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July 11, 2001

Malignant Hyperthermia and Apparent Heat Stroke

Author Affiliations

Stephen J.LurieMD, PhD, Senior EditorIndividualAuthorJody W.ZylkeMD, Contributing EditorIndividualAuthor

JAMA. 2001;286(2):168-169. doi:10.1001/jama.286.2.168

To the Editor: Malignant hyperthermia (MH) is an autosomal dominant condition in which certain anesthetics trigger calcium dysregulation in skeletal muscle, resulting in a catastrophic, life-threatening hypermetabolic syndrome.1 More than 50% of families with MH have mutations in the gene encoding the ryanodine receptor (RYR1).2 In a porcine model of MH, nonanesthetic, stress-induced deaths have been reported in pigs homozygous for the Arg614Cys mutation in the RYR1 gene,3 but this phenomenon has not been reported in humans with MH mutations. To our knowledge, we report the first case of nonanesthetic, stress-induced hyperpyrexic death in an individual with a history of MH.