title: [Myotonic dystrophy severity (Steinert's disease) (author's transl)].
abstract: Four family cases of Steinert, myodystrophy are reported. The severity of the early onset form is specially noted. This form presents pulmonary complications and mental deficit. The influence of the myodystrophy on the pregnancy is discussed and an explanation of the disturbance of the oesophaguspharynx motility is presented. Physiopathogenesis problems of the Steinert's disease are noted.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004576:Electromyography; D004947:Esophagus / Q000503:physiopathology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D010375:Pedigree; D010614:Pharynx / Q000503:physiopathology; D012131:Respiratory Insufficiency / Q000209:etiology
vernacular_title: La gravité de la dystrophie myopathique myotonique de Steinert.