkhardt had been an efficient highway inspector, but in his forty-fifth year he had begun to be unable to do his work satisfactorily. His wife had become somewhat afraid of him. He had had somnolent spells in his chair and had squandered money. The mental symptoms had lasted for some six months, but had become more marked during the month preceding admission. Burkhardt would at times fail to recognize his friends.
The general =physical condition= of Burkhardt was very good. The pupils were irregular and reacted sluggishly both to light and to accommodation. The knee-jerks and ankle-jerks were absent. There were no other neurological disorders upon systematic examination. There was a speech defect. =Mentally=, little could be determined except a certain sluggishness.
History and physical examination at once suggested general paresis. The serum W. R. was doubtful, but the spinal fluid reaction was positive, as was the gold sol reaction (which was “paretic”); the globulin and albumin were greatly increased; there were 48 cells per cmm. Antisyphilitic treatment, consisting of salvarsan twice a week and potassium iodid by mouth, was followed by a rapid mental improvement. After two months, Burkhardt was discharged apparently normal, with all the blood and spinal fluid tests negative. He has been taken back into the highway service.
1. What is the proper definition of pseudoparesis? Fournier termed pseudoparesis certain cases that looked like paresis but were not syphilitic in origin. Of these cases the most characteristic group is that of alcoholic pseudoparesis. It is clear that there will be no difficulty in the definition of a disease pseudoparesis whose entity is presented in the adjective that precedes the term (_e.g._, alcoholic pseudoparesis). According to this usage, a case of pseudoparesis would be one in which the symptoms and possibly some of the signs somewhat resemble the symptoms of paresis itself but for which another etiology could be fairly established.
2. Are there any cases of syphilitic pseudoparesis? We are of the opinion that the term should be dropped. It is true that there are cases which clinically look like general paresis and exhibit the appropriate laboratory signs of general paresis but seem to differ from paresis in their course even when they receive no treatment whatever. In the present phase of doubt as to the classification of paretic and non-paretic forms of neurosyphilis, it seems to us of doubtful utility to characterize a case as pseudo simply because it differs in its course, particularly as the literature has always duly recognized that a number of cases of general paresis have had long courses and sometimes very long remissions.
There is also another group of cases that have been termed cases of pseudoparesis, namely: certain cases of neurosyphilis which clinically look like general paresis and seem to be following its classical course but are interrupted by treatment. Here again it seems to us doubtful whether the designation pseudo should be attached to this group of cases, particularly while the whole therapeutic question in the paretic group of neurosyphilis cases remains _sub judice_. Accordingly we are tempted to include in the group of paretic neurosyphilis cases that either get well of themselves, or get well under treatment, or pursue a very long course, or are subject to very long remissions. But we make this decision in terminology without prejudice to the therapeutic question and it is open to any critic to throw these cases into an atypical non-paretic group of neurosyphilis cases.
3. How shall we explain the absence of ataxia of case Burkhardt when knee-jerks are absent and when, therefore, we are entitled to conclude a certain degree of spinal disease? As stated in connection with case Sullivan (16), the absence of knee-jerks is not a warrant for terming a case—paresis of the tabetic form. The fact is that the lesion in paresis tends to be intraspinal, just as the higher brain lesions tend to occur within the brain substance. The meninges are relatively spared both within the cranium and within the spinal canal. The characteristic degeneration of posterior nerve roots which we find in tabes dorsalis is not necessarily found in general paresis even when there are somewhat extensive spinal lesions. Accordingly the absence of sensory returns by way of the posterior nerve roots which characterizes tabes dorsalis is not necessarily a phenomenon of general paresis. The mechanism by which the knee-jerks are lost depends upon histological detail. They may be lost when under tabetic conditions the posterior roots are severely diseased and when under paretic conditions only intraspinal collaterals or a small portion of fibres are affected. The whole question hinges upon where and to what degree the various reflex arcs are cut in the disease. The tabetic phenomena are, as so commonly stated, intradural; that is, the sensory or gangliospinal neurones at certain levels are affected all the way in from the points at which they pierce the dura mater. The affection of these and other neurones in general paresis is an intraspinal and parenchymatous affection.
=Neurosyphilis; auditory hallucinations; ideas of persecution; attacks of excitement. SYPHILITIC PARANOIA (Kraepelin)?=
=Case 59.= Bridget Curley was a case that was discharged from the Psychopathic Hospital, recovered, after 26 days in hospital. The symptoms so resembled those of alcoholic hallucinosis that the diagnosis was made despite the fact that the patient consistently denied the use of intoxicants. There was, in fact, no proof that she drank alcohol. The case was, however, not clearly one of alcoholic hallucinosis or of any other well-defined form of mental disease. A provisional diagnosis of manic-depressive psychosis, manic phase, had, in fact, also been made.
The illness had begun with depression and inactivity, Bridget’s friends accounted for these conditions on the ground that a lover had departed for Ireland. A few days after the depression began, Bridget became dizzy and refused to give a boarder his breakfast, stating that she had lost her memory and had begun to hear bells ringing and people talking. She then became greatly excited and was brought to hospital, where the prolonged baths quieted her.
It seems that Bridget had had stomach trouble and headaches at the top of her head or sometimes in her temples. =Physical examination= showed the left pupil to be larger than the right, a slight tremor of the lips, a slight systolic murmur at the apex, slightly irregular pulse, and moderate edema of ankles. The blood serum was negative to the W. R., but lumbar puncture was executed and the fluid showed a positive W. R.
The patient was tested by the Binet and other methods, and although 35 years of age, seemed to be by the mental tests hardly over 11 years old. She was inclined to be feverish, somewhat restive, and pugnacious; rather slow of speech, sometimes refusing to answer and grimacing. Her pugnacity was, however, easily controllable, and the excitement was largely at night. This excitement subsided rapidly in the course of a few days.
1. What is the diagnosis in this case? The following diagnoses and suggestions for diagnosis were made at the staff meetings:
Unclassified mania. Manic-depressive psychosis, manic phase. Toxic delirium. Dementia praecox. Bacterial infection of the brain. Unclassified delirium. Acute delirium. Infectious psychosis. Acute confusional psychosis. Psychopathic personality by use of alcohol. Mental deficiency with atypical mental state. Syphilitic paranoia.
2. Is this a case of syphilitic paranoia? The so-called syphilitic paranoia of Kraepelin is a rare and uncertain type of syphilitic mental disease. Delusions and hallucinations are prominent. As a rule, the onset is stated to be slow and insidious, or at any rate there are a variety of indefinite prodromata. Jealousy is a prominent feature, sometimes attended with marked sexual excitement. Auditory hallucinations and ideas of persecution are particularly in evidence. The most striking feature in Kraepelin’s group was a sudden occurrence and equally sudden disappearance of violent excitement, with or without external cause. Thus, an excitement would be produced by a few words spoken, and immediately after, the phase of excitement would pass and the patient would become entirely friendly and accessible once more, as if nothing had happened. About half of Kraepelin’s cases showed a positive serum W. R. He does not report lumbar puncture findings, and grounds the existence of disease upon certain autopsied cases. The speech and writing disorder of paresis as well as the characteristic disorientation for time and muscular weakness of general paresis were absent in the group. It appears that most cases of the group have hitherto been placed in dementia praecox.
=The clinical symptoms of CHRONIC ALCOHOLISM are sometimes largely identical with those of PARETIC NEUROSYPHILIS (“general paresis”): differentiation by means of the laboratory findings.=
To demonstrate this proposition, the cases of Francis Murphy (60) and David Collins (61) are in point, being sharp foils to one another.
=Case 60.= A laboring man about 44 years of age was brought to the Psychopathic Hospital one summer day, in a stupor. This patient, Francis Murphy, had been at his regular work as axeman in the Park Service, when he suddenly fell in a heavy convulsion. He was carried to a general hospital, still in convulsions, and ether was administered to quiet the movements. The convulsions shortly ceased, but the patient’s consciousness failed to clear; hence his transfer to the Psychopathic Hospital.
Here he remained much disturbed and was placed in a room with a mattress on the floor. On this mattress he would crouch on all fours for a considerable time, looking fixedly downward as if at an object on the floor, unresponsive to questions but compliant with efforts to place him on his back. He gave the impression of daze and either disorientation or confusion.
Within twenty-four hours the patient became more tranquil and consciousness became clearer, but the patient was at a loss to bring to memory either recent or remote events. However, he replied to questions, giving some different story each time he was approached. Curiously enough, the patient seemed very contented and good-natured and would even laugh foolishly at times, saying that he felt fine and all ready to go out to work.
The general impression conveyed by Francis Murphy at once suggested the possibility of neurosyphilis. Convulsions, perhaps initial in middle age, with a post-convulsive stupor, followed by a partial clearing up, with persistent amnesia and a suggestion of fabrications with euphoria, bore out the suggestion.
The =physical examination= strengthened the impression of neurosyphilis. Well developed and nourished, florid, with a manual tremor and sweating of the palms, the patient was in general without physical symptoms. =Neurologically=, however, whereas the left pupil was larger than the right and reacted properly to light, the right pupil was a bit contracted, somewhat irregular, and either reacted not at all to light or very slightly so (reacting perfectly to accommodation). The knee-jerks could be obtained only with reinforcement, and several other reflexes could not be elicited (triceps, radial, ulnar, periosteal, Achilles, umbilical). Moreover, the heel-to-knee test was poorly performed; some of the common tests phrases were very poorly repeated; there was marked tremor in writing; and the paragraphia seemed to be not merely peripheral, for syllables were left out in words and ordinary words spelled incorrectly (psychographic disturbance).
We do not care here to insist that the right pupil was really an example of the Argyll-Robertson phenomenon since the slightest tinge of doubt is important if a positive diagnosis is practically equivalent to asserting syphilis. Practically, however, the right pupil was regarded as an Argyll-Robertson pupil under hospital conditions (flash-light reaction). Argyll-Robertson pupil, areflexia, speech disorder, writing disorder, memory disorder, conduct disorder, and euphoria, all with a history of convulsions, certainly warranted the tentative diagnosis of neurosyphilis.
As usual, resort was made to the W. R. in the serum and in the spinal fluid. One of the first results to come through from the laboratory was the absence of globulin, normal albumin, negative gold sol reaction, and a cell count of two cells per cmm. in the spinal fluid. Later the _W. R.’s_ were returned _negative_ for blood and spinal fluid.
In the meantime, an illuminating change had occurred in the patient, for two days later,—three days after the first convulsion in the park,—the patient had apparently quite recovered; his consciousness became nearly clear; he could remember every event up to the time of the convulsion, and his memory came back in appropriate degree for both remote and recent events.
The patient, it appeared, had for some time been drinking more and more heavily. In recent days, he had been taking five or six whiskeys and a half dozen beers daily on the average, and often much more. About ten years before, the patient narrated, there had been a convulsion at a ballgame, and this convulsion the patient himself called a “rum fit.”
Here, then, is a case of ALCOHOLIC PSEUDOPARESIS. Without the W. serum test and without the spinal fluid examination, it is probable that the diagnosis of general paresis might have clung to the patient for some time on account of the apparent Argyll-Robertson pupil, which had to be accepted as such on the flash-light data. In point of fact, in this case the pupil later reacted more normally to light, and the speech and writing disorders measurably cleared up.
1. Can alcohol produce the Argyll-Robertson pupil? The majority of neurologists would today answer, Yes.
2. If in the case of Francis Murphy, the W. R. in the blood had happened to be positive on account of a non-neural syphilitic infection (spinal fluid negative), would the diagnosis _general paresis_ be warranted? Probably the diagnosis _general paresis_ would have been made. If the patient had been lost to observation, he might well have been regarded as an atypical paretic with prodromal convulsions.
3. Would positive globulin and excess albumin in the spinal fluid alone or in association with a positive serum W. R. warrant the diagnosis _general paresis_ or _neurosyphilis_? The chances are that most neurologists would advocate proceeding to treatment in any case of positive serum reaction, whether or not there was globulin or excess albumin; but the positive globulin and excess albumin would probably not warrant the diagnosis _general paresis_ or _neurosyphilis_ in the absence of excess cells and the characteristic gold sol reaction and W. R. in the fluid.
4. Is the case of Francis Murphy one of alcoholic epilepsy (as suggested by Murphy’s own phrase, “rum fits”)? It must be remembered that epileptics become alcoholic and that epileptic convulsions increase or become more severe with alcoholism. On the other hand, the literature indicates that alcoholism can produce convulsions, as can many other factors. The literature also indicates that there is a condition of epilepsy in which the convulsive tendency sets in as a result of alcoholism in a patient not previously disposed to epilepsy; it appears also that sometimes, though very rarely, the epilepsy continues after withdrawal of alcohol, and even after giving up the habit. Francis Murphy appears to have had but two spells of convulsions, both of them following heavy bouts with alcohol. There is so far, then, no warrant for calling Francis Murphy’s case one of alcoholic epilepsy.
5. Does the use of alcohol by a subject destroy the value of the W.R.? It has been held by some that alcoholism interferes with the accuracy of the W. R. This has not been our experience and for the present we are of opinion that the results have the same value in alcoholics as in non-alcoholics. The next case (Collins, 61) is one in which a positive W. R. occurred in an alcoholic. When dealing with paretic neurosyphilis it is especially true that the W. R. is disturbed very rarely, if at all, by toxins or drugs, except antisyphilitic drugs.
=Alcoholism may cloud the diagnosis of NEUROSYPHILIS. Differentiation by laboratory tests.=
=Case 61.= David Collins was a steamfitter of about 43 years of age, picked up at 6.45 a.m. in the midst of convulsions and talking incoherently, in a state apparently of fairly clear consciousness. On arrival at the hospital, the patient was able to tell how he had always been a hard drinker, and how during the past week of unemployment he had taken large quantities of poor whiskey,—perhaps an average of a pint a day. Collins also told how he had had delirium tremens several times, but he said the present spell was quite unlike delirium tremens. There was no disorientation or impairment of memory, and the patient did not in any wise suggest a mental case a few hours after admission.
It appears, according to Collins, that he had obtained some work the night before, and had quit work about 6.30, whereupon he stepped into a barroom, took one drink of whiskey, left the barroom, walked down the street, and suddenly lost track of the world, coming to consciousness in a carriage with two policemen, but remaining, as he said, “dopy,” inattentive, and confused. After a meal, however, the patient began to feel better and soon felt quite all right.