A young adult male is described with muscular dystrophy of probable X-linked 
recessive inheritance. An onset of muscle weakness in late adolescence was 
preceded by contractures of the neck and elbows dating back to childhood. The 
distribution of muscle weakness was proximal in the upper limbs and both 
proximal and distal in the lower. The mixed pattern of muscle involvement in the 
legs favours the view that cases of Emery-Dreifuss muscular dystrophy with 
proximal weakness in both the upper and lower limbs and X-linked scapuloperoneal 
muscular dystrophy represent the same disorder. A muscle biopsy in the present 
case showed unique appearances.