Abundant minute myotubes in a patient who later developed centronuclear myopathy

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ID: 117561
1970
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Abstract
Centronuclear myopathy (CNM) is a congenital myopathy which manifests itself as a severe neonatal (also termed myotubular myopathy), early-onset, or adult form. The histological pattern of each is marked by a considerable number of nuclei of muscle fibers being internally placed. Owing to their remote resemblance to myotubes, and their expression of developmentally regulated proteins, most authors now favor the concept that myogenesis is arrested or delayed in this disease. We here present two muscle biopsy specimens of a patient with early-onset CNM, taken at the age of 5 months and 14 years, respectively. The first biopsy sample contained internally placed nuclei in 7% of the muscle fibers, abundant minute myotubes, and hypertrophic muscle fibers. The second biopsy sample showed internally placed nuclei in 40% of the muscle fibers, and hypotrophic fibers. We suggest that the histological findings in early-onset CNM are the result of a complex dynamic process, which includes a delay in maturation.
Reference Key
wöckel1970actaabundant Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Lars Wöckel;Uwe-Peter Ketelsen;Mechthild Stötter;Sven Laule;Richard Meyermann;A. Bornemann;Lars Wöckel;Uwe-Peter Ketelsen;Mechthild Stötter;Sven Laule;Richard Meyermann;A. Bornemann;
Journal acta neuropathologica
Year 1970
DOI
doi:10.1007/s004010050836
URL
https://link.springer.com/article/10.1007/s004010050836
Keywords
pathology neurosciences

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