RESUMEN

The paper treats of a case of progressive myodystrophy coupled with multiple contractures of the large joints. In an Uzbek family placed under observation and living in the Uzbek SSR, the disease was revealed in 3 members of the family (in mother and two children). Contractures of the large joints grew on parallel with the myodystrophic process. The pattern of myodystrophy seen in the family was characterized by a number of unusual clinical features and by pronounced clinical pleomorphism. The differences of the clinical manifestations within the family lay in variability of the debut, localization and intensity of the contractures, and in the rate of the myodystrophy course.

Asunto(s)

Articulación del Tobillo , Contractura/genética , Articulación del Codo , Articulación de la Rodilla , Distrofias Musculares/genética , Articulación de la Muñeca , Adolescente , Adulto , Contractura/diagnóstico , Contractura/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Distrofias Musculares/clasificación , Distrofias Musculares/complicaciones , Linaje