Death occurred in January, 1895, after an obstinate attack of status epilepticus." Paramyoclonus multiplex is a condition of chronic muscular spasm affecting the trunk, occasionally the muscles of the face, abdomen, or diaphragm. The muscles affected are usually in the trunk and in the limbs, and not in the toes and hand; occasionally the movements are tonic as well as clonic;the degree of spasm varies greatly, and according to Gray may sometimes be so violent as to throw the patient down or out of the chair.
Saltatoric spasm is an extremely rare condition, first observed by Bamberger in 1859. The calf, hip, knee, and back-muscles are affected by clonic spasm, causing springing or jumping movements when the patient attempts to stand. The disease is transient, and there are no mental symptoms.
Progressive muscular atrophy has been observed as far back as Hippocrates, but it is only in recent times that we have had any definite knowledge of the subject. It is divided into four types, the hand type (causing the griffin-or claw-hand, or the ape-hand); the juvenile type (generally in the muscles of the shoulder and arm); the facial type; and the peroneal type.
Generalized progressive atrophy leads to a condition that simulates the appearance of a "living skeleton."Facial hemiatrophy is an incurable disease, as yet of unknown pathology. It consists of wasting of the bones, subcutaneous tissues, and muscles of one-half of the face or head, the muscles suffering but slightly. The accompanying illustration shows a case in which there was osseous depression of the cranium and a localized alopecia. The disease is very rare, only about 100cases having been reported. Of five cases seen by Dana, three were in females and two in males; in all the cases that could be found the origin was between the tenth and twentieth years. It is a chronic affection, usually beginning in early life, increasing slowly for years, and then becoming stationary. It is distinguished from one-sided muscular atrophy by the electric reaction, which is not lost in the facial muscles in facial hemiatrophy, and there is no atrophy of other muscles of the body.
Burr contributes an exhaustive paper on hemiatrophy of the tongue with report of a case as follows: "L. B., female, mulatto, thirty-one years old, married, came to the Medico-Chirurgical Hospital, Philadelphia, September 23, 1895, complaining that her 'tongue was crooked.' Save that she had had syphilis, her personal history is negative. In February, 1895, she began to suffer from headache, usually behind the left ear, and often preventing sleep. At times there is quite severe vertigo. Several weeks after the onset, headache persisting, she awoke in the night and found the left side of the tongue swollen, black, and painless. For some hours she could neither speak nor chew, but breathing was not interfered with. After a few days all symptoms passed away except headache, and she thought no more of the matter until recently, as stated above, she noticed by accident that her tongue was deformed. She is a spare, poorly-fed, muddy-skinned mulatto girl. The left half of the tongue is only about one-half as large as the right. The upper surface is irregularly depressed and elevated. There are no scars. When protruded it turns sharply to the left. Fibrillary twitching is not present. The mucous membrane is normal. Common sensation and taste are preserved. The pharyngeal reflex is present. The palate moves well. There is no palsy or wasting of the face. The pupils are of normal size and react well to light and with accommodation. Station and gait are normal. There is no incoordination of movement in the arms or legs. The knee-jerks are much increased. There is an attempt at, but no true, clonus;that is, passive flexion of the foot causes two or three jerky movements. There is no glandular swelling or tumor about the jaw or in the neck. Touch and pain-sense are normal in the face and hands, but she complains of numbness in the hands as if she had on tight gloves. There is no trouble in speaking, chewing, or swallowing. There is no pain or rigidity in the neck muscles.
Examination of the pharynx reveals no disease of the bones. Under specific treatment the patient improved."Astasia-abasia was named by Blocq, who collected 11 cases.
According to Knapp, four cases have been reported in America. The disease consists in an inability to stand erect or walk normally, although there is no impairment of sensation, of muscular strength, or of the coordination of other muscles in walking than the lower extremities. In attempting to walk the legs become spasmodic; there are rapid flexions and extensions of the legs on the thighs, and of the thighs on the pelvis. The steps are short, and the feet drag; the body may make great oscillations if the patient stands, walks, or sits, and the head and arms make rhythmical movements; walking may become impossible, the patient appearing to leap up on one foot and then up on the other, the body and head oscillating as he advances; he may be able to walk cross-legged, or by raising the legs high; or to walk on his hands and feet; he may be able to walk at certain times and not at others; or to hop with both feet together; he may succeed with great strides and with the arms extended; or finally he may be able to use his legs perfectly if suspended (Gray). There are various types which have been called the paralytic, the choreic, and the saltatory. A tendency to go backward or retropulsion has been observed, according to Gray, as has also a tendency to go forward or propulsion. A curious phenomenon in this disease is that the patient can use the legs perfectly well lying in bed.
The prognosis seems to be favorable.
