[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: M Anetseder, F Pohl, R Klein, R Müller, A Hoyer, H Horbaschek, W Roggendorf, E Hartung, N Roewer
• Journal: Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS (Anasthesiol Intensivmed Notfallmed Schmerzther) Vol. 34 Issue 10 Pg. 626-33 (Oct 1999) ISSN: 0939-2661 [Print] Germany
• Vernacular Title: Sind morphologische Veränderungen im Skelettmuskel von Patienten mit Maligner Hyperthermie diagnostisch verwertbar?
• PMID: 10548959 (Publication Type: Clinical Trial, English Abstract, Journal Article)

Topics

• Adolescent
• Adult
• Aged
• Child
• Child, Preschool
• Female
• Histocytochemistry
• Humans
• Male
• Malignant Hyperthermia (diagnosis, enzymology, pathology, physiopathology)
• Middle Aged
• Muscle Contraction
• Muscle, Skeletal (enzymology, pathology, physiopathology)
• Staining and Labeling