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Feinstrukturelle Veränderungen von Muskelfasern bei progressiver Muskeldystrophie vom Typ Duchenne

Ultrastructural changes of muscle fibers in progressive muscular dystrophy of Duchenne type

Summary

Examinations of 10 muscle biopsies in Duchenne type of progressive muscular dystrophy were performed. The different particular myopathic changes are analysed and their specificity is discussed. Two groups of changes are observed, one concerning total fiber involvement, the other the focal degenerations. In the first group muscle fiber necrosis, associated regenerations, fiber degeneration with initial disturbance of the filamentous arrangement and atrophia simplex are described. Focal myopathic changes appeared predominantly in type I fibers and consisted of Z-streaming and lipid storage in central fiber parts. None of the particular fiber changes are specific, only the distribution of their frequency shows a characteristic pattern. Pathogenic factors of the different particular fiber changes are discussed, especially of the lipid accumulation in the type I fibers.

Zusammenfassung

Muskelgewebe von 10 Knaben mit Duchennescher Form von PMD wurden licht- und elektronenmikroskopisch untersucht. Die einzelnen myopathischen Veränderungen wurden feinstrukturell analysiert und hinsichtlich ihrer Spezifität beurteilt. Es treten Fasernekrose, davon abhängig Faserregenerate, weiters Faserdegenerationen mit primärer Filamentenordnungsstörung und konsekutiver Filamentendegeneration, sowie einfache Faseratrophie als Gesamtfaserschädigung auf. Fokale myopathische Veränderungen, vorwiegend auf Typ I-Fasern beschränkt, sind Z-Streaming und Lipidspeicherung in zentralen Faserabschnitten. Keine der Einzelveränderungen ist für sich spezifisch, lediglich ihre Häufigkeitsverteilung ergibt ein charakteristisches Muster. Pathogenetische Faktoren der Einzelveränderungen, besonders Lipidakkumulation in Typ I-Fasern, werden diskutiert.

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Ostenda, M., Sluga, E. Feinstrukturelle Veränderungen von Muskelfasern bei progressiver Muskeldystrophie vom Typ Duchenne. Acta Neuropathol 18, 173–189 (1971). https://doi.org/10.1007/BF00687605

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Key-Words

  • Progressive Muscular Dystrophy
  • Duchenne Type
  • Ultrastructure
  • Lipidstorage
  • Type I Fibers