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Muscle histology in becker muscular dystrophy

  • National Center of Neurology and Psychiatry Kodaira

Research output: Contribution to journalArticlepeer-review

25 Scopus citations

Abstract

Twenty patients with Becker muscular dystrophy (BMD), confirmed by dystrophin tests, were studied histologically. There were several morphological differences between younger (≤15‐year‐old) and older (>15‐year‐old) patients. In the younger patients, active muscle fiber necrosis followed by a regenerating process was conspicuous. In the older patients, the active degenerative changes appeared less prominent and, instead, more chronic myopathic changes such as moth‐eaten fibers, fiber splitting, and hypertrophic fibers were evident. These age‐dependent differences in the pathology of BMD were irrespective of the duration of clinical symptoms, i.e., BMD patients of a similar age showed a similar morphological feature regardless of age at onset. Although the presence of mild fiber type grouping and some small angulated atrophic fibers suggested a certain degree of neurogenic involvement, none of biopsies showed significant grouped atrophy as seen in neuropathic disorders. There was no correlation between the histological changes and the specific dystrophin abnormality.

Original languageEnglish
Pages (from-to)1067-1073
Number of pages7
JournalMuscle and Nerve
Volume14
Issue number11
DOIs
StatePublished - Nov 1991

Keywords

  • Becker muscular dystrophy
  • dystrophin
  • fiber type analysis
  • muscle histochemistry
  • myonecrosis

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