[Late onset Becker muscular dystrophy. A case report and literature review]. / Dystrophie musculaire de Becker à révélation tardive. À propos d'un nouveau patient et de 12 observations de la littérature.

ABSTRACT

INTRODUCTION: The first manifestations of Becker's muscular dystrophy usually occur during childhood, as Duchenne's muscular dystrophy, a related disease. However, clinical presentation is usually more heterogeneous and less severe. CASE REPORT We report a 54-year-old man who presented with a late onset of Becker's muscular dystrophy, and review 12 additional similar cases previously reported in the literature. The disease onset ranged from 30 to 65 years old. Genetic deletion involved mainly exons 45 to 55 with variable phenotype distal muscular hypertrophy in four cases, cardiac involvement (electrocardiography, radiography or echocardiography) in six out of the 12 studied cases. Compared to the paediatric form, functional impairment was milder, as only three patients after seven to 20 years of follow-up were wheelchair bound. CONCLUSION: The first manifestations of Becker's muscular dystrophy may occur after the age of 30 with heterogeneous phenotypes. Severity and limb disability is milder in these patients.