Neuropathology of variants of progressive supranuclear palsy
Neuropathology of variants of progressive supranuclear palsy
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DOI：
10.1097/wco.0b013e32833be924
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发表时间：
2010-08-01
影响因子：
4.8
通讯作者：
Josephs, Keith A.
中科院分区：
文献类型：
--
作者：
Dickson, Dennis W.;Ahmed, Zeshan;Josephs, Keith A.
神经退行性tau病变，其中进展性核上性麻痹（PSP）是最常见的一种，在临床上具有异质性，反映了tau病理分布和生化组成的差异。这篇综述强调了PSP及其变体的临床和病理表现。进行性核上性麻痹是一种4R脑病，在基底节区、间脑、脑干和小脑的神经解剖学特异性核中发生神经元和神经胶质免疫反应性病变，新皮层受累受限。分级聚类分析的临床和病理特征表明，PSP有明显的临床病理变异。在表现为局灶性皮质综合征的PSP变体中，如额颞叶痴呆、皮质基底综合征和言语失用症，比典型的PSP有更大的皮质病理。在表现为左旋多巴反应性帕金森病以及纯运动障碍和步态衰竭的PSP变体中，与典型的PSP相比，主要核（苍白球、丘脑底核和黑质）的皮质病理更少，退化更严重。PSP的临床变异反映了tau病理的不同解剖分布，但它们与典型PSP具有相同的组织病理、生化和遗传特征。PSP及其变异的解剖学选择性易感性的基础仍有待确定。
Purpose of reviewNeurodegenerative tauopathies, of which progressive supranuclear palsy (PSP) is one of the most common, are clinically heterogeneous, reflecting differences in distribution and biochemical composition of tau pathology. This review highlights the range of clinical and pathologic presentations of PSP and its variants.Recent findingsProgressive supranuclear palsy is a 4R tauopathy with neuronal and glial tau-immunoreactive lesions in neuroanatomically specific nuclei in the basal ganglia, diencephalon, brainstem and cerebellum, with restricted involvement of the neocortex. Hierarchical cluster analyses of clinical and pathologic features of PSP indicate that there are distinct clinicopathologic variants of PSP. In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP. In variants of PSP presenting with levodopa-responsive Parkinsonism, as well as pure akinesia and gait failure, there is less cortical pathology and more severe degeneration in the cardinal nuclei - globus pallidus, subthalamic nucleus and substantia nigra - than in typical PSP.SummaryClinical variants in PSP reflect varying anatomical distribution of tau pathology, but they share histopathologic, biochemical and genetic features with typical PSP. The basis for anatomical selective vulnerability in PSP and its variants remains to be determined.