Distal Miyoshi muscular dystrophy on a Moroccan patient

BACKGROUND: Muscular dystrophy usually involves a deficiency in the proxima l muscles. We report a case of distal muscular dystrophy in a Moroccan pati ent CASE REPORT: A 21-year-old Moroccan complained of progressive onset weaknes s of the lower limbs and frequent falls. The neurological examination demon strated amyotrophy involving both legs and almost complete regression of th e posterior muscle compartment Clinical findings were confirmed by muscle s cans. The electromyogram showed a progressive myogenic process. Creatine ki nase was elevated. The severe dystrophic aspect was evidenced at muscle bio psy and led to the diagnosis of distal Miyoshi muscular dystrophy. DISCUSSION: Miyoshi muscular dystrophy, a distal myopathy transmitted by au tosomal recessive inheritance, is usually observed in young adults who pres ent characteristic amyotrophy of the posterior leg muscles. The proximal mu scles of the lower and upper limbs may become involved during the disease c ourse. Approximately one-third of the patients require wheel chair assistan ce after 10 years. Creatine kinase levels are constantly elevated. The diff erential diagnosis with other muscular dystrophies predominating in the dis tal muscles, particularly Steinert's myotonic dystrophy and inclusion body myositis, is discussed. (C) 1999, Masson, Paris.