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Drug treatment for myotoniaTrip J, Drost GG, van Engelen BGM, Faber CG
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SummaryDrug therapy to treat myotonia (delayed muscle relaxation after contraction) in muscle diseases such as myotonic dystrophy and myotonia congenitaMyotonia is an abnormal delay in the relaxation of muscles after contraction. It is a key symptom in a number of muscle diseases called myotonic disorders. It can be mild or severe, interfering with daily activities such as walking, climbing stairs or opening and closing the eyelids. It can be worse after periods of rest or triggered by cold or fatigue. People with mild myotonia can manage their disease without medication but in severe cases treatment is usually necessary. Drugs that have been used to treat myotonia include sodium channel blockers such as procainamide, phenytoin and mexiletine, tricyclic antidepressant drugs such as clomipramine or imipramine, benzodiazepines, calcium antagonists, taurine and prednisone. This review describes ten randomised controlled trials which review the effectiveness of twelve different drug treatments. The ten trials included a total of 143 participants of which 113 had myotonic dystrophy and 30 had myotonia congenita. The trials were generally small and of poor quality. Meta-analysis was not possible due to a lack of appropriate trials and data. Two small studies indicated that clomipramine and imipramine had a short-term beneficial effect on the myotonia of myotonic dystrophy and one small study indicated that taurine had a long-term beneficial effect in myotonic dystrophy. Minor side effects such as dry mouth and dizziness were reported with clomipramine and imipramine, but not with taurine. It was not possible to determine whether drug therapy is safe and effective for myotonia in the treatment of people with a myotonic disorder based on the evidence from the ten trials included in this review. Larger, well-designed randomised controlled trials are needed.
This is a Cochrane review abstract and plain language summary, prepared and maintained by The Cochrane Collaboration, currently published in The Cochrane Database of Systematic Reviews 2010 Issue 1, Copyright © 2010 The Cochrane Collaboration. Published by John Wiley and Sons, Ltd.. The full text of the review is available in The Cochrane Library (ISSN 1464-780X).
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January 25. 2006 AbstractBackgroundAbnormal delayed relaxation of skeletal muscles, known as myotonia, can cause disability in myotonic disorders. Sodium channel blockers, tricyclic antidepressive drugs, benzodiazepines, calcium-antagonists, taurine and prednisone may be of use in reducing myotonia. ObjectivesTo consider the evidence from randomised controlled trials on the efficacy and tolerability of drug treatment in people with clinical myotonia due to a myotonic disorder. Search strategyWe searched the Cochrane Neuromuscular Disease Group Trials Register (January 2008), MEDLINE (January 1966 to December 2007) and EMBASE (January 1980 to December 2007). Grey literature was handsearched and reference lists of identified studies and reviews were examined. Authors, disease experts and manufacturers of anti-myotonic drugs were contacted. Selection criteriaWe considered all (quasi) randomised trials of participants with myotonia treated with any drug treatment versus no therapy, placebo or any other active drug treatment. The primary outcome measure was: Secondary outcome measures were: Data collection and analysisTwo review authors extracted the data independently onto standardised extraction forms and disagreements were resolved by discussion. Main resultsTen randomised controlled trials were found comparing active drug treatment versus placebo or another active drug treatment in participants with myotonia due to a myotonic disorder. Included trials were double-blind or single-blind crossover studies involving a total of 143 participants of which 113 had myotonic dystrophy type 1 and 30 had myotonia congenita. The studies were of poor quality. Therefore, we were not able to analyse the results of all identified studies. Two small crossover studies without a washout period demonstrated a significant effect of imipramine and taurine in myotonic dystrophy. One small crossover study with a washout period demonstrated a significant effect of clomipramine in myotonic dystrophy. Meta-analysis was not possible. Authors' conclusionsDue to insufficient good quality data and lack of randomised studies, it is impossible to determine whether drug treatment is safe and effective in the treatment of myotonia. Small single studies give an indication that clomipramine and imipramine have a short-term beneficial effect and that taurine has a long-term beneficial effect on myotonia. Larger, well-designed randomised controlled trials are needed to assess the efficacy and tolerability of drug treatment for myotonia. |