Pathophysiologic and anesthetic considerations for patients with myotonia congenita or periodic paralyses

Abstract

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.

Keywords: anesthetic considerations; hyperkalemic and hypokalemic periodic paralysis; myotonia congenita.