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Source: Damian MS, et al. Modafinil for excessive daytime sleepiness in myotonic dystrophy. Neurology. 2001;56: 794-796.
Hypersomnia is a prominent feature of myotonic dystrophy (MyD), although, interestingly, it is more often remarked upon by relatives than by patients. Not felt to be true narcolepsy, its cause remains obscure. Modafinil, useful in narcolepsy, may nevertheless be efficacious for excessive daytime sleepiness (EDS) in MyD. In an open label trial, Damian and colleagues describe 8 patients with genetically proven MyD (expanded CTG repeat on chromosome 19q13.13) and 1 with maternally inherited proximal myotonic myopathy (PROMM). Each patient received 200-400 mg/d modafinil for a minimum of 16 weeks. All felt disabled by their EDS prior to the modafinil. With treatment, mean sleep latency (measured by MSLT) significantly increased, mean Epworth Sleepiness Scale score decreased, and all patients felt subjectively better. Modafinil was well tolerated, without significant side effects, blood pressure remained normal, and nighttime sleep was unimpaired. Clearly, it is time to proceed with a placebo-controlled, double-blind, multicenter trial.
With this effective response and the relatively small number of persons who used it, it seems to Damian et al that its duration of effect should first be tested by the enlarging pioneer patients and providing a 6-month test time table.—Michael Rubin