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PATIENT SAFETY

Core Myopathies and Risk of Malignant Hyperthermia

Werner Klingler, MD^*, Henrik Rueffert, MD, Frank Lehmann-Horn, MD, Thierry Girard, MD, and Philip M. Hopkins, MD^||

From the Departments of *Anaesthesiology, and Applied Physiology, University of Ulm, Ulm, Germany; Department of Anaesthesiology and Intensive Care Medicine, University of Leipzig, Leipzig, Germany; Department of Anaesthesia, University Hospital of Basel, Basel, Switzerland; and ||Academic Unit of Anaesthesia, St. James's University Hospital, Leeds, UK.

Address correspondence and reprint requests to Philip M. Hopkins, MD, Academic Unit of Anaesthesia, University of Leeds, St James's University Hospital, Leeds LS9 7TF, UK. Address e-mail to p.m.hopkins{at}leeds.ac.uk.

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.

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