Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism
Synucleinopathy pathology and REM sleep behavior disorder plus dementia or parkinsonism
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DOI：
10.1212/01.wnl.0000073619.94467.b0
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发表时间：
2003-07-08
期刊：
影响因子：
9.9
通讯作者：
Mahowald, MW
中科院分区：
文献类型：
--
作者：
Boeve, B;Silber, MH;Mahowald, MW
目的：确定突触核蛋白病病理学是否与快速眼动睡眠行为障碍 (RBD) 加上痴呆或帕金森病相关。方法：对 1990 年 1 月至 2002 年 4 月在罗切斯特梅奥诊所评估的所有被诊断患有 RBD 和神经退行性疾病的尸检病例的临床和神经病理学结果进行分析。所有病例均使用泛素和/或α-突触核蛋白免疫细胞化学。临床和神经病理学诊断基于已发布的标准。结果：发现 15 例病例（14 名男性）。所有人都有明确的梦境表现行为史，其中 10 人经多导睡眠图证实患有 RBD。 10 名 (66.7%) 患者出现 RBD 的时间中位数为 10 年（范围为 2 至 29 年）。临床诊断包括路易体痴呆 (DLB) (n = 6)；多系统萎缩（MSA）（n = 2）；合并 DLB、AD 和血管性痴呆 (n = 1)；痴呆症（n = 1）；帕金森病痴呆（n = 1）； PD（n = 1）；患有痴呆症的 PD (n = 1)；痴呆/帕金森病/运动神经元疾病 (n = 1)；和 AD/宾斯旺格病 (n = 1)。神经病理学诊断为 12 例路易体病 (LBD)（11 例新皮质病，1 例边缘系统病）和 3 例 MSA。3 例还患有嗜银颗粒病理。在 LBD 病例中，有 6 例伴有 AD 病理（其中 1 例还伴有 Binswanger 病理，1 例还伴有多发皮质下梗塞）。结论：在退行性痴呆或帕金森病的情况下，RBD 通常反映潜在的突触核蛋白病。
Objective: To determine if synucleinopathy pathology is related to REM sleep behavior disorder (RBD) plus dementia or parkinsonism. Methods: The clinical and neuropathologic findings were analyzed on all autopsied cases evaluated at Mayo Clinic Rochester from January 1990 to April 2002 who were diagnosed with RBD and a neurodegenerative disorder. Ubiquitin and/or alpha-synuclein immunocytochemistry was used in all cases. The clinical and neuropathologic diagnoses were based on published criteria. Results: Fifteen cases were identified (14 men). All had clear histories of dream enactment behavior, and 10 had RBD confirmed by polysomnography. RBD preceded dementia or parkinsonism in 10 (66.7%) patients by a median of 10 (range 2 to 29) years. The clinical diagnoses included dementia with Lewy bodies (DLB) (n = 6); multiple-system atrophy (MSA) (n = 2); combined DLB, AD, and vascular dementia (n = 1); dementia (n = 1); dementia with parkinsonism (n = 1); PD (n = 1); PD with dementia (n = 1); dementia/parkinsonism/motor neuron disease (n = 1); and AD/Binswanger's disease (n = 1). The neuropathologic diagnoses were Lewy body disease (LBD) in 12 (neocortical in 11 and limbic in 1) and MSA in 3. Three also had argyrophilic grain pathology. In the LBD cases, concomitant AD pathology was present in six (one also with Binswanger's pathology, and one also with multiple subcortical infarcts). Conclusion: In the setting of degenerative dementia or parkinsonism, RBD often reflects an underlying synucleinopathy.