CHAPTER V
THE PSYCHOSES WITH CEREBRAL SYPHILIS
The indications are at the present time that the psychiatry of the future will not deal with a consideration of general paralysis and cerebral syphilis, as such, but will differentiate preferably between parenchymatous and interstitial, or mesoblastic, syphilitic processes of the nervous system. The retention of the designation general paresis is little, if anything, more than a concession to the claims of tradition. Cerebral syphilis may be said in a general way at this time to include all syphilitic involvements of the brain other than general paresis, which must be accorded the precedence due to priority of recognition if nothing else. In the light of our present knowledge we may speak in rather definite terms in considering cerebral syphilis from the standpoint of pathology. On an anatomical basis it is usually divided into three forms,—the meningitic, the endarteritic and the gummatous types. It is, of course, not to be understood that these represent separate and distinct processes. Combined forms are nearly always to be expected and the different types practically always coexist more or less.
The onset of the disease may be expected anywhere from one to ten or even fifteen years from the date of the initial lesion. The early appearance of cerebral symptoms would indicate brain syphilis as a general rule rather than general paresis. Oppenheim[182] in his second edition says that cerebral syphilis often develops within a year after infection, a majority of the cases being noted within two years. He finds it a very rare occurrence after ten years. "Because," as Barker[183] puts it, "of the lawlessness of the occurrence of syphilitic lesions in the central nervous system, all clinical classifications of these cases are based only on the predominance of certain associations of lesions." Certainly the pathology of the disease is quite varied in its manifestations.
The meningeal form is the one most often encountered. This may appear on the convexity or on the base of the brain and is spoken of as being either localized or diffuse in character. It may or may not be associated with gummatous formations or cortical vascular involvement. The essential process is a leptomeningitis. The pia is thickened, opaque and adherent to the cortex. The microscope shows the presence of inflammatory elements consisting largely of lymphocytes and plasma cells which may be confined entirely to the meninges or may extend downward to the superficial cortical layers directly or by extension along the adventitial sheaths of the vessels. An examination of the cortex, however, shows a limitation of this invasion to the immediate neighborhood of the meninges. The cortical involvement, in other words, is entirely secondary and is not the important part of the pathological picture that it always is in general paresis. The meningeal condition is practically the same in the two diseases but more likely to be localized in syphilitic processes. Dunlap[184] calls attention to the important fact that in a group of cases occurring many years after infection he found involvements of the deeper cortical layers strongly suggesting general paresis pathologically and impossible of differentiation clinically. In these cases, even in the deep cortical vessel walls, occasional lymphoid and plasma cells were found, as well as typical syphilitic endarteritis in some instances. There is frequently, in addition to the simple meningeal involvement at the base, a widespread gummatous infiltration of the pia-arachnoid or in some instances numerous miliary granulomas. This is especially common in the region of the chiasm and may involve the origin of various cranial nerves, obviously in such cases determining the symptomatology to be expected. The optic and oculomotor nerves particularly are affected. The large vessels at the base are often involved either by syphilitic inflammatory processes or by direct invasion of their walls by gummas. An extensive specific meningo-encephalitis may lead either to foci or extensive areas of actual softening.
The endarteritis which occurs in syphilis is characteristic and diagnostic. This has been studied exhaustively by Heubner. The smaller vessels show an infiltration of lymphoid and plasma cells in their adventitia, as well as in the perivascular lymph spaces. The larger vessels show a great thickening of the intima which is consecutive, or, as Lambert described it, "girdling" in character. This is associated with a splitting of the membrana elastica. The proliferated intimal tissue is very susceptible to degenerative processes. Thrombosis and the formation of anemic infarctions may follow the obliteration of the vascular channels. The involvement of the larger vessels may lead to very distinctive focal symptoms. Thus, as Barker[185] has pointed out, there may be an obliterating process in the middle cerebral with hemiplegia and aphasia, invasion of the basilar artery with pontile or bulbar symptoms, or an involvement of the posterior cerebral may lead to hemianesthesia or hemianopsia, while an affection of the vertebral may show a unilateral bulbar paralysis with hemianesthesia of the same side and a hemiplegia of the opposite side. The extensive involvements of the base are usually meningeal, with gumma formation and with a secondary endarteritis in addition. Large solitary gummata may, moreover, occur practically anywhere in the brain, although they are somewhat unusual. On microscopical examination they show a characteristic infiltration of the periphery and a caseous center. They are more likely to occur in the course of a large vessel.
The symptomatology of brain syphilis necessarily varies with the nature, extent and location of the lesion. In the earlier stages of a diffuse meningitis the prominent symptoms to be expected first are headache and dizziness. In an individual with a definite specific history a persistence of such symptoms should suggest salvarsan therapy. Vomiting is a common complication. Cranial nerve palsies, optic neuritis or hemiplegia in such a case would, of course, be conclusive. Stuporous, confused or delirious states may occur, with or without hallucinations. When the syphilitic process is an extensive one with a widespread meningitis or gummatous involvement of the base, numerous focal symptoms are to be expected. Choked disc, optic tract lesions, paralysis of the ocular muscles, facial neuralgias, facial palsies, deafness, or anesthesias may occur. Mental deterioration naturally advances with the progress of the disease, but the personality is much better preserved than in general paresis. Periods of unconsciousness are not infrequent and convulsive attacks may appear. These may be general or local and paralyses often follow. These may assume the form of a hemiplegia or may involve only certain groups of muscles. Ptosis is often noted. Paralysis of other eye muscles is common, and pupillary rigidity is sometimes a symptom. Hemianopsia and diplopia are often observed: An important feature of the disease is the fact that these conditions are more or less transitory and rarely become permanent. Apoplectiform attacks followed by hemiplegia are results of gummatous growth or may be associated with areas of softening. These are due to vascular disturbances. Aphasia is not an unusual occurrence. Hemiplegias appearing suddenly in individuals under forty years of age are likely to be of specific origin. Epilepsies developing in later years should always be viewed with suspicion. The Korsakow symptom complex has been found in some cases of brain syphilis. Memory defect is present in most instances. When a marked mental deterioration takes place it is usually late in the disease. Argyll-Robertson pupils are infrequent in cerebral syphilis. Speech defect is practically never so conspicuous as it is in general paresis. Writing difficulties are also much less marked. Euphoria and grandiose delusions occasionally occur in brain syphilis but much less frequently than in general paresis. Hemiplegias, when they occur, are much more likely to be permanent than they are in general paresis. Paranoid complexes are sometimes clinical features of the disease and if they persist strongly suggest syphilis rather than paresis.
There should be a positive Wassermann reaction in the blood serum of both diseases. It is more persistent, however, in the syphilitic form. In the spinal fluid the reverse is the case and negative results are often noted in cerebral syphilis. There is usually some increase sooner or later in the albumen and globulin content in both diseases. There may be a lymphocytosis in both, although usually much greater in general paresis. A typical colloidal gold reaction is more indicative of general paresis than syphilitic conditions. Several clinical groupings have been proposed. Plant, for instance, speaks of various forms of mental deterioration, pseudo-paresis, paranoid types, epileptiform varieties, symptomatic disturbances and affective reactions suggesting manic-depressive insanity. The important contribution made by Kraepelin[186] to the literature of this subject is worthy of careful study. He describes a syphilitic neurasthenia, a mental disturbance due to the psychic effect of the disease, and various conditions resulting from gummatous growths. His most important group is a syphilitic pseudo-paralysis, which he divides into a simple dementia, delirious forms, expansive types and a variety showing the characteristic Korsakow syndrome. He also speaks of syphilitic apoplexies and epilepsy, tabetic psychoses and syphilitic paranoid conditions.
Syphilitic neurasthenia as described by Kraepelin is an affection which is likely to occur early in the disease and manifest itself shortly after the initial infection. In the milder forms, evidences of nervousness appear,—difficulty of thought, irritability, disturbances of sleep, pressure in the head, with indefinite and changeable abnormal sensations and vague pains. Later, feelings of anxiety, depression, dizziness, mental dulness, a difficulty in finding words, transient weaknesses, disturbances of sensation, nausea and a slight rise of temperature are observed. He admits that there is some question as to whether this constitutes a clinical entity and if so, whether it is directly due to the infectious process or is to be attributed to psychic disturbances. Nervous reactions of various kinds are to be found in syphilitics without psychosis. Thus, Meyer in sixty-one cases of secondary syphilis found eighteen with sluggish pupils, thirty-two with increased reflexes, and twelve with general nervous manifestations such as headache, vertigo, etc., appearing shortly after the period of infection. In only five of these patients were there any evidences of an organic disease. In twelve tertiary cases he found indications of an involvement of the nervous system in only two. In thirty examinations following lumbar puncture a lymphocytosis and an abnormal protein content were observed. Buttino, in a study of thirty syphilitics, reported that fourteen showed a diminished light reaction within one year of the time of infection. Later, after unmistakable symptoms of cortical involvement have existed for some time, neurasthenic complexes are common. These take the form of a difficulty of thought, absentmindedness, forgetfulness, and a reduction of interests. The mood may be irritable, surly, depressed, anxious, fearful, and changeable, showing at the same time considerable indifference and dulness. Some are quiet and reserved while others are excited and violent. Severe headaches may be common, more often at night. There are also occasional attacks of dizziness or fainting, disturbances of sensation, sleeplessness, sensitiveness to alcohol, and occasional diplopia. These are preliminary to more severe disturbances, which simulate nervous exhaustion, and are not strikingly unlike the earlier stages of general paresis. They may be differentiated by examination of the spinal fluid.
Another group of cases is characterized by conditions due to an increased intracranial pressure. These are marked by thoughtlessness, dulness, and indifference terminating in a complete lethargy and somnolence, during which the patient occasionally demonstrates that he is not so badly damaged mentally as he appears. Physically there may be weakness, twitchings, fainting spells, convulsions, ataxias, paralyses, dysesthesias, choked disc, etc. The basis of this disturbance is a gummatous growth, its location, of course, largely determining the symptoms. Kraepelin suggests the possibility of getting this disease picture in a syphilitic as the result of a growth of some other kind—a glioma or endothelioma.