CHAPTER XI.

PARALYTIC DEMENTIA.

Synonyms.-Dementia paralytica; Progressive general paralysis; General paresis ; General paralysis of the insane.

Definition.-Paralytic dementia, as its name implies, is a disorder characterized chiefly by progressive enfeeblement of the mind, together with a progressive general paralysis of the whole body. It is essentially a cortical disease, but its symptomatology is frequently modified by spinal complications. The psychic symptoms, in addition to the characteristic progressive dementia, present multiform phases, neurasthenic, hysterical, hypochondriacal, melancholic, maniacal, circular, paranoiac, etc. An expansive phase with delusions of grandeur is very common at one period or another in the course of the malady.

Etiology. Intellectual overwork or strain, working on a foundation impaired by syphilis or alcoholism, or both, may be said to be the chief cause of general paresis. Heredity, undoubtedly, plays a part in the causation of this form of mental disorder, though perhaps not so great as in other classes of insanity. The rôle of heredity has been variously computed at from ten to forty per cent. As regards sex, it may be stated that on an average, among all classes of society, twelve times as many males as females are affected-the disproportion seems to be less among lower orders of people. The age of onset is usually during the fourth or fifth decad, bespeaking in general the climacteric period of human life. But general paralysis may be encountered at almost any Nearly one hundred cases have been recorded as occurring in children. Occasionally late cases are met with after the age of sixty. It is a common disease in the great centers of civilization, where the intellectual stresses are most severe, and is comparatively rare among lower races. For instance, it is seldom observed among the native Egyptians or Icelanders. The disease is more frequent among men of ability in professional or business life than among the ignorant and uncultured.

As regards the position of syphilis as an etiological factor, it may be said that a certain history of syphilis is obtainable in at least fifty per cent. of the cases, and it is probable that the true relation is considerably larger. Several years ago, in a study of this subject, I examined the contributions of no fewer than seventy authors to the elucidation of this problem. There was wide divergence in the statistics presented; but from my examination of all these figures, it was assumed that between sixty and seventy per cent. of all cases of general paralysis were syphilitic, if not far more.

By a comparison of statistics of the relation of syphilis to all other forms of insanity, which I have estimated to be from six to ten per cent., we have the further fact that syphilis is seven to ten times as frequent in dementia paralytica as in insanity in general.

The fact is thus established beyond dispute that syphilis is a striking etiological factor in general paresis, and many modern authorities have come to look upon it as a true parasyphilitic disease.

A much more difficult problem is to determine the exact nature of the relationship between syphilis and general paresis. Is it a direct cause, or merely a contributing agent? Is it in syphilitic cases a postsyphilitic affection, or is foregone syphilis merely a predisposing factor? The problem may be examined from several standpoints. In the first place, we have the rather remarkable statistics of Lewin of 20,000 cases of syphilis, one per cent. of which became insane, and in which not a single case of general paresis developed. Then we have the further fact, to which I have already alluded, that among the native Egyptians, where syphilis is one of the most wide-spread of disorders, scarcely a case of general paresis has been reported; and in the asylums at Cairo, which I visited a few years ago, not one such case was to be found. It is significant, by the way, that alcoholism is seldom or never observed among them, the drinking of spirituous liquors being interdicted by the Koran. Such facts as these it is impossible to reconcile with a hypothesis ascribing to syphilis the direct causation of paralytic dementia.

Again, from the pathological standpoint, it is well known that the direct invasion of the brain by syphilis is characterized by changes in the blood-vessels (endarteritis obliterans), by the formation of gummata, or by diffuse meningeal infiltration (specific leptomeningitis or meningo-encephalitis). The first and third of these processes are most frequent in and about the base of the brain. The second is more common in cortical regions. On the other hand, in general paralysis we have a chronic meningitis of the convexity with atrophy of the cortex, and the processes in this disease and in syphilis are quite distinct, although there are cases in which a syphilitic meningo-encephalitis may closely simulate symptomatically dementia paralytica. The pathological processes are different.

The best recent English exposition of the relationship of syphilis to general paresis is by Dr. Frederick Mott, in the "Arch. of Neurology," published by the Pathological Laboratory of the London County Asylums, Vol. I. He does not consider the dictum "no syphilis, no general paralysis," proven, but believes all the evidence is in favor of the strong influence of syphilis in its production, and brings forward this evidence in a convincing way. One of the most cogent reasons is to be found in his study of twenty-two cases of juvenile general paralysis occurring from the age of eight to twenty-three years. Syphilis could not be excluded in any of these, but was positively determined to be present in thirteen cases, and in the parents in four, making a total of eighty per cent. He feels, however, that all were syphilitic. Dr. Mott considers the pathological process in both tabes and general paralysis to be identical, and agrees with Möbius, who calls them metasyphilitic, the same disease affecting different parts of the nervous system. Since my own studies of this subject in 1892, I have devoted more than usual attention to an extremely careful examination of the history and of the

bodily conditions in paretics in whom syphilis was denied, and feel sure that the percentage is much higher than the figures given by me at that time. There are still some few cases in which it is not possible to discover, either upon the person or in the history, any evidence of previous syphilis, but we know that patients are often seen in whom the specific infection has been so mild in character that no residua are left upon their persons, and they have no memory of initial lesions, yet the conditions are such that they must have had syphilis. As Mott says, women, for instance, are often infected and have no characteristic lesions. They may be immunized by a spermatically infected fetus, so that they have the disease in so mild a form that there is no noticeable impairment of health.

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Alcohol would seem to be a factor in some twenty per cent. of etics. Other toxic agents (lead, tobacco, rheumatism, etc.) are also believed to take a part at times in the etiology. Trauma has often been mentioned as an occasional cause of paresis, but there is no well-authenticated instance in literature of such etiology, and until better evidence is offered we must doubt the sufficiency of this factor.

In most cases, as already intimated, several of the causes named are associated in the production of the disease.

Symptomatology.-The disease is best studied in its three stages -the prodromal period, the established disorder (which may be exalted, depressed, or hallucinatory), and the terminal period of dementia.

Prodromal Period.-General paresis is one of the most insidious forms of insanity as regards its gradual, almost unnoticeable onset. Very often this early stage presents symptoms which lead to its being mistaken for neurasthenia. Indeed, the earliest symptoms may be neurasthenic in character, or even a combination of hysteria with neurasthenia. Sleeplessness, tremor, irritability of mood, hypochondriacal depression, dull headache, ophthalmic migraine, pains in various parts of the body, general malaise, loss of appetite, and digestive disorders— these are the manifestations which may be readily misinterpreted as purely of functional nature. It is only when other symptoms in addition to these are presented that a suspicion of a more serious malady may be entertained or the diagnosis actually established. These symptoms are, on the mental side: little faults of memory; errors in speech or writing; the misuse of words; the leaving out of letters, syllables, or words, or their reduplication in writing; growing indifference to the higher sentiments; loss of the critical faculty; small lapses in the proprieties, and failure of interest in the more important affairs of life. As these mental features become more and more pronounced, the patient loses and mislays things, makes mistakes in money matters, errs in appointments, confuses persons and objects, forgets his way, becomes easily angered, markedly offends the proprieties, shows extravagance in the use of money, evinces distinct loss of the ethical feelings, exhibits proclivities to sexual and alcoholic excess, and becomes negligent of his dress.

In the earlier period the patient, like any neurasthenic, has a dis

tinct consciousness of his own illness and observes his symptoms. But with the progress of the malady-and it is in this that we find an important contrast to the course of neurasthenia-he loses that sense of being ill, takes no further notice of his own symptoms. On the physical side there are a number of significant marks which are helpful in making an early diagnosis: defective innervation of one side of the face, causing a slight paralysis; transitory ocular palsies, diminished sensibility to pain, Argyll-Robertson pupils; diminished, lost, or exaggerated tendon-reflexes; a dark, pale, greasy complexion ; lack of facial expression; jerky tremor of the faciolingual muscles at the beginning of voluntary movement; slight difficulties of articulation; rushings of blood to the head, and attacks of syncope or of mild or severe epileptiform convulsions. A number of other early symptoms have been described by various authors to which some value attaches: loss of memory of localization of tactile sensations (Ziehen); loss of the cremasteric reflex; testicular insensibility; peculiar respiration, with short inspirations, followed from time to time by prolonged sighing expirations (Régis); gastric and vesical crises (Hurd); calcification of the sternum, with incurvation of the xiphoid appendix and consequent interference with thoracic breathing (Régis).

Period of Establishment of the Disease.- When the disorder is fully established after a prodromal period which may range over months or years, it is marked by both physical and mental symptoms which are usually characteristic :

Chief Physical Symptoms.-(1) Peculiar articulation and writing the "paretic speech" and "paretic writing"; (2) tremor; (3) pupillary disorders; (4) lost or exaggerated tendon-reflexes; (5) muscular weakness; (6) apoplectiform and epileptiform crises; (7) emaciation; (8) trophic disorders.

Mental Symptoms.-(1) Failure of memory for both recent and old events; (2) diminishing number of concrete, abstract, special and general ideas; (3) weakening of judgment; (4) loss of sense of time and place (lack of orientation); (5) delusions (marked by enormous exaggeration, whether exalted or depressed); (6) hallucinations and illusions; (7) emotional irritability; (8) exalted, sometimes depressed, mood; (9) loss of ethical and esthetic feeling.

We will now examine these symptoms somewhat in detail.

The paretic speech is so characteristic that, heard a few times, it is always remembered; yet it is difficult to describe. There are shades of difference in various individuals, so that authors qualify the disorder of speech as drawling, stammering, hesitating, scanning, spasmodic, ataxic, and so on. It has some resemblance to the speech of a drunken man. Doubtless the main seat of the lesion affecting the speech of the paretic is in the cortical motor speech-center, but sometimes the lesion is probably in the bulbar centers connected with the elaboration of the motor impulses requisite to articulation. The jerky tremor or ataxia of the speech-muscles, together with incoördinated impulses from the cortical motor speech-center, is responsible for the peculiarities in speech. Labials and certain consonants are the most difficult for the

paretic to enunciate, and the typical speech is shown in the attempt to pronounce such words or phrases as "electricity," "artillery and cavalry brigade," "immovability," etc., in which the consonants may be left out, drawled over, misplaced, or even reduplicated thus: "electericity," "artillililery," "bigrade," "immobilty." immobilty." As the disease advances, the words are run more and more together, until finally the speech is utterly incomprehensible.

The handwriting of the patient is of equal, and in the earliest stages even of greater, importance. Lapses of words, repetitions of words or even sentences, and especially elisions and reduplications of letters or syllables are extremely significant.

The tremor in paretics affects all parts of the body, but is especially noteworthy in the face and tongue. In the tongue it often takes on a fine, fibrillary character. It is very rare in even pronounced neuras

thenic conditions to observe tremor of the facial muscles. Still we do meet with it at times, and the distinction that I would draw between the facial tremor of profound neurasthenia and that of paresis is that in the latter disorder there is a peculiar jerkiness and ataxia in the tremor, especially at the beginning of a voluntary movement. Thus, in asking the paretic to wrinkle his forehead, an ataxic tremor will be set up in the occipitofrontalis. In snarling up the nose, it is observed in the small muscles about the cheek and nose. In showing the teeth, the ataxic tremor becomes marked in the levators of the lip. In protruding the tongue, there is a rapid, jerky tremor at the beginning of the

movement.

As regards the pupils, the most important sign is absence of the reflex to light. Next in order comes extreme miosis (pin-hole pupils), and next in importance a variable inequality (one pupil being larger at one time and the other at another time). Irregularity of outline of either or both pupils is significant. Simple inequality of the pupils is less distinctive because met with in other forms of insanity, and occasionally in normal persons. Marked mydriasis is very common in the

latest stage of the disease.

In tabic forms of the disorder the knee-jerks are diminished or lost. In all other forms the tendon-reflexes are apt to be enormously exaggerated, so that we get not only extreme knee-jerks, but quadriceps clonus, ankle-clonus, jaw-jerk, jaw-clonus, and extreme wrist- and elbowjerks. With this spastic condition we observe also considerable rigidity of the muscles, with a tendency in the latest stage to marked contractures. Often in tabic forms, when the knee-jerks are at first lost, they become finally exaggerated. Hence, while the term tabic is often used to describe a form of paresis in which we have lost or diminished kneejerks, together with Argyll-Robertson pupils, this is simply a descriptive designation, and does not necessarily imply that we have a combination of locomotor ataxia with paresis.

As previously stated, one of the chief symptoms of paralytic dementia is a progressive weakening of the muscles in general of the whole body. It is rather an enfeeblement than a paralysis. It is manifested mainly by localized pareses in various muscles or groups of