Psychostimulants for hypersomnia (excessive daytime sleepiness) in myotonic dystrophy.

backgroundExcessive daytime sleepiness is a common symptom of myotonic dystrophy. Psychostimulants are drugs that are increasingly used to treat hypersomnia in myotonic dystrophy.

objectivesTo assess the effects of psychostimulants in myotonic dystrophy patients with hypersomnia. SEARCH

methodsWe searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, ClinicalTrials.gov, and WHO ICTRP on 5 January 2023. We also checked the bibliographies of identified papers and made enquiries of the authors of the papers. SELECTION CRITERIA: We considered all randomised controlled trials that have evaluated any type of psychostimulant (versus a placebo or no treatment) in children or adults with myotonic dystrophy, confirmed by clinical and electromyographic diagnostic, or genetic testing, and hypersomnia. DATA COLLECTION AND ANALYSIS: Two review authors independently scrutinised potentially relevant papers for study inclusion, with any disagreements resolved by discussion. Two review authors independently performed data extraction. We obtained unpublished data from some study authors. We assessed the methodological quality of trials and applied GRADE to assess the certainty of evidence. Review authors did not contribute to eligibility or risk of bias assessment or data extraction of trials in which they had participated. When cross-over trials were included in the analysis, treatment effects were summarised as mean difference (MD) between treatment effects and standard error, and analysed by generic inverse variance. MAIN

resultsWe included six trials (136 participants). All studies included only adult outpatients, aged from 18 to 70 years old, and followed them only in the short term (up to four weeks). Five trials had a cross-over design. We judged five trials as being at low risk of bias. Primary outcome Data for mean improvement in the Maintenance of Wakefulness Test were available from three trials. The MD was 3.59 (95% confidence interval (CI) -0.06 to 7.24) minutes, and there was marked heterogeneity across studies (I AUTHORS'

conclusionsIn myotonic dystrophy, the effects of psychostimulants on excessive daytime sleepiness as assessed by the Maintenance of Wakefulness Test or Multiple Sleep Latency Test and on quality of life are very uncertain. Psychostimulants may improve hypersomnia as self-evaluated by the Epworth Sleepiness Scale and may increase the risk of adverse events. More randomised trials are needed to evaluate the efficacy and safety of psychostimulants in both the short and long term.