Sodium channel defects in myotonia and periodic paralysis

Stephen C. Cannon

Research output: Contribution to journalReview articlepeer-review

67 Scopus citations


Myotonias and periodic paralyses constitute a diverse group of inherited disorders of muscle in which the primary defect is an alteration in the electrical excitability of the muscle fiber. The ion channel defects underlying these excitability derangements have recently been elucidated at the molecular and functional levels. This review focuses on sodium channel mutations that disrupt inactivation and thereby cause both the enhanced excitability of myotonia (muscle stiffness due to repetitive discharges) and the inexcitability resulting from depolarization during attacks of paralysis.

Original languageEnglish (US)
Pages (from-to)141-164
Number of pages24
JournalAnnual Review of Neuroscience
StatePublished - 1996


  • hyperkalemia
  • ion channel
  • muscle
  • paramyotonia

ASJC Scopus subject areas

  • Neuroscience(all)


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