Sporadic inclusion body myositis: morphology, regeneration, and cytoskeletal structure of muscle fibres

J Neurol Neurosurg Psychiatry. 2004 Jun;75(6):917-20. doi: 10.1136/jnnp.2003.018994.

Abstract

Objective: To characterise morphological abnormalities in relation to muscle fibre type in sporadic inclusion body myositis (s-IBM).

Methods: 14 muscle biopsies from 11 patients with s-IBM were characterised for morphological abnormalities and fibre type composition as well as muscle fibre regeneration and cytoskeletal structure, using histochemical and immunohistochemical techniques.

Results: Morphological abnormalities included inflammatory infiltrates and "rimmed vacuoles," and pronounced variation in fibre size. There were no significant differences in fibre type composition between s-IBM patients and controls based on the myofibrillar ATPase staining. A differential effect on muscle fibre sizes was noted, type II fibres being smaller in the s-IBM patients than in the controls. Conversely, the mean type I muscle fibre diameter in the s-IBM patients was larger than in the controls, though this difference was not significant. An ongoing intense regeneration process was present in s-IBM muscle, as indicated by the expression of neonatal myosin heavy chain, vimentin, and CD56 (Leu-19) in most of the muscle fibres. The cytoskeletal proteins dystrophin and desmin were normally expressed in s-IBM muscle fibres that were not undergoing degeneration or regeneration.

Conclusions: There are extensive morphological and morphometric alterations in s-IBM, affecting different muscle fibre types in different ways. The cytoskeletal structure of type I and II muscle fibres remains unaffected in different stages of the disease.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Biopsy
  • Cytoskeletal Proteins / metabolism
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Muscle Fibers, Fast-Twitch / cytology
  • Muscle Fibers, Fast-Twitch / metabolism
  • Muscle Fibers, Fast-Twitch / pathology
  • Muscle Fibers, Skeletal / metabolism
  • Muscle Fibers, Skeletal / pathology
  • Muscle Fibers, Skeletal / physiology
  • Muscle Fibers, Slow-Twitch / cytology
  • Muscle Fibers, Slow-Twitch / metabolism
  • Muscle Fibers, Slow-Twitch / pathology
  • Muscle, Skeletal / metabolism
  • Muscle, Skeletal / pathology*
  • Muscle, Skeletal / physiology
  • Myosins / metabolism
  • Myositis, Inclusion Body / metabolism
  • Myositis, Inclusion Body / pathology*
  • Myositis, Inclusion Body / physiopathology
  • Regeneration / physiology

Substances

  • Cytoskeletal Proteins
  • Myosins