Emery-dreifuss muscular dystrophy

摘要: A man had weakness of humeroperoneal distribution associated with limited range motion the cervical spine and elbows. At age 25 he developed permanent atrial paralysis, a cardiac pacemaker was inserted. Although this case sporadic, most others have been transmitted as an X-linked recessive trait. Mixed patterns in electromyography muscle histology caused nosological confusion, but unique clinical signs seem to define distinct form muscular dystrophy, warranting designation "emery-Dreifuss" type.