Drug treatment for myotonia
Abstract
Background: Abnormal delayed relaxation of skeletal muscles, known as myotonia, can cause disability in myotonic disorders. The main myotonic disorders are non-dystrophic myotonia and myotonic dystrophy. Non-dystrophic myotonia is a genetic muscle channelopathy predominantly causing myotonia. Myotonic dystrophic is a more systemic neuromuscular disorder causing myotonia as well as progressive myopathy and systemic manifestations, such as arrhythmias and cataracts. Myotonia manifests as stiffness, cramps, locking, pain, and fatigue, and can cause marked morbidity and disability. Sodium channel blockers, tricyclic antidepressive drugs, benzodiazepines, calcium antagonists, taurine, and prednisone may reduce myotonia. This is an update of a review first published in 2005 and updated in 2006.
Objectives: To review evidence from randomised controlled trials (RCTs) on the efficacy and tolerability of drug treatment in people with clinical myotonia due to myotonic disorders.
Search methods: We searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, ClinicalTrials.gov, and World Health Organization ICTRP on 29 March 2023. We handsearched the grey literature and contacted disease experts and antimyotonic drug manufacturers.
Selection criteria: We included RCTs involving participants with myotonia treated with any drug treatment versus no therapy, placebo, or any other active drug treatment. We included clinical trials where the reported primary outcome was…
How strong is the evidence?
Evidence Hub reads this as a meta-analysis, done in people. Its evidence level is 1 of 5 (Highest · pooled evidence). Pooled evidence from several studies sits at the top of the evidence pyramid. Reported size: 392 participants or records.
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