Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia
Corticobasal degeneration and its relationship to progressive supranuclear palsy and frontotemporal dementia
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DOI：
10.1002/ana.10570
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发表时间：
2003-01-01
影响因子：
11.2
通讯作者：
Litvan, I
中科院分区：
文献类型：
--
作者：
Boeve, BF;Lang, AE;Litvan, I
描述皮质基底性变性(CBD)的核心临床特征及其与进行性核上性瘫痪(PSP)和额颞部痴呆(FTD)的关系。本文详细描述了皮质基综合征的一系列临床特征。这些症状包括渐进性不对称强直和失用症；异肢现象；皮质感觉丧失；肌阵挛；镜像运动；肌张力障碍；震颤；L多巴反应缺乏；痴呆；神经精神特征；眼运动和失用症。本文综述了皮质-基底综合征的实验室表现，重点介绍了神经心理学、电生理、结构神经影像和功能神经影像的表现。随后，文章讨论了CBD和PSP的相似性，认为它们可能是同一病理生理过程的变异体，但指出CBD和PSP在临床表现和病理特征上的差异值得进一步研究。关于CBD和PSP是否是同一病理生理过程的变体的争论仍在继续，需要进一步澄清。对具有CBS临床特征和/或CBD病理特征的罕见家族的其他研究，无论是否与tau突变相关，都可能提供关键的见解。(PulcINFO数据库记录(C)2016 APA，版权所有)
Describes the core clinical features of Corticobasal Degeneration (CBD) and its relationships to progressive supranuclear palsy (PSP) and frontotemporal dementia (FTD). The article describes in detail the constellation of clinical features, termed as Corticobasal Syndrome. These are progressive asymmetrical rigidity and apraxia; alien limb phenomenon; cortical sensory loss; myoclonus; mirror movements; dystonia; tremor; lack of L-dopa response; dementia; neuropsychiatric features; ocular motor and apraxia. The article reviews the laboratory findings in the corticobasal syndrome, focusing on the neuropsychological, electrophysiological, structural neuroimaging and functional neuroimaging findings. Later, the article discusses the similarities between CBD and PSP which suggest that they may be the variants of the same pathophysiological process but points out that the differences in clinical presentations and pathological features of CBD and PSP warrant further study. The debate continues about whether CBD and PSP are variants of the same pathophyiological process and needs further clarification. Additional studies on the rare kindreds with the clinical features of the CBS and/or pathological features of CBD, whether associated with mutations in tau or not, may offer key insights.(PsycINFO Database Record (c) 2016 APA, all rights reserved)