Summary
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:
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1.
Muscle cells of normal diameter without any distinct or only slight changes.
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2.
Small muscle cells with a large central nucleus with the appearance of myoblast at a later stage of development.
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3.
Cells resembling myotubes containing in a common basement membrane two to four cells at different stages of maturation.
Myoblast and myotube-like cells seemed to resemble foetal muscle fibres. Such fibres appear to represent an arrest in maturation rather than muscle atrophy.
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This work was supported with the Polish Academy of Science within the project 09.4.1.
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Fidziańska, A. Ultrastructural changes in muscle in spinal muscular atrophy —Werdnig-Hoffmann's disease. Acta Neuropathol 27, 247–256 (1974). https://doi.org/10.1007/BF00687634
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DOI: https://doi.org/10.1007/BF00687634