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Core Myopathies and Risk of Malignant Hyperthermia

2009; Lippincott Williams & Wilkins; Volume: 109; Issue: 4 Linguagem: Inglês

10.1213/ane.0b013e3181b5ae2d

1526-7598

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

Advanced MRI Techniques and Applications

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.