[Are morphologic changes in skeletal muscles of patients with malignant hyperthermia diagnostically useful?].
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.
Anetseder
M
M
Klinik für Anästhesiologie, Universität Würzburg. ANETS@ANAESTHESIE.UNI-WUERZBURG.DE
Pohl
F
F
Klein
R
R
Müller
R
R
Hoyer
A
A
Horbaschek
H
H
Roggendorf
W
W
Hartung
E
E
Roewer
N
N
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