Résumé :
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Objective: To evaluate the efficacy and tolerability of methylphenidate for the treatment of excessive daytime somnolence (EDS) in patients with myotonic dystrophytype 1(DM1). Methods: twenty-four patients with DM1 with the Epworth Sleepiness score at =10 were invited to participate in a randomized double-blind crossovertrial of 20mg/day methylphenidate versus placebo, with three weeks in each arm of the study separated by a 2-weeks washout period. Before and after each trial,subjects completed the Daytime Sleepiness scale (DSS), Epworth Sleepiness Scale (ESS), the quality-of-life measures (RAND 36-item) and the profile of Mood States(POMS). The mean latency sleep (MLS) was measured using the OLSER test. Results: The mean DSS scores decreased from 8,87 ± 0.53 to 6,11 ± 0.51 ascompared with placebo (P=0,0007) and that of ESS decreased from 10.38 ± 0.79 to 7.6 ± 0.69 (P=0,0032). There was no significant change in MLS (P=0,237) butmethylphenidate significantly (P=0.04) decreased the number of microsleeps compared with untreated or placebo. The POMS and RNAD 36-item quality of life scaleshowed no significant change. Three patients withdrew from the study because of side effects, one for diarrheas and two for irritability. Conclusion: Methylphenidatereduces significantly hypersomnolence and should be considered in the treatment of EDS in patients with DM1.
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