Régis, [172] before the cause of the disease was definitely determined, defined general paralysis as a "cerebral disorder, sometimes cerebro-spinal (diffuse chronic interstitial meningo-myelo-encephalitis) essentially characterized by progressive symptoms of dementia and paralysis (paralytic dementia) with which are frequently associated various accessory symptoms, and especially an insanity of the maniacal, melancholic, or circular type (paralytic insanity)."
Since the time the disease was described by Bayle, general paresis has usually been spoken of as being represented clinically by three different stages. White[173] speaks of a prodromal period, one of full development and a terminal stage. In the first period he emphasizes the importance of physical symptoms, more particularly the oculomotor and tendon reflex disturbances. These include the sluggish reaction to light (28.3 per cent) or an actual Argyll-Robertson pupil (45 per cent), with an increased, decreased or absent knee-jerk, the exaggerated form being the most common. The mental symptoms may be entirely overlooked in the first stage. There is a gradual progressive deterioration of the personality, with a loss of efficiency, impairment of memory, and failure of judgment. There may be episodes of excitement, depression or delirium, with or without hallucinations and delusions, the latter being either hypochondriacal or grandiose. "The demented type, without marked delusions or sensory falsifications, is the truly typical variety of the disease and the dementia the basal element of all forms" (White). There may be an incipient speech disorder and beginning tremor.
Characteristic of the second stage is a marked increase of the physical symptoms already described, together with the appearance of seizures. Muscular weakness develops and the patient often shows a marked gain in weight. The mental symptoms are merely an exacerbation of those shown in the first stage. The expansive variety constitutes the classic form so often spoken of. There may be agitations, depressions, alternations of these symptoms or even paranoid forms.
In the third stage there is a continued exaggeration of the physical signs of the disease with an advancing mental deterioration. The patient becomes helpless and practically speechless, contractures and bedsores develop, and death often occurs as the result of an unusually violent seizure. The description of this disease in the three traditional stages so often referred to is practically without significance and of very questionable value. It is, of course, a well-known fact that the disease may progress rapidly to a termination in two or three years or may continue for an almost indefinite period of time. It may manifest itself, furthermore, in various ways. The physical signs show much greater constancy than the mental symptoms.
Kraepelin[174] describes demented, depressed, expansive and agitated forms of general paresis. The "demented" form he finds to be much more common than the others. This is characterized by a progressive mental deterioration with "paralysis." The onset is marked by a poverty of thought, forgetfulness, moodiness, instability and indifference. Consciousness gradually becomes somewhat clouded and the patient more or less disoriented. Transitory delusions supervene. These are of a depressive type, somatic or expansive in nature. The delusional ideas as a general rule are rather childish. Memory disorder becomes conspicuous and delirious excitements occur at times. All of this leads to a gradual deterioration. Speech defects appear sooner or later and conduct disorders are common. Kraepelin finds that fifty-three per cent of his Heidelberg cases were of the demented form. At Munich they constituted fifty-six per cent of the men and seventy-three per cent of the women. Forty-four per cent of the cases died within the first two years.
The "depressive" form of paresis as described by Kraepelin is characterized by emotional depression or anxiety with delusions of various kinds. It may begin with a general sensation of illness and a gradual weakness of memory or intellect followed by symptoms of mental dulness. The unpleasant ideas are hypochondriacal in nature and often of an extravagant type. The delusions are quite frequently somatic in origin. Sometimes these are associated with self-accusation or there may be complaints of persecution. Hallucinations occur at times. In spite of this deplorable state of affairs a marked indifference on the part of the patient is the rule. Excitement, violence or suicidal impulses nevertheless occur, and stuporous states are described. Kraepelin found that the depressive form constituted twelve per cent of his cases at Heidelberg. He is of the opinion that the duration is short, much more so than in some of the other types of the disease. Fifty-eight and six-tenths per cent died within the first two years. Convulsions, however, were less frequent.
The "expansive form," according to Kraepelin, may begin with an initial depression or show excitement early. Megalomanic symptoms of the most extravagant variety soon appear. The marked mental weakness is, however, very manifest. Hallucinations of sight and hearing are frequently present but transitory. The mood is usually happy, although hypochondriacal ideas occur for short periods now and then. Excitability is more common, sometimes with unusual violence. The course tends to a complete deterioration, with occasional exacerbations of excitement. Kraepelin found that the expansive form constituted about thirty per cent of his Heidelberg cases. Convulsions were less frequent and remissions more common than in other types. He found that this form of the disease, moreover, occurred later in life. Forty per cent died within the first two years. Some cases, on the other hand, were of long duration; one of seven, another of eight, and one of fourteen years. He also noted mixed varieties with alternations between excitement and depression.
The "agitated" form as described by Kraepelin is that type in which extreme excitements predominate. It is often of sudden onset. Grandiose ideas, even more extravagant than those of the expansive form, appear. A flight of ideas may be observed at times and stupor often intervenes. The most severe cases are those which have been referred to by some writers as "galloping" paresis. An actual delirium may lead to an early termination in death. The agitated type constituted 6.3 per cent of Kraepelin's cases. He finds this condition somewhat analogous to the delirious states due to alcoholism.
Remissions are more common in the agitated and expansive forms of the disease and may vary in duration from a few months in some instances to one of fourteen years reported by Dobrschansky. Nissl confirmed the diagnosis of paresis at autopsy in a case observed by Tuczek which had been stationary for nearly twenty years. Alzheimer reported another with a known duration of thirty-two years. Kraepelin has found, however, that fifty per cent of his paretics die within the first two years. He reports unequal pupils in from fifty to sixty per cent of those examined. He also finds that pupillary irregularity is one of the earliest physical signs in many individuals. Complete loss of light reaction was found in from fifty to sixty per cent of all cases, with a reduced range of reaction in from thirty to forty per cent. He found epileptiform or other attacks present in from thirty to forty per cent of those studied. Decreased or absent patellar reflexes were noted about twice as often as were increased reflexes. In from two-thirds to three-fourths of all cases he found both the posterior column and lateral tracts of the cord involved.
The characteristic physical signs noted in all textbooks are described in detail by Kraepelin[175] as common to all of the clinical forms of the disease. The inequality, irregularity and immobility of the pupils, the speech defect, difficulty in writing, tremor of the lips, facial muscles and tongue, the marked changes in both superficial and deep reflexes, the alterations in the gait, the muscular incoordination, the presence of the Babinski reflex or ankle clonus, the sensory, motor, vasomotor and trophic disturbances constitute a combination of physical signs which is to be found practically nowhere else within the domain of psychiatry. The seizures, either epileptiform, apoplectiform or resembling syncopes, are almost pathognomonic when taken into consideration with the physical signs alone.