Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy
Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy
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DOI：
10.1002/mds.1120
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发表时间：
2001-07-01
影响因子：
8.6
通讯作者：
Parisi, JE
中科院分区：
文献类型：
--
作者：
Boeve, BF;Silber, MH;Parisi, JE
我们的目的是研究快速眼动（REM）睡眠行为障碍是否在多系统萎缩（MSA）、帕金森病（PD）和路易体痴呆（DLB）中发生的频率不成比例地高，这些疾病统称为突触核蛋白病，与其他非突触核蛋白病神经退行性疾病相比。在研究1中，我们回顾了在罗切斯特梅奥诊所连续评估的398例帕金森病和/或认知障碍患者的临床记录。将疑似和多导睡眠图（PSG）证实的REM睡眠行为障碍（RBD）在突触核蛋白病MSA、PD或DLB患者中的发生频率与非突触核蛋白病阿尔茨海默病（AD）、额颞叶痴呆（FTD）、皮质基底变性（CBD）、进行性核上性麻痹（PSP）、轻度认知障碍（MCI）、原发性进行性失语（PPA）和后皮质萎缩（PCA）患者中的发生频率进行比较。在研究2中，我们回顾了在杰克逊维尔梅奥诊所连续评估的360例帕金森病和/或认知障碍患者的临床记录。PD和DLB患者与AD和MCI患者可能发生RBD的频率进行比较。在研究3中，我们回顾了23名梅奥诊所罗切斯特患者的脑活检或尸检诊断，这些患者已被临床检查为可能的RBD和神经退行性疾病。在研究1中，与非突触核蛋白病患者相比，MSA、PD或DLB患者更容易发生可能的RBD和psg确诊的RBD（可能的RBD 77/120=64% vs. 7/278=3%, p < 0.01； psg确诊的RBD 47/120=39% vs. 1/278=0%, p < 0.01）。在研究2中，PD和DLB患者比AD和MCI患者更容易发生RBD （56% vs. 2%, p < 0.01）。在研究3中，23例被质疑可能存在RBD的尸检患者中，有10例被临床诊断为RBD。10例神经病理诊断为路易体病9例，MSA 1例。在其他13例患者中，12例无RBD病史，1例有RBD病史的患者在快速眼动睡眠时的PSG和尸检PSP结果显示肌电图张力正常。这13人中只有1人有突触核蛋白病。研究1-3中RBD提示突触核蛋白病变的阳性预测值分别为91.7%、94.3%和100.0%。与其他神经退行性疾病相比，临床怀疑和psg证实的RBD在VISA、PD和DLB中发生的频率不成比例地高。在退行性痴呆和/或帕金森的情况下，我们假设RBD是一种进化的突触核蛋白病的表现。(C) 2001运动障碍学会。
Our objective was to examine whether rapid eye movement (REM) sleep behavior disorder occurs in disproportionally greater frequency in multiple system atrophy (MSA), Parkinson's disease (PD), and dementia with Lewy bodies (DLB), collectively known as the synucleinopathies, compared to other nonsynucleinopathy neurodegenerative disorders.In study 1, we reviewed the clinical records of 398 consecutive patients evaluated at Mayo Clinic Rochester for parkinsonism and/or cognitive impairment. The frequency of suspected and polysomnogram (PSG)-confirmed REM sleep behavior disorder (RBD) among subjects with the synucleinopathies MSA, PD, or DLB was compared to the frequency among subjects with the nonsynucleinopathies Alzheimer's disease (AD), frontotemporal dementia (FTD), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), mild cognitive impairment (MCI), primary progressive aphasia (PPA), and posterior cortical atrophy (PCA). In study 2, we reviewed the clinical records of 360 consecutive patients evaluated at Mayo Clinic Jacksonville for parkinsonism and/or cognitive impairment. The frequency of probable RBD among patients with PD and DLB was compared to the frequency among patients with AD and MCI. In study 3, we reviewed the brain biopsy or postmortem autopsy diagnoses of 23 Mayo Clinic Rochester patients who had been clinically examined for possible RBD and a neurodegenerative disorder.In study 1, patients with MSA, PD, or DLB were more likely to have probable and PSG-confirmed RBD compared to subjects with the nonsynucleinopathies (probable RBD 77/120=64% vs. 7/278=3%, p < 0.01; PSG-confirmed RBD 47/120=39% vs. 1/278=0%, p < 0.01). In study 2, patients with PD and DLB were more likely to have probable RBD compared to those with AD and MCI (56% vs. 2%, p < 0.01). In study 3, of the 23 autopsied patients who had been questioned about possible RBD, 10 were clinically diagnosed with RBD. The neuropathologic diagnoses in these 10 included Lewy body disease in nine, and MSA in one. Of the other 13 cases, 12 did not have a history suggesting RBD, and the one case who did had normal electromyographic atonia during REM sleep on PSG and autopsy findings of PSP. Only one of these 13 had a synucleinopathy. The positive predictive values for RBD indicating a synucleinopathy for studies 1-3 were 91.7%, 94.3%, and 100.0%, respectively.Clinically suspected and PSG-proven RBD occurs with disproportionally greater frequency in VISA, PD, and DLB compared to other neurodegenerative disorders. In the setting of degenerative dementia and/or parkinsonism, we hypothesize that RBD is a manifestation of an evolving synucleinopathy. (C) 2001 Movement Disorder Society.