title: [Malignant hyperthermia].
abstract: Malignant hyperthermia is an autosomal dominant disorder of the skeletal muscle that predisposes affected individuals to a life-threatening hypermetabolic reaction in response to volatile anaesthetics and depolarizing muscle relaxants. The underlying heterogeneous genetic defects are mainly point mutations within the ryanodine receptor gene of the sarcoplasmic reticulum. Following the introduction of efficient diagnostic and therapeutic tools--the in vitro contracture test and intravenous treatment with dantrolene--a dramatic decline in mortality rates has been observed. The association of malignant hyperthermia-like reactions with other neuromuscular disorders requires the collaboration of several clinical disciplines to achieve a timely recognition of this still life-threatening disorder.
authors.affiliation: Universitätsklinik für Neurologie, Wien, Osterreich.
authors.forename: Fritz
authors.initials: F
authors.lastname: Zimprich
authors.forename: Hans Georg
authors.initials: HG
authors.lastname: Kress
authors.forename: Josef
authors.initials: J
authors.lastname: Zeitlhofer
mesh_terms: D000328:Adult; D001706:Biopsy; D002648:Child; D003620:Dantrolene / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007275:Injections, Intravenous; D008305:Malignant Hyperthermia / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000401:mortality / Q000473:pathology; D009119:Muscle Contraction; D009125:Muscle Relaxants, Central / Q000008:administration & dosage / Q000627:therapeutic use; D018482:Muscle, Skeletal / Q000187:drug effects / Q000473:pathology; D017354:Point Mutation; D012307:Risk Factors; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D012519:Sarcoplasmic Reticulum / Q000235:genetics; D012680:Sensitivity and Specificity
chemical_list: D009125:Muscle Relaxants, Central; D019837:Ryanodine Receptor Calcium Release Channel; D003620:Dantrolene