Neuropathology of Parkinson's Disease and Parkinsonism.

IF 7.8 2区 医学 Q1 MEDICINE, RESEARCH & EXPERIMENTAL
Dennis W Dickson
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Abstract

Parkinsonism, the clinical term for a disorder with prominent bradykinesia and variably associated extrapyramidal signs and symptoms, is virtually always accompanied by degeneration of the nigrostriatal dopaminergic system, with neuronal loss and gliosis in the substantia nigra at autopsy. Neuronal loss is particularly marked in the ventrolateral cell groups of the substantia nigra, which project to the putamen via the nigrostriatal pathway. Parkinsonism is pathologically heterogeneous, with the most common pathologic substrates related to abnormalities in the presynaptic protein α-synuclein or the microtubule-binding protein tau. In idiopathic Parkinson's disease (PD), α-synuclein accumulates in neuronal perikarya (Lewy bodies) and neuronal processes (Lewy neurites). The disease process is multifocal and involves select central nervous system neurons, as well as neurons in the peripheral autonomic nervous system. The particular set of neurons affected determines nonmotor clinical presentations. Multiple system atrophy (MSA) is the other major α-synucleinopathy. It is also associated with autonomic dysfunction and in some cases with cerebellar signs. The hallmark histopathologic feature of MSA is an accumulation of α-synuclein within glial cytoplasmic inclusions (GCIs). The most common of the Parkinsonian tauopathies is progressive supranuclear palsy (PSP), which is clinically associated with severe postural instability leading to early falls. The tau pathology of PSP also affects both neurons and glia.

帕金森病和帕金森症的神经病理学。
帕金森病是一种以显著运动迟缓和锥体外系不同相关体征和症状为特征的疾病的临床术语,尸检时几乎总是伴有黑质纹状体多巴胺能系统的变性,并伴有神经元丢失和黑质胶质增生。神经元损失在黑质腹侧细胞群中尤为明显,这些细胞群通过黑质纹状体途径投射到壳核。帕金森病在病理上是异质性的,最常见的病理底物与突触前蛋白α-突触核蛋白或微管结合蛋白tau的异常有关。在特发性帕金森病(PD)中,α-突触核蛋白在神经元核周(路易小体)和神经元突(路易神经突)中积累。疾病过程是多灶性的,涉及中枢神经系统神经元和外周自主神经系统的神经元。受影响的特定神经元组决定了非运动性临床表现。多系统萎缩(MSA)是另一种主要的α-突触核蛋白病。它也与自主神经功能障碍有关,在某些情况下伴有小脑体征。MSA的标志性组织病理学特征是α-突触核蛋白在胶质细胞质包涵体(gci)内的积累。最常见的帕金森病是进行性核上性麻痹(PSP),它在临床上与严重的姿势不稳定相关,导致早期跌倒。PSP的tau病理学也影响神经元和神经胶质。
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来源期刊
Cold Spring Harbor perspectives in medicine
Cold Spring Harbor perspectives in medicine MEDICINE, RESEARCH & EXPERIMENTAL-
CiteScore
14.30
自引率
1.90%
发文量
44
审稿时长
4-8 weeks
期刊介绍: Cold Spring Harbor Perspectives in Medicine is a monthly online publication comprising reviews on different aspects of a variety of diseases, covering everything from the molecular and cellular bases of disease to translational medicine and new therapeutic strategies. Cold Spring Harbor Perspectives in Medicine is thus unmatched in its depth of coverage and represents an essential source where readers can find informed surveys and critical discussion of advances in molecular medicine.
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