巴氯芬治疗发作性睡病日间过度嗜睡的管理。

Management of Excessive Daytime Sleepiness in Narcolepsy With Baclofen.

机构信息

Department of Child Neurology, Geisinger Medical Center, Janet Weis Children's Hospital, Danville, Pennsylvania; Department of Sleep Medicine, Geisinger Medical Center, Janet Weis Children's Hospital, Danville, Pennsylvania.

Division of Pediatric Pulmonology and Cystic Fibrosis Center, Cohen Children's Medical Center, Lake Success, New York; Pediatric Neurology Service Line for Northwell Health, Department of Pediatrics & Neurology, Zucker School of Medicine at Hofstra/Northwell, Cohen Children's Medical Center, Lake Success, New York.

出版信息

Pediatr Neurol. 2019 Apr;93:39-42. doi: 10.1016/j.pediatrneurol.2018.10.020. Epub 2018 Nov 22.

DOI:10.1016/j.pediatrneurol.2018.10.020

PMID:30595352

Abstract

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.

摘要

背景

发作性睡病是一种使人丧失能力的睡眠-觉醒障碍，其特征是出现日间过度嗜睡、睡眠瘫痪、睡眠碎片化、睡眠相关幻觉和猝倒这五组症状。目前尚无针对发作性睡病的治愈性疗法。因此，治疗主要针对症状。症状管理通常旨在改善日间过度嗜睡、睡眠碎片化和猝倒。针对日间过度嗜睡的一线治疗通常是每日使用促觉醒药物，如莫达非尼或阿莫达非尼，或兴奋剂治疗，如哌甲酯或安非他命。或者，也可以每晚使用羟丁酸钠来改善猝倒、睡眠巩固和次日的清醒度。由于疗效不足、耐受性差或副作用，这些疗法在某些患者中可能受到限制。