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快速眼动睡眠行为障碍与神经退行性疾病。

Rapid Eye Movement Sleep Behavior Disorder and Neurodegenerative Disease.

机构信息

Department of Neurology, University of Minnesota, Minneapolis.

Department of Psychiatry, University of Minnesota, Minneapolis.

出版信息

JAMA Neurol. 2015 Jun;72(6):707-12. doi: 10.1001/jamaneurol.2014.4563.

DOI:10.1001/jamaneurol.2014.4563
PMID:25867792
Abstract

IMPORTANCE

The dream enactment of rapid eye movement sleep behavior disorder (RBD) is often the first indication of an impending α-synuclein disorder, such as Parkinson disease, multiple-system atrophy, or dementia with Lewy bodies.

OBJECTIVE

To provide an overview of RBD from the onset of dream enactment through the emergence of a parkinsonian disorder.

EVIDENCE REVIEW

Peer-reviewed articles, including case reports, case series, retrospective reviews, prospective randomized trials, and basic science investigations, were identified in a PubMed search of articles on RBD from January 1, 1986, through July 31, 2014.

FINDINGS

Under normal conditions, vivid dream mentation combined with skeletal muscle paralysis characterizes rapid eye movement sleep. In RBD, α-synuclein abnormalities in the brainstem disinhibit rapid eye movement sleep motor activity, leading to dream enactment. The behaviors of RBD are often theatrical, with complexity, aggression, and violence; fighting and fleeing actions can be injurious to patients as well as bed partners. Rapid eye movement sleep behavior disorder is distinguished from other parasomnias by clinical features and the demonstration of rapid eye movement sleep without atonia on polysomnography. Consistent with early neurodegeneration, patients with RBD demonstrate subtle motor, cognitive, and autonomic impairments. Approximately 50% of patients with spontaneous RBD will convert to a parkinsonian disorder within a decade. Ultimately, nearly all (81%-90%) patients with RBD develop a neurodegenerative disorder. Among patients with Parkinson disease, RBD predicts a non-tremor-predominant subtype, gait freezing, and an aggressive clinical course. The most commonly cited RBD treatments include low-dose clonazepam or high-dose melatonin taken orally at bedtime.

CONCLUSIONS AND RELEVANCE

Treatment of RBD can prevent injury to patients and bed partners. Because RBD is a prodromal syndrome of Parkinson disease (or related disorder), it represents a unique opportunity for developing and testing disease-modifying therapies.

摘要

重要性

快速眼动睡眠行为障碍 (RBD) 的梦境发作通常是即将发生的α-突触核蛋白紊乱的第一个迹象,如帕金森病、多系统萎缩或路易体痴呆。

目的

提供从梦境发作到出现帕金森病的 RBD 概述。

证据回顾

在 PubMed 上搜索了 1986 年 1 月 1 日至 2014 年 7 月 31 日发表的有关 RBD 的文章,确定了同行评议的文章,包括病例报告、病例系列、回顾性综述、前瞻性随机试验和基础科学研究。

发现

在正常情况下,生动的梦境伴随着骨骼肌瘫痪,构成了快速眼动睡眠。在 RBD 中,脑干中的α-突触核蛋白异常使快速眼动睡眠运动活动失去抑制,导致梦境发作。RBD 的行为通常具有戏剧性、复杂性、攻击性和暴力性;战斗和逃跑动作会对患者和床伴造成伤害。快速眼动睡眠行为障碍通过临床特征和多导睡眠图显示快速眼动睡眠无弛缓来与其他睡眠障碍区分开来。与早期神经退行性变一致,RBD 患者表现出轻微的运动、认知和自主神经损伤。大约 50%的自发性 RBD 患者将在 10 年内转化为帕金森病。最终,几乎所有(81%-90%)的 RBD 患者都会发展为神经退行性疾病。在帕金森病患者中,RBD 预测非震颤为主型、步态冻结和侵袭性临床病程。最常引用的 RBD 治疗方法包括低剂量氯硝西泮或高剂量褪黑素口服睡前服用。

结论和相关性

治疗 RBD 可以防止患者和床伴受伤。由于 RBD 是帕金森病(或相关疾病)的前驱综合征,因此它为开发和测试疾病修饰疗法提供了独特的机会。

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