The Risk of Malignant Hyperthermia in Children With Suspected Myopathy

Despite continuing controversy, it is widely assumed that children with known or suspected myopathies are at increased risk for malignant hyperthermia (MH) (1). Previous reports have suggested a relationship between MH risk and a variety of neuromuscular disorders including Duchenne type muscular dystrophy (DMD), osteogenesis imperfecta, myotonia congenita, the Schwartz-Jampel syndrome, and others (2). Additionally, Kearns-Sayre syndrome and other mitochondrial myopathies have also been associated with an increased risk for MH (3,4). Others have failed to demonstrate an association between mitochondrial disorders and MH (5). There are currently only two disorders for which a clear link to MH has been established; they are central core disease and King syndrome. Both are quite rare, well defined clinically, autosomal dominant, and frequently diagnosed without muscle biopsy (6).