4.4 Article

Management of Excessive Daytime Sleepiness in Narcolepsy With Baclofen

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PEDIATRIC NEUROLOGY
卷 93, 期 -, 页码 39-42

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ELSEVIER SCIENCE INC
DOI: 10.1016/j.pediatrneurol.2018.10.020

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Narcolepsy; Cataplexy; GABA; Adolescent sleep; Baclofen; Sodium oxybate

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Background: Narcolepsy is a disabling sleep-wake disorder characterized by the pentad symptoms of excessive daytime sleepiness, sleep paralysis, sleep fragmentation, sleep-related hallucinations, and cataplexy. There is no curative therapy for narcolepsy. Treatment is therefore symptom directed. Symptom management is generally directed at improving excessive daytime sleepiness, sleep fragmentation, and cataplexy. First-line treatment for excessive daytime sleepiness is typically daily use of wake-promoting agents, such as modafinil or armodafinil, or stimulant therapy, such as methylphenidate or amphetamines. Alternatively, sodium oxybate can be used nightly for improved cataplexy, sleep consolidation, and following day wakefulness. These therapies can be limited in some patients because of inadequate efficacy, poor tolerability, or side effects. Methods: We describe five narcolepsy patients with severe excessive daytime sleepiness who had an inadequate response or experienced side effects with the initial therapies but had a positive response to treatment with baclofen. Results: These patients reported subjective improvement in sleep maintenance without fragmentation and daytime sleepiness. Average Epworth Sleepiness Scale assessment before treatment was 15.8 with post-treatment assessment being 10.4 (P < 0.05). Conclusions: Baclofen may be an effective treatment for excessive daytime sleepiness and sleep fragmentation in narcolepsy and warrants further study. (C) 2018 Elsevier Inc. All rights reserved.

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