Rapid eye movement sleep disturbances in Huntington disease
Rapid eye movement sleep disturbances in Huntington disease
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DOI：
10.1001/archneur.65.4.482
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发表时间：
2008-04-01
影响因子：
--
通讯作者：
Durr, Alexandra
中科院分区：
文献类型：
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作者：
Arnulf, Isabelle;Nielsen, Jorgen;Durr, Alexandra
背景：睡眠障碍包括失眠、睡眠中运动和白天嗜睡是亨廷顿病（HD）的常见疾病，但研究很少。目的：评价HD不同阶段患者睡眠-觉醒表型（包括睡眠中异常运动活动）及CAG废止的长度。因为在一些HD患者（以下简称HD患者）的大脑中发现了轻度的下丘脑分泌素缺乏，我们也对HD患者进行了发作性睡症的检测。设计和患者：对25例HD患者（包括2例先兆携带者）进行临床访谈、夜间视频和睡眠监测以及白天多次睡眠潜伏期试验。他们的结果与发作性睡病患者和对照组患者的结果进行了比较。结果：HD患者失眠频繁，睡眠时间早，睡眠效率低，I期睡眠增加，快速眼动（REM）睡眠延迟和缩短，周期性腿部运动增加。3例HD患者（12%）有REM睡眠行为障碍。CAG重复长度与睡眠异常无相关性。在前显性携带者和非常轻微的HD患者中存在快速眼动睡眠时间缩短（但不存在快速眼动睡眠行为障碍），并随着疾病严重程度的加重而恶化。与发作性睡症患者相比，HD患者没有猝倒、睡眠幻觉或睡眠瘫痪。4例HD患者有异常低的白天睡眠潜伏期（< 8分钟），但没有一例有多次睡眠发作的REM期。结论：HD患者的睡眠表型包括失眠、睡眠阶段提前、腿部周期性运动、快速眼动睡眠行为障碍和快速眼动睡眠减少，但无发作性睡病。快速眼动睡眠减少可能先于舞蹈病。突变的亨廷顿蛋白可能对快速眼动睡眠和睡眠中的运动控制产生影响。
Background: Sleep disorders including insomnia, movements during sleep, and daytime sleepiness are common but poorly studied in Huntington disease (HD).Objective: To evaluate the HD sleep-wake phenotype (including abnormal motor activity during sleep) in patients with various HD stages and the length of CAG repeals. Because a mild hypocretin deficiency has been found in the brains of some patients with HD (hereinafter referred to as HD patients), we also tested the HD patients for narcolepsy.Design and Patients: Twenty-five HD patients (including 2 premanifest carriers) underwent clinical interview, nighttime video and sleep monitoring, and daytime multiple sleep latency tests. Their results were compared with those of patients with narcolepsy and control patients.Results: The HD patients had frequent insomnia, earlier sleep onset, lower sleep efficiency, increased stage I sleep, delayed and shortened rapid eye movement (REM) sleep, and increased periodic leg movements. Three HD patients (12%) had REM sleep behavior disorders. No sleep abnormality correlated with CAG repeat length. Reduced REM sleep duration (but not REM sleep behavior disorders) was present in premanifest carriers and patients with very mild HD and worsened with disease severity. In contrast to narcoleptic patients, HD patients had no cataplexy, hypnagogic hallucinations, or sleep paralysis. Four HD patients had abnormally low (< 8 minutes) daytime sleep latencies, but none had multiple sleep-onset REM periods.Conclusions: The sleep phenotype of HD includes insomnia, advanced sleep phase, periodic leg movements, REM sleep behavior disorders, and reduced REM sleep but not narcolepsy. Reduced REM sleep may precede chorea. Mutant huntingtin may exert an effect on REM sleep and motor control during sleep.