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

Oliver Bandschapp, Paul A Iaizzo

Research output: Contribution to journalReview article

13 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)824-833
Number of pages10
JournalPaediatric anaesthesia
Volume23
Issue number9
DOIs
StatePublished - Sep 1 2013

Keywords

  • anesthetic considerations
  • hyperkalemic and hypokalemic periodic paralysis
  • myotonia congenita

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