Involvement of respiratory muscles in cytoplasmic body myopathy - A pathological study

E. Bertini, E. Ricci, R. Boldrini, S. Servidei, S. Fusilli, C. Dionisi-Vici, C. Bosman, E. Bonilla

Research output: Contribution to journalArticlepeer-review

Abstract

A muscle biopsy and autopsy study of a child who died at 14 months of respiratory failure is described. A diagnosis of infantile cytoplasmic body myopathy was made due to the high percentage of cytoplasmic bodies (CBs), particularly in respiratory muscles. No pathological abnormalities were found in the central nervous system, peripheral nerves or visceral organs. Immunohistochemical studies suggested that the central core of CBS was stained for fibrillary actin, being surrounded by a positive signal for desmin. A differential diagnosis as to other conditions involving proliferation of CBs is discussed.

Original languageEnglish
Pages (from-to)798-806
Number of pages9
JournalBrain and Development
Volume12
Issue number6
Publication statusPublished - 1990

Keywords

  • actin
  • Cytoplasmic bodies
  • cytoplasmic body myopathy
  • desmin
  • respiratory failure

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Neurology

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