REM sleep behavior disorder and degenerative dementia - An association likely reflecting Lewy body disease

REM sleep behavior disorder and degenerative dementia - An association likely reflecting Lewy body disease
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DOI:
10.1212/wnl.51.2.363
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发表时间:
1998-08-01
期刊:
影响因子:
9.9
通讯作者:
Petersen, RC
Petersen, RC
中科院分区:
医学1区
文献类型:
--
作者:
Boeve, BF;Silber, MH;Petersen, RC

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背景资料:REM睡眠行为障碍(RBD)已被报道与各种神经退行性疾病,最常见的是路易体病理学疾病。RBD通常先于PD发作,最近的一项前瞻性研究表明,38%的RBD患者最终发展为PD。研究方法:我们确定了37例退行性痴呆患者,他们有睡眠期间手臂和腿部剧烈运动爆发的病史,伴有发声,并与梦回忆有关。比较了有和没有两个或两个以上帕金森综合征体征的患者。临床,实验室和神经心理特征进行了分析,并与路易体痴呆(DLB)的临床诊断标准适用于所有患者。结果:37例患者中有34例为男性,RBD的平均发病年龄为61.5岁,认知功能下降的平均发病年龄为68.1岁。除两名患者外,所有患者的RBD均在痴呆之前或同时开始。帕金森综合征(两个或两个以上的迹象)发生在54%的样本(20/37),平均发病年龄为69.1岁。多导睡眠图(PSG)证实了所有研究患者的RBD。神经心理学测试表明,受损的感知组织能力,语言流畅性:和显着的结构性运动障碍,超过一半的患者。有和没有帕金森综合征的患者之间的临床特征或神经心理学表现的频率没有统计学上的显着差异。34例患者(92%)符合临床可能或很可能DLB的标准。三名患者进行了尸检,所有的边缘带或不带新皮质路易体。结论:我们报告了一组主要为男性的RBD和退行性痴呆的特征性关联患者。与对照组患者的临床和神经心理测量学特征。没有帕金森症的人是相似的。我们假设这些患者的潜在病理是DLB。
Background: REM sleep behavior disorder (RBD) has been reported with various neurodegenerative disorders, most frequently in disorders with Lewy body pathology. RBD often precedes the onset of PD, and a recent prospective study showed that 38% of patients with RBD eventually developed PD. Methods: We identified 37 patients with degenerative dementia and a history of bursts of vigorous movement of the arms and legs with vocalization during sleep and associated with dream recall. Patients with and without two or more signs of parkinsonism were compared. Clinical, laboratory, and neuropsychometric features were analyzed, and criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) were applied to all patients. Results: Thirty-four of the 37 patients were male with mean age at onset of 61.5 years for RBD and 68.1 years for cognitive decline. RBD commenced before or concurrently with dementia in all patients but two. Parkinsonism (two or more signs) occurred in 54% of the sample (20/37), with a mean age at onset of 69.1 years. Polysomnography (PSG) confirmed RBD in all patients studied. Neuropsychological testing demonstrated impaired perceptual-organizational skills, verbal fluency: and marked constructional dyspraxia in more than one-half the patients. There were no statistically significant differences in the frequency of clinical features or in neuropsychological performance between patients with and without parkinsonism. Thirty-four patients (92%) met criteria for clinically possible or probable DLB. Three patients were autopsied; all had limbic with or without neocortical Lewy bodies. Conclusions: We report a group of predominantly male patients with a characteristic association of RBD and degenerative dementia. The clinical and neuropsychometric features of the groups of patients with and. without parkinsonism are similar. We hypothesize that the underlying pathology in these patients is DLB.