Abstract

An ultrastructural study was carried out on muscle biopsies of 7 children with Werdnig-Hoffmann's disease. The muscle fibres were classified into 3 categories: 1. Muscle cells of normal diameter without any distinct or only slight changes. 2. Small muscle cells with a large central nucleus with the appearance of myoblast at a later stage of development. 3. Cells resembling myotubes containing in a common basement membrane two to four cells at different stages of maturation.

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