title: [Are morphologic changes in skeletal muscles of patients with malignant hyperthermia diagnostically useful?].
abstract: Malignant Hyperthermia (MH) represents a functional myopathy triggered by volatile anesthetics and depolarizing muscle relaxants, and leading to metabolic disturbances of intracellular Calcium homeostasis. Central-Core-like-structures (CCLS) were recently described as central defects in enzyme-histochemical stains and well correlated to the autosomal-dominant MH-predisposition. We studied the correlation of a MH-predisposition with specific myopathological signs. Skeletal muscles of suspected MH-individuals were histochemically stained by SDH-, NADH-, COX-, Gomori-Trichrome-, ATPase-, Acid Phosphatase-, Oil-red O- und PAS-stain und evaluated without knowing MH-diagnosis by the in-vitro-contracture test. Out of 118 patients (30% MHS ["susceptible"], 63% MHN [normal], 7% MHE ["equivocal"]) 19% revealed pathological findings corresponding to CCLS. 45% of these findings were associated with MHS/MHE. With HE-staining internal nuclei were not specific, but increased with the probability of MHS/MHE from 24% to 80%. Central Cores were correlated in 100% with MHS/MHE (4 out of 118 patients). CCLS were found with about similar frequency in skeletal muscle of MHS/MHE and MHN individuals. Internal nuclei were, however, not specifically, associated with MHS. In contrast, Central Cores correlated significantly with MHS/MHE diagnosis. In conclusion, histopathological findings in skeletal muscle seem to be a reliable marker for MH-predisposition only with Central Cores.
authors.affiliation: Klinik für Anästhesiologie, Universität Würzburg. ANETS@ANAESTHESIE.UNI-WUERZBURG.DE
authors.forename: M
authors.initials: M
authors.lastname: Anetseder
authors.forename: F
authors.initials: F
authors.lastname: Pohl
authors.forename: R
authors.initials: R
authors.lastname: Klein
authors.forename: R
authors.initials: R
authors.lastname: Müller
authors.forename: A
authors.initials: A
authors.lastname: Hoyer
authors.forename: H
authors.initials: H
authors.lastname: Horbaschek
authors.forename: W
authors.initials: W
authors.lastname: Roggendorf
authors.forename: E
authors.initials: E
authors.lastname: Hartung
authors.forename: N
authors.initials: N
authors.lastname: Roewer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D008305:Malignant Hyperthermia / Q000175:diagnosis* / Q000201:enzymology / Q000473:pathology / Q000503:physiopathology; D008875:Middle Aged; D009119:Muscle Contraction; D018482:Muscle, Skeletal / Q000201:enzymology / Q000473:pathology* / Q000503:physiopathology; D013194:Staining and Labeling