Fernández-Arcos Ana, Iranzo Alex, Serradell Mónica, Gaig Carles, Santamaria Joan
Neurology Service, Multidisciplinary Sleep Disorders Unit, Hospital Clinic de Barcelona, University of Barcelona Medical School, IDIBAPS, CIBERNED, Barcelona, Spain.
Sleep. 2016 Jan 1;39(1):121-32. doi: 10.5665/sleep.5332.
To describe the clinical phenotype of idiopathic rapid eye movement (REM) sleep behavior disorder (IRBD) at presentation in a sleep center.
Clinical history review of 203 consecutive patients with IRBD identified between 1990 and 2014. IRBD was diagnosed by clinical history plus video-polysomnographic demonstration of REM sleep with increased electromyographic activity linked to abnormal behaviors.
Patients were 80% men with median age at IRBD diagnosis of 68 y (range, 50-85 y). In addition to the already known clinical picture of IRBD, other important features were apparent: 44% of the patients were not aware of their dream-enactment behaviors and 70% reported good sleep quality. In most of these cases bed partners were essential to convince patients to seek medical help. In 11% IRBD was elicited only after specific questioning when patients consulted for other reasons. Seven percent did not recall unpleasant dreams. Leaving the bed occurred occasionally in 24% of subjects in whom dementia with Lewy bodies often developed eventually. For the correct diagnosis of IRBD, video-polysomnography had to be repeated in 16% because of insufficient REM sleep or electromyographic artifacts from coexistent apneas. Some subjects with comorbid obstructive sleep apnea reported partial improvement of RBD symptoms following continuous positive airway pressure therapy. Lack of therapy with clonazepam resulted in an increased risk of sleep related injuries. Synucleinopathy was frequently diagnosed, even in patients with mild severity or uncommon IRBD presentations (e.g., patients who reported sleeping well, onset triggered by a life event, nocturnal ambulation) indicating that the development of a neurodegenerative disease is independent of the clinical presentation of IRBD.
We report the largest IRBD cohort observed in a single center to date and highlight frequent features that were not reported or not sufficiently emphasized in previous publications. Physicians should be aware of the full clinical expression of IRBD, a sleep disturbance that represents a neurodegenerative disease.
A commentary on this article appears in this issue on page 7.
描述睡眠中心接诊时特发性快速眼动（REM）睡眠行为障碍（IRBD）的临床表型。
回顾1990年至2014年间连续收治的203例IRBD患者的临床病史。IRBD通过临床病史以及视频多导睡眠图显示REM睡眠期肌电图活动增加并伴有异常行为来诊断。
患者中80%为男性，IRBD诊断时的中位年龄为68岁（范围50 - 85岁）。除了IRBD已知的临床表现外，其他重要特征也很明显：44%的患者未意识到自己的梦呓行为，70%报告睡眠质量良好。在大多数这些病例中，床伴对于说服患者寻求医疗帮助至关重要。11%的患者仅在因其他原因就诊时经特定询问才引出IRBD。7%的患者不记得有不愉快的梦境。24%的患者偶尔会离床，这些患者最终常发展为路易体痴呆。由于REM睡眠不足或并存呼吸暂停导致的肌电图伪差，16%的患者需要重复进行视频多导睡眠图以正确诊断IRBD。一些合并阻塞性睡眠呼吸暂停的患者报告持续气道正压通气治疗后RBD症状部分改善。未使用氯硝西泮治疗会增加睡眠相关损伤的风险。即使在病情轻度或IRBD表现不常见的患者（如报告睡眠良好、由生活事件触发起病、夜间行走的患者）中也经常诊断出突触核蛋白病，这表明神经退行性疾病的发生与IRBD的临床表现无关。
我们报告了迄今为止在单一中心观察到的最大IRBD队列，并强调了先前出版物中未报告或未充分强调的常见特征。医生应了解IRBD的完整临床症状，这是一种代表神经退行性疾病的睡眠障碍。
关于本文的述评见本期第7页。
