Abstract
A family is presented wherein seven members in two sibships exhibit muscular atrophy of the girdle muscles of the lower limbs. The atrophy seems to be of neurogenic origin; it is a recessive non sex-linked condition with massive penetrance. There is close blood relationship of the parents of the two sibships. Clinically the condition is similar to muscular dystrophy, but both pathological and electromyographic examinations confirm its neurogenic origin. It is a slowly progressing condition allowing functional independence for many years.

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