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April 1939

SCAPULOPERONEAL AMYOTROPHY

Author Affiliations

LENINGRAD, U. S. S. R.

Arch NeurPsych. 1939;41(4):694-701. doi:10.1001/archneurpsyc.1939.02270160050004
Abstract

In 19271 I observed a peculiar form of muscular atrophy which I regarded as a variety of the Charcot-Marie type, with typical distal distribution of the atrophy of the lower extremities, affecting chiefly the extensor and abductor muscles of the foot. On the other hand, the atrophy of the upper extremities was localized proximally and involved the muscles of the shoulder girdle and partially those of the humerus, whereas the hypesthesias, both of the upper and of the lower extremities, showed a characteristic distal distribution. Observations in 2 cases were in favor of my opinion, the disease in the first being sporadic-none of the relatives had been examined—and in the other familial, the anomaly showing a regular dominant heredity (fig. 1).

The syndrome was conditionally termed scapuloperoneal amyotrophy. In both cases the disease was characterized by partial reaction of degeneration, by a tendency of the toes to form pes

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