Rinsho Shinkeigaku (Clinical Neurology)


Malignant hyperthermia-like reactions in Duchenne or Becker muscular dystrophy: review and hypothesis

Akio Takagi, M.D.1)2) and Hirofumi Nakase, M.D.2)

1)Medical Office of MOF, Ministry of Finance [Kasumigaseki, Tokyo 100-8940, Japan]
2)Department of Neurology, Toranomon Hospital [Toranomon, Tokyo, 105-8470, Japan]

Adverse reactions to genral anesthesia, which partly resembled malignant hyperthermia (MH), were more frequent in muscular dystrophy than in controls. In the present study, 35 cases so far reported in Duchenne or Becker muscular dystrophy (DMD or BMD) were analyzed and their pathogenesis was discussed. Cardiac involvements were sole manifestations in 7 cases. In other 28 cases, the acute rhabdomyolysis was the most prevailing manifestation. About 60% of myolysis cases were associated with muscle contracture (rigidity) or other hypermetabolic signs such as hypercapnia, hyperthermia and metabolic acidosis. Cases with BMD were more hyperthermic than with DMD. These results suggest Ca ion-induced hypermetabolic reactions are also present in dystrophinopathy, which have been assumed as core syndromes of the classical (gene-defined) MH. However, question whether the abnormal Ca ion is from the extracellular or intracellular stores is still unclear. Circumstancial evidences suggest that the Ca-induced Ca release (CICR) mechanism might also be involved. Endogenous redox modulators such as nitric oxide or reactive oxygen species in the dystrophic muscle might contribute to the perturbed Ca ion homeostasis.
Full Text of this Article in Japanese PDF (118K)

(CLINICA NEUROL, 48: 101|105, 2008)
key words: malignant hyperthermia, dystrophinopathy, calcium ion, ryanodine receptor, redox modulators

(Received: 3-Sep-07)