Key result
This review outlines the pathomechanisms of myotonia congenita and periodic paralyses and provides recommendations for the management of anesthesia in affected patients.
Provides recommendations for the anesthetic management of patients with hereditary skeletal muscle channelopathies.
Myotonia congenita and periodic paralyses are hereditary skeletal muscle channelopathies. In these disorders, various channel defects in the sarcolemma lead to a severely disturbed membrane excitability of the affected skeletal muscles. The clinical picture can range from severe myotonic reactions (e.g., masseter spasm, opisthotonus) to attacks of weakness and paralysis. Provided here is a short overview of the pathomechanisms behind such wide-ranging phenotypic presentations in these patients, followed by recommendations concerning the management of anesthesia in such populations.
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Bandschapp et al. (2013) conducted a review in Myotonia congenita and periodic paralyses. Anesthesia management was evaluated. This review outlines the pathomechanisms of myotonia congenita and periodic paralyses and provides recommendations for the management of anesthesia in affected patients.
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