Institute of Clinical Neurobiology, Alberichgasse 5/13, 1150, Vienna, Austria.
J Neural Transm (Vienna). 2021 May;128(5):687-699. doi: 10.1007/s00702-021-02345-9. Epub 2021 Apr 29.
Lewy body dementia (LBD) and Parkinson's disease-dementia (PDD) are two major neurocognitive disorders with Lewy bodies (LB) of unknown etiology. There is considerable clinical and pathological overlap between these two conditions that are clinically distinguished based on the duration of Parkinsonism prior to development of dementia. Their morphology is characterized by a variable combination of LB and Alzheimer's disease (AD) pathologies. Cerebral amyloid angiopathy (CAA), very common in aged persons and particularly in AD, is increasingly recognized for its association with both pathologies and dementia. To investigate neuropathological differences between LB diseases with and without dementia, 110 PDD and 60 LBD cases were compared with 60 Parkinson's disease (PD) cases without dementia (PDND). The major demographic and neuropathological data were assessed retrospectively. PDD patients were significantly older than PDND ones (83.9 vs 77.8 years; p < 0.05); the age of LB patients was in between both groups (mean 80.2 years), while the duration of disease was LBD < PDD < PDND (mean 6.7 vs 12.5 and 14.3 years). LBD patients had higher neuritic Braak stages (mean 5.1 vs 4.5 and 4.0, respectively), LB scores (mean 5.3 vs 4.2 and 4.0, respectively), and Thal amyloid phases (mean 4.1 vs 3.0 and 2.3, respectively) than the two other groups. CAA was more common in LBD than in the PDD and PDND groups (93 vs 50 and 21.7%, respectively). Its severity was significantly greater in LBD than in PDD and PDND (p < 0.01), involving mainly the occipital lobes. Moreover, striatal Aβ deposition highly differentiated LBD brains from PDD. Braak neurofibrillary tangle (NFT) stages, CAA, and less Thal Aβ phases were positively correlated with LB pathology (p < 0.05), which was significantly higher in LBD than in PDD < PDND. Survival analysis showed worse prognosis in LBD than in PDD (and PDND), which was linked to both increased Braak tau stages and more severe CAA. These and other recent studies imply the association of CAA-and both tau and LB pathologies-with cognitive decline and more rapid disease progression that distinguishes LBD from PDD (and PDND).
路易体痴呆（LBD）和帕金森病痴呆（PDD）是两种主要的神经认知障碍，其路易体（LB）的病因不明。这两种疾病在临床上有很大的重叠，根据痴呆发生前帕金森病的持续时间来区分。它们的形态学特征是 LB 和阿尔茨海默病（AD）病理学的可变组合。脑淀粉样血管病（CAA）在老年人中非常常见，特别是在 AD 中，其与这两种病理学和痴呆的关系越来越受到重视。为了研究 LB 疾病伴或不伴痴呆的神经病理学差异，比较了 110 例 PDD 和 60 例 LBD 病例与 60 例无痴呆的帕金森病（PDND）病例。回顾性评估主要的人口统计学和神经病理学数据。PDD 患者明显比 PDND 患者年龄大（83.9 岁比 77.8 岁；p<0.05）；LB 患者的年龄介于两组之间（平均 80.2 岁），而疾病的持续时间为 LBD<PDD<PDND（平均 6.7 年比 12.5 年和 14.3 年）。LBD 患者的神经纤维缠结 Braak 分期（平均 5.1 级比 4.5 级和 4.0 级）、LB 评分（平均 5.3 级比 4.2 级和 4.0 级）和 Thal 淀粉样蛋白阶段（平均 4.1 级比 3.0 级和 2.3 级）均高于其他两组。与 PDD 和 PDND 组相比，LBD 组 CAA 更常见（93%比 50%和 21.7%）。LBD 组的 CAA 严重程度显著高于 PDD 和 PDND 组（p<0.01），主要涉及枕叶。此外，纹状体 Aβ 沉积高度区分了 LBD 大脑和 PDD。Braak 神经原纤维缠结（NFT）分期、CAA 和较少的 Thal Aβ 阶段与 LB 病理学呈正相关（p<0.05），LBD 明显高于 PDD<PDND。生存分析显示 LBD 的预后比 PDD 差（和 PDND），这与 Braak tau 分期的增加和更严重的 CAA 有关。这些和其他最近的研究表明，CAA 以及 tau 和 LB 病理学与认知能力下降和疾病进展更快有关，这将 LBD 与 PDD（和 PDND）区分开来。
