When the child is held under the arms, so as to feel the ground with his feet, directly he attempts to walk the thighs are closely pressed together, the knees are slightly bent, the feet are inverted, and the ankles extended so that only the points of the toes touch the floor ; the legs become rigid and soon cross one over the other. In bad cases the heels are not brought into contact with the ground at all. Sometimes the child, although he cannot walk, is able to stand, supporting himself against some object. The rigidities appear to contribute to his helplessness as much as the motor weakness ; and sometimes the attempt at voluntary movement, conflicting with the stiffness of the muscles, results in a sort of chorea.
The back is often very weak, and the muscles of the abdomen may be come hard when the skin is irritated. Control over the sphincters is not interfered with ; there is no paralysis of the face, nor any tendency to the formation of sores or sloughs upon the parts exposed to pressure. The degree of intelligence varies in different cases. Often the child seems as quick as others of his age, but sometimes he is dull and stupid. .Articula tion may be affected, but, as a rule, the patients speak readily and clearly.
Occasionally the arms are affected. In a case reported by Dr. Gee— a little girl, eight years old, in whom the paralysis had existed certainly from the age of twelve months, perhaps from an earlier period—the arms as well as the became stiff when the girl was noticed. The arms were rotated outwards ; the elbows were strongly extended and the wrists pro nated ; the hands were also extended strongly and thrown back at the wrist ; the fingers were flexed. The child could move the opposing mus cles, but with difficulty," and after movement the arms soon returned into the position described. The left arm was more affected than the right. Dr. Gee has described eight cases of this interesting malady, of which the first was observed before the publications of Erb and Charcot had attracted general attention to the disease.
The constant rigidity of the muscles affected is not accompanied, as a . rule, by any wasting, although in exceptional cases, when the disease is of long standing, one or more (not all) of the implicated muscles may show some signs of atrophy. The rigidity is a permanent phenomenon, persist ing during sleep, and only disappearing temporarily when the child is placed under the complete influence of chloroform. The tendinous reflexes are
more active than in the normal state, and the response to faradism is rapid and energetic. Sensation is unimpaired.
In many cases the actual amount 'of weakening of the muscles appears to be slight. The impediment to walking seems to be more the result of rigidities and contractions of muscles, which prevent the foot and limb from beino. placed in a fitting position to support the weight of the body and frustrate the voluntary impulse, rather than of any actual paralysis. From observations made upon the adult sufferer, contractions are found to occur as a later phenomenon, the muscles being merely rigid at first without any shortening in their length. When the contractions come on the paresis becomes more noticeable. Eventually it may amount to comprete loss of voluntary motor power. This is, however, generally of unequal intensity in different regions, being well developed in certain groups of muscles, im perfect in others. Usually the disease is more advanced in one of the limbs than it is in its fellow.
If a child, the subject of this disease, be able to walk, his gait is very peculiar. The patient behaves as if giddy, and sways from side to side. His limbs are widely separated, and he moves each leg awkwardly forward, often along the ground. The tendency appears to be to point the foot so that the heel is not in full contact with the floor. Conse quently the toes are apt to catch at any unevenness of the ground, and the child would fall on his face if not supported.
As the disease advances all the symptoms become intensified. The rigidities, the contractions, the paresis, and the reflex irritability, all be come increased. The lesion does not appear to be fatal to life. Of its later stages little is known, for after a certain degree of intensity is reached, and the patient has been rendered quite helpless, the disease seems to undergo no further change.
Diagnosis.—The essential features of the disease are a slowly growing paralysis of the lower extremities, without wasting, but accompanied by excessive spasmodic rigidity of muscle and increased activity of the tendi nous reflexes. The disease is therefore readily distinguished from infan tile spinal paralysis, in which wasting and arrest of growth iu the affected limb are the rule ; the joints, far from being rigid, are excessively relaxed, and the tendinous reflexes are abolished.