Muscle channelopathies: management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Muscle channelopathies: management. Lamotrigine: this is recommended as first line, Mexiletene. Fully referenced neurology checklist.

Evidenced-treatment of myotonia

  • Lamotrigine: this is recommended as first line
  • Mexiletene
  • Lidocaine: for myotonic crisis

Less-evidenced treatment of myotonia

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Anaesthesia management

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Pregnancy management

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School management

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Lifestyle modification

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References

  1. Matthews E, Neuwirth C, Jaffer F, et al. Atypical periodic paralysis and myalgia: A novel RYR1 phenotype. Neurology 2018; 90:e412-e418. 
  2. Matthews E, Palace J, Ramdas S, et al. Care recommendations for the investigation and management of children with skeletal muscle channelopathies. Pediatr Neurol 2023; 145:102-111.
  3. Andersen G, Hedermann G, Witting N, Duno M, Andersen H, Vissing J. The antimyotonic effect of lamotrigine in non-dystrophic myotonias: a double-blind randomized study. Brain 2017; 140:2295-2305.
  4. Statland JM, Bundy BN, Wang Y, et al; Consortium for Clinical Investigation of Neurologic Channelopathies. Mexiletine for symptoms and signs of myotonia in nondystrophic myotonia: a randomized controlled trial. JAMA 2012; 308:1357-1365.

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