Core myopathies and risk of malignant hyperthermia.

Werner Klingler, Henrik Rueffert, Frank Lehmann-Horn, Thierry Girard, Philip M Hopkins

Journal: Anesthesia and analgesia 2009;109(4):1167-73

PMID: 19762745

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Abstract

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.

Address: Department of Anaesthesiology, University of Ulm, Ulm, Germany.

DOI: 10.1213/ANE.0b013e3181b5ae2d

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MeSH Terms: Anesthesia, Calcium, Genetic Predisposition to Disease, Humans, Malignant Hyperthermia, Muscle, Skeletal, Muscular Diseases, Mutation, Myopathies, Nemaline, Myopathy, Central Core, Phenotype, Risk Assessment, Risk Factors, Ryanodine Receptor Calcium Release Channel