An unusual variant of Becker muscular dystrophy.

de Visser, M; Bakker, E; Defesche, J C; Bolhuis, P A; van Ommen, G J · Ann Neurol · 1990

case_series · Level IV

Where this comes from

Abstract

We report on 5 brothers with slowly progressive limbgirdle weakness. Calf hypertrophy was absent. The levels of creatine kinase, electromyography, and findings from a muscle biopsy specimen were compatible with muscular dystrophy. The propositus's biopsy specimen also showed numerous rimmed vacuoles. DNA analysis revealed a deletion in the dystrophin gene, establishing a diagnosis of Becker muscular dystrophy. Both the absence of calf hypertrophy and the presence of rimmed vacuoles are unusual features in this disorder.

Medical subject headings