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发作性睡病伴猝倒症状缓解及食欲素持续缺乏

Narcolepsy with resolution of cataplexy and persisting orexin deficiency.

作者信息

Wasserman Danielle, Bassetti Claudio L A, Rosenzweig Ivana

机构信息

Sleep and Brain Plasticity Centre, Department of Neuroimaging, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King's College London, United Kingdom.

Sleep Disorders Centre, Guy's and St, Thomas' National Health Service Foundation Trust, London, United Kingdom.

出版信息

J Clin Sleep Med. 2020 Aug 15;16(8):1383-1386. doi: 10.5664/jcsm.8572.

Abstract

Narcolepsy type 1 is a debilitating chronic neurological disorder, whose main symptoms of excessive daytime sleepiness and cataplexy may partially improve with time, but typically do not fully resolve. The irreversible loss of orexin neurons is considered to be the pivotal mechanistic link underlying the development of cataplectic attacks in narcolepsy type 1. Here we describe a case of untreated narcolepsy type 1with low cerebrospinal orexin levels (< 50 pg/mL), where cataplexy fully resolved in the first 5-6 years after disease onset, whereas excessive daytime sleepiness persisted.

摘要

1型发作性睡病是一种使人衰弱的慢性神经疾病,其主要症状日间过度嗜睡和猝倒可能会随时间部分改善,但通常不会完全消失。食欲素神经元的不可逆丧失被认为是1型发作性睡病中猝倒发作发展的关键机制环节。在此,我们描述一例未经治疗的1型发作性睡病患者,其脑脊液中食欲素水平较低(<50 pg/mL),该患者的猝倒在疾病发作后的最初5 - 6年内完全消失,而日间过度嗜睡仍然存在。

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