Core Myopathies and Risk of Malignant Hyperthermia

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 ME 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. (Anesth Analg 2009;109:1167-73)