Progressing muscular dystrophia - the essential progressing degeneration of the muscular tissue arising outside of any lesion of nervous system both leading serious atrophies and delicacy of certain groups of muscles.

Etiology, pathogenesis. The reason is unknown. Disease often happens family or hereditary. There is a progressing destruction of muscular fibers to replacement by their connecting tissue.

Signs, current. Disease is usually shown in the childhood. There is a delicacy of proximal departments of extremities, muscles humeral and (or) a pelvic girdle. A gait becomes duck, there is a hyperlordosis, a scoliosis, "pterygoid" scapulas. The atrophy of muscles progresses, and in some groups of muscles delicacy is accompanied by a pseudo-hypertrophy; there are contractures. In the amazed muscular groups gradually die away deep reflexes. The electromyography finds out changes, characteristic for primarily-muscular (not neurogenic) lesions. As a rule, the level of some enzymes in a blood raises.