Neuropathology of Lewy body disease: Clinicopathological crosstalk between typical and atypical cases
Neuropathology of Lewy body disease: Clinicopathological crosstalk between typical and atypical cases
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DOI：
10.1111/neup.12597
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发表时间：
2019-09-09
期刊：
影响因子：
2.3
通讯作者：
Wakabayashi, Koichi
中科院分区：
文献类型：
--
作者：
Kon, Tomoya;Tomiyama, Masahiko;Wakabayashi, Koichi
路易体病（Lewy body disease，LBD）的特征在于存在路易体（Lewy bodies，LB）和路易神经突，并且包括诊断谱，其包括帕金森病（Parkinson's disease，PD）、PD伴痴呆和痴呆伴LB。LB和Lewy神经突是主要由磷酸化的α-突触核蛋白组成的不溶性聚集体，并且可以广泛分布在整个中枢和外周神经系统中。LB的分布可能决定LBD的表型。Braak假设路易氏病从周围神经系统到嗅球和脑干，然后到其他脑区，呈上升趋势。Braak的PD分期表明LBD是一种朊病毒样疾病。大多数典型的PD病例符合Braak的PD分期，但该方案在某些情况下失败。阿尔茨海默病、进行性核上性麻痹、皮质基底综合征、多系统萎缩、额颞叶变性、克雅氏病、脑血管疾病和特发性震颤是病理证实的LBD的常见误诊。LBD在临床和病理环境中表现出相当大的异质性，这使得临床诊断具有挑战性。
Lewy body disease (LBD) is characterized by the presence of Lewy bodies (LBs) and Lewy neurites and comprises a diagnostic spectrum that includes Parkinson's disease (PD), PD with dementia, and dementia with LBs. LBs and Lewy neurites are insoluble aggregates composed mainly of phosphorylated alpha-synuclein and can be widely distributed throughout the central and peripheral nervous systems. The distribution of LBs may determine the LBD phenotype. Braak hypothesized that Lewy pathology progresses ascendingly from the peripheral nervous system to the olfactory bulbs and brainstem and then to other brain regions. Braak's PD staging suggests that LBD is a prion-like disease. Most typical PD cases fit with Braak's PD staging, but the scheme fails in some cases. Alzheimer's disease, progressive supranuclear palsy, corticobasal syndrome, multiple system atrophy, frontotemporal lobar degeneration, Creutzfeldt-Jakob disease, cerebrovascular diseases, and essential tremor are common misdiagnoses for pathologically confirmed LBD. LBD exhibits considerable heterogeneity in both clinical and pathological settings, which makes clinical diagnosis challenging.