STUDIES ON DISEASES OF MUSCLE

Abstract

Progressive muscular dystrophy is characterized by degeneration and atrophy of striated muscle. The disease is heredofamilial. It is accompanied by an abnormality in creatine metabolism, with creatinuria in adults or by excessive urinary excretion of creatine in children. Within recent years the study of diseases of muscle has received new impetus from several sources. New information concerning the origin and metabolism of creatine, the discovery of a deficiency disease in animals which resembles progressive muscular dystrophy in man and new knowledge of the physiology of muscle and the chemistry of muscle contraction have made it advisable to reexamine the syndrome of progressive muscular dystrophy in the light of these advances. This paper presents a clinical description and the results of preliminary studies of 40 cases of this disease. Original descriptions of the syndrome of progressive muscular dystrophy are credited to Meryon, 1852,¹ and to Duchenne, 1861.² Erb.³