Core myopathies and risk of malignant hyperthermia

Anesth Analg. 2009 Oct;109(4):1167-73. doi: 10.1213/ANE.0b013e3181b5ae2d.


In this article, we analyze myopathies with cores, for which an association to malignant hyperthermia (MH) has been suggested. We discuss the clinical features, the underlying genetic defects, subsequent effects on cellular calcium metabolism, and in vitro muscle responses to MH triggers. We describe in detail central core disease, multiminicore disease, and nemaline rod myopathy. We categorize the diseases according to the affected proteins and discuss the risk for MH, which is high or theoretically possible when the calcium-conducting proteins are affected.

Publication types

  • Review

MeSH terms

  • Anesthesia / adverse effects*
  • Calcium / metabolism
  • Genetic Predisposition to Disease
  • Humans
  • Malignant Hyperthermia / etiology*
  • Malignant Hyperthermia / metabolism
  • Muscle, Skeletal / metabolism*
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology
  • Muscular Diseases / complications*
  • Muscular Diseases / genetics
  • Muscular Diseases / metabolism
  • Mutation
  • Myopathies, Nemaline / complications
  • Myopathy, Central Core / complications
  • Phenotype
  • Risk Assessment
  • Risk Factors
  • Ryanodine Receptor Calcium Release Channel / genetics


  • Ryanodine Receptor Calcium Release Channel
  • Calcium