Neuropathology of Parkinson disease.

Neuropathology of Parkinson disease.
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DOI:
10.1016/j.parkreldis.2017.07.033
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发表时间:
2018-01
影响因子:
4.1
通讯作者:
Dickson DW
Dickson DW
中科院分区:
医学2区
文献类型:
--
作者:
Dickson DW

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帕金森病(Parkinson's disease,PD)是一种以运动迟缓、僵硬、姿势不稳和震颤为特征的疾病。几种病理过程可以产生这种综合征,但神经变性伴随着由α-突触核蛋白(路易体)组成的神经元包涵体被认为是PD的典型病理相关性。根据个人经验和文献回顾,PD的神经病理学特征进行了综述。从细胞生物学和动物实验两方面总结了PD的分子病理学。与PD的体征和症状相关的病理特征是黑质中的神经元损失和纹状体的多巴胺能去神经支配。黑质神经元变性优先影响腹外侧细胞群,该细胞群投射到后外侧壳核,并伴随由聚集的α-突触核蛋白组成的路易体的形成。部分PD患者在尸检时发现有其他病理过程,如多系统萎缩、进行性核上性麻痹和脑血管疾病(血管性帕金森综合征)。周围自主神经系统也受到影响。PD的触发事件尚不清楚,但最近的研究表明核膜完整性丧失的作用。一旦α-突触核蛋白聚集体形成,证据支持细胞到细胞的繁殖。PD是一种多系统突触核蛋白病,由遗传和环境因素引起,在选择性脆弱的神经元群体中产生变性。
Parkinson’s disease (PD) is characterized by bradykinesia, rigidity, postural instability and tremor. Several pathologic processes can produce this syndrome, but neurodegeneration accompanied by neuronal inclusions composed of α-synuclein (Lewy bodies) is considered the typical pathologic correlate of PD. The neuropathologic features of PD are reviewed based upon personal experience and review of the literature. Molecular pathology of PD is summarized from cell biological and animal studies. The pathologic feature that correlates with signs and symptoms of PD is neuronal loss in the substantia nigra with dopaminergic denervation of the striatum. Neuronal degeneration in the substantia nigra preferentially affects the ventrolateral cell group that projects to posterolateral putamen and is accompanied by formation of Lewy bodies composed of aggregated α-synuclein. Some patients with PD are found at autopsy to have other pathologic processes, such as multiple system atrophy, progressive supranuclear palsy and cerebrovascular disease (vascular Parkinsonism). The peripheral autonomic nervous system is also affected. The triggering event in PD is unknown, but recent studies suggest a role for loss of nuclear membrane integrity. Once α-synuclein aggregates forms, evidence supports cell-to-cell propagation. PD is a multisystem synucleinopathy caused by poorly characterized genetic and environmental factors that produces degeneration in selectively vulnerable neuronal populations.
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