A system of practical medicine. By American authors. Vol. 5

by Unknown

SYNONYMS.—Rigidité musculaire avec impuissance de la volonté; Rigidité et hypertrophie musculaire; Myotomé congenitale.

In the year 1876, Thomsen,¹ the medical officer of Kappeln, described a curious form of nervous disease which affected several members of his own family, himself included. It consisted of a tendency to cramp and limited spasm when a voluntary act was attempted, and a seeming and sudden loss of power. This condition disappeared after the performance of the act and its repetition. An attempt at walking would be attended by spastic rigidity of the flexor muscles of the lower extremities, by flexion of the thighs and legs at an angle of 120°, so that the patient often fell forward upon his knees. An attempt to arise from the chair was attended by the same difficulty, the person becoming utterly helpless. The arms and legs were most frequently affected, the trunk-muscles being usually exempt. In some cases the muscles supplied by cranial nerves were the seat of spasm, so that the patient could not close his mouth nor shut his eyes. Westphal, Erb, Peters, Schönfeld, Engel, and others have reported about eight cases in all, and in addition to the symptoms above referred to it was found that mental excitement and cold chiefly precipitated the seizure. The sphincters were never affected, and the general nervous functions were not impaired. The patients talked stiffly, and this was probably due to a spasmodic affection of the lips, tongue, and other articulating organs. The tendinous reflexes were normal, but irritation of the soles produced a peculiar cramp of the leg-flexors and bending of the knees. There is a species of muscular increase which resembles pseudo-hypertrophic paralysis.

¹ Berliner klin. Woch., Mar. 12, 1883.

The malady seems to be of an hereditary nature, if we are to judge by Thomsen's cases. It begins early in life, and does not materially shorten the same. I have seen one case, a young child, which presented the main symptoms of, and was first mistaken for, pseudo-hypertrophic paralysis.

Westphal regards the affection as an “anomaly of muscular tonus;” others have shed no light upon its pathology.

I believe the condition to be an occasional feature of certain organic disorders, notably posterior spinal sclerosis, and there is a variety of paralysis agitans where there is no tremor which presents all the symptoms. The very rare nature of the malady and its peculiar expression render diagnosis easy. It possibly may be mistaken for pseudo-hypertrophic paralysis in young subjects, but in this latter disease we find electrical change in the muscles, and an absence of the tendon reflex. An examination of the enlarged muscles will not reveal fatty increase.

Painless Facial Spasm.

There is a form of spasm of the muscles supplied by the facial nerves which differs from ordinary tic douloureux by the fact that there is no pain in the former. The trouble may be one of a very limited nature, consisting of the involvement of a few fibres, or of limited groups of muscles, or of all the muscles of the side of the face. I never have seen a case of double spasm, though such undoubtedly exist.

The nature of the paroxysm is cumulative, and, as a rule, the attack increases until it reaches an acme of intensity, then quite suddenly ceases. It often begins by a slight drawing of the corner of the mouth, the levator anguli oris and other muscles in the neighborhood being the seat of chronic spasms. This limited spasmodic action is followed by further facial contractions. The orbicularis palpebrarum is often the sole seat of the convulsive movement, and this is common in excessive smokers.

As a rule, the trouble is peripheral and due to some reflex cause, such as cold, injury, bad teeth, which produces reflected irritation through the branches of the fifth nerve, or in rare cases it may be central or due to some bony or other pressure upon the nerve in its passage through the skull. In a recent case the trouble was of evident central origin, and I obtained a history of lightning pains in the lower extremities, some inco-ordination of muscles in both upper and lower extremities, and some paresis of the facial muscles. In this case the spasms involved all the muscles of the right side of the face, and recurred every few minutes. They had first appeared five or six years before I saw the patient, and had gained in frequency from two or three daily until within the past four months they occurred, as I have said, every few minutes. The other symptoms were of insignificant character compared to the spasms.