We report the case of a 28 year-old woman with left scapuloperoneal syndrome since the
age of 24. The course was slowly progressive and diffuse weakness was observed 4 years
later. Serum creatine kinase levels were moderately elevated (× 3 normal value) and EMG
showed mixed neurogenic and myogenic patterns. Muscle biopsy showed type I
predominance and numerous reducing bodies in muscle fibers. Reducing bodies were
strongly immunoreactive with antibodies to dystrophin, α-sarcoglycan, vimentin and …