Erb's Dystrophy

Tuesday, May 15, 2012

Erb's dystrophy, or progressive muscular dystrophy, is a hereditary and familial disease which has a progressive and chronic evolution. It is characterized by muscular atrophies of facial, pelvic, and pectoral girdle muscles (of clavicle and scapula), sometimes accompanied by an excessive development of fatty tissue. The muscular atrophy is probably due to an alteration of muscular metabolism, by which the muscle fibers has difficulties in synthesizing phosphagens. Degenerative lesions in sympathetic ganglia and non-myelinated nerve fibers, would also play a role in the sarcoplasmatic muscular wasting. Erb's dystrophy begins at a young age (early teenage) and rarely after 30 years of age.