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Autosomal recessive distal dystrophy

Department of Medicine (Dr. Barohn), Division of Neurology, University of Texas Health Science Center at San Antonio, San Antonio, TX; Department of Neurology (Dr. Miller), Children's Hospital of San Francisco, San Francisco, CA; and Department of Neurology (Dr. Griggs), University of Rochester School of Medicine and Dentistry, Rochester, NY.

We describe five new cases of autosomal recessive distal dystrophy (Miyoshi myopathy) and emphasize the distinctive clinical and laboratory features of this unusual muscular dystrophy. Symptoms began at age 15 to 25, the gastrocnemius muscles were selectively involved, and creatine kinase was elevated more than 10 times normal. The EMG showed abundant brief motor units with numerous fibrillations. Dystrophic features without vacuoles were best seen in the biceps femoris muscle. Asymptomatic creatine kinase elevation was present years prior to the development of weakness. The disorder appears to be inherited in an autosomal recessive pattern. Miyoshi myopathy can be distinguished from other distal muscular dystrophies. We propose a new classification for the distal muscular dystrophies.

Address correspondence and reprint requests to Dr. Richard J. Barohn, Assistant Professor of Medicine (Neurology), Department of Medicine (Neurology), University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78284.

Received January 10, 1991. Accepted for publication in final form February 21, 1991.

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