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"Cap disease"

New congenital myopathy

Department of Neurology, School of Medicine, Warsaw, Poland

In a 7-year-old boy with delayed motor development, a congenital non progressive myopathy was diagnosed. Histochemical and ultrastructural examination of a muscle specimen revealed unusual pathologic findings. In 70% of muscle fibers, peripherally located zones lacking in ATPase activity and consisting of abnormally arranged myofibrils were observed. The characteristic position of the peripherally located myofibrils and their abnormal sarcomere pattern seem to point to an error in the fusion as well as in the synthesis of muscle protein.

Address correspondence and reprint requests to Dr Fidzianska, Akademia Medyczna W Warszanne, Klinika Neurologiczna, 02 007 Wopszawa, Poland

The study was supported by NIH-Bethesda-Marie Sklodowska-Curie Joint Fund under agreement No J-05-09-4-N and AMDA grant 1979–1980

Accepted for publication October 22, 1980.

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