General paralysis, which is a very common disease, was first recognized in France; it was identified by J. E. D. Esquirol, and further described and elaborated by A. L. J. Bayle, Delaye and J. L. Calmeil, the latter giving it the name of paralysie générale des aliénés.
As first described by the earlier writers the disease was regarded as being invariably associated with delusions of grandeur. At the present day this description does not apply to the majority of cases admitted into asylums. The change may be explained as being either due to an alteration in the type of the disease, or more probably the disease is better understood and more frequently diagnosed than formerly, the diagnosis being now entirely dependent on the physical and not on the mental symptoms. This latter may also be the explanation why general paralysis is much more common at the present day in British asylums than it was. The total death-rate from this disease in English and Scottish asylums rose from 1321 in 1894 to 1795 in 1904.
General paralysis attacks men much more frequently than women, and occurs between the ages of 35 and 50 years. It is essentially a disease of town life. In asylums which draw their patients from country districts in Scotland and Ireland, the disease is rare, whereas in those which draw their population from large cities the disease is extremely common.
Considerable diversity of opinion exists at present regarding the causation of general paralysis. Hereditary predisposition admittedly plays a very small part in its causation. There is, however, an almost universal agreement that the disease is essentially the result of toxaemia or poisoning, and that acquired or inherited syphilitic infection is an important predisposing factor. A history of syphilitic infection occurs in from 70 to 90% of the patients affected. At first it was held that general paralysis was a late syphilitic manifestation, but as it was found that no benefit followed the use of anti-syphilitic remedies the theory was advanced that general paralysis was a secondary auto-intoxication following upon syphilitic infection. The latest view is that the disease is a bacterial invasion, to which syphilis, alcoholism, excessive mental and physical strain, and a too exclusively nitrogenous diet, only act as predisposing causes. This latter theory has been recently advanced and elaborated by Ford Robertson and McRae of Edinburgh.
Whatever the cause of general paralysis may be, the disease is essentially progressive in character, marked by frequent remissions and so typical in its physical symptoms and pathology that we regard the bacterial theory with favour, although we are far from satisfied that the actual causative factor has as yet been discovered.
For descriptive purposes the disease is most conveniently divided into three stages,—called respectively the first, second and third,—but it must be understood that no clear line of demarcation divides these stages from one another.
The onset of general paralysis is slow and gradual, and the earliest symptoms may be either physical or mental. The disease may commence either in the brain itself or the spinal cord may be primarily the seat of lesion, the brain becoming affected secondarily. When the disease originates in the spinal cord the symptoms are similar to those of locomotor ataxia, and it is now believed that general paralysis and locomotor ataxia are one and the same disease; in the one case the cord, in the other the brain, being the primary seat of lesion. The early physical symptoms are generally motor. The patient loses energy, readily becomes tired, and the capacity for finely co-ordinated motor acts, such as are required in playing games of skill, is impaired. Transient attacks of partial paralysis of a hand, arm, leg or one side of the body, or of the speech centre are not uncommon. In a few cases the special senses are affected early and the patient may complain of attacks of dimness of vision or impairment of hearing. Or the symptoms may be purely mental and affect the highest and most recently acquired attributes of man, the moral sense and the faculty of self-control. The patient then becomes irritable, bursts into violent passions over trifles, changes in character and habits, frequently takes alcohol to excess and behaves in an extravagant, foolish manner. Theft is often committed in this stage and the thefts are characterized by an open, purposeless manner of commission. The memory is impaired and the patient is easily influenced by others, that is to say he becomes facile. In other cases a wild attack of sudden excitement, following upon a period of restlessness and sleeplessness may be the first symptom which attracts attention. Whatever the mode of onset the physical symptoms which characterize the disease come on sooner or later. The speech is slurred and the facial muscles lose their tone, giving the face a flattened expression. The muscular power is impaired, the gait is straddling and the patient sways on turning. All the muscles of the body, but particularly those of the tongue, upper lip and hands, which are most highly innervated, present the symptom of fine fibrillary tremors. The pupils become irregular in outline, often unequal in size and either one or both fail to react normally to the stimuli of light, or of accommodation for near or distant vision.
As the disease advances there is greater excitability and a tendency to emotionalism. In classical cases the general exaltation of ideas becomes so great as to lead the patient to the commission of insanely extravagant acts, such as purchases of large numbers of useless articles, or of lands and houses far beyond his means, numerous indiscriminate proposals of marriage, the suggestion of utterly absurd commercial schemes, or attempts at feats beyond his physical powers. The mental symptoms, in short, are very similar to those of the elevated stage of manic-depressive insanity.
Delusions of the wildest character may also be present. The patient may believe himself to be in possession of millions of money, to be unsurpassed in strength and agility, to be a great and overruling genius, and the recipient of the highest honours. This grandiose condition is by no means present in every case and is not in itself diagnostic of the disease. But mental facility, placid contentment, complete loss of judgment and affection for family and friends, with impaired memory, are symptoms universally present. As the disease advances the motor symptoms become more prominent. The patient has great difficulty in writing, misses letters out of words, words out of sentences, and writes in a large laboured hand. The expression becomes fatuous. The speech is difficult and the facial muscles are thrown into marked tremors whenever any attempt at speech is made. The voice changes in timbre and becomes high-pitched and monotonous. The gait is weak and uncertain and the reflexes are exaggerated. In the first stage the patient, through restlessness and sleeplessness, becomes thin and haggard. As the second stage approaches sleep returns, the patient lays on flesh and becomes puffy and unhealthy in appearance. The mental symptoms are marked by greater facility and enfeeblement, while the paralysis of all the muscles steadily advances. The patient is now peculiarly liable to what are called congestive seizures or epileptiform attacks. The temperature rises, the face becomes flushed and the skin moist. Twitchings are noticed in a hand or arm. These twitchings gradually spread until they may involve the whole body. The patient is now unconscious, bathed in perspiration, which is offensive. The bowels and bladder empty themselves reflexly or become distended, and bedsores are very liable to form over the heels, elbows and back. Congestive seizures frequently last for days and may prove fatal or, on the other hand, the patient may have recurrent attacks and finally die of exhaustion or some accidental disease, such as pneumonia. In the second stage of the disease the patient eats greedily, and as the food is frequently swallowed unmasticated, choking is not an uncommon accident. The special senses of taste and smell are also much disordered. We have seen a case of general paralysis, in the second stage drink a glass of quinine and water under the impression that he was drinking whisky.
The third stage of the disease is characterized by sleeplessness and rapid loss of body weight. Mentally the patient becomes quite demented. On the physical side the paralysis advances rapidly, so that the patient becomes bedridden and speechless. Death may occur as the result of exhaustion, or a congestive seizure, or of some intercurrent illness.