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皮质基底节综合征-阿尔茨海默病之谜。

The corticobasal syndrome-Alzheimer's disease conundrum.

机构信息

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

出版信息

Expert Rev Neurother. 2011 Nov;11(11):1569-78. doi: 10.1586/ern.11.153.

Abstract

Corticobasal syndrome (CBS), once thought to be pathognomonic for corticobasal degeneration pathology, is increasingly reported with various underlying pathologies. Alzheimer's disease is one such pathology, also once believed to be unique for its clinical syndrome of dementia of the Alzheimer's type. CBS is believed to result from topography of asymmetric parietofrontal cortical lesion involvement, rather than lesion subtype. However, this topographical pattern is strikingly different to that typically associated with AD for unclear reasons. This article will focus on CBS with underlying AD pathology (CBS-AD), and will review associated clinical, imaging and demographic factors. Predicting AD pathology is of marked interest as disease-modifying therapies loom on the horizon, with biomarkers and imaging research underway. By reviewing the literature for CBS-AD case reports and series and contrasting them with CBS with underlying corticobasal degeneration pathology cases, the article aims to examine factors that may predict AD pathology. How AD pathology may produce this clinical phenotype, rather than the prototype dementia of the Alzheimer's type, will also be reviewed.

摘要

皮质基底节综合征(CBS),曾被认为是皮质基底节变性病理学的特征性疾病,现在越来越多地与各种潜在的病理学相关报道。阿尔茨海默病就是这样一种病理学,也曾被认为是其特有的阿尔茨海默病型痴呆的临床综合征。CBS 被认为是由于不对称顶额叶皮质病变受累的拓扑结构,而不是病变亚型引起的。然而,由于不明原因,这种拓扑模式与通常与 AD 相关的模式明显不同。本文将重点介绍 AD 病理学(CBS-AD)基础的 CBS,并将回顾相关的临床、影像学和人口统计学因素。由于疾病修饰疗法即将出现,生物标志物和影像学研究正在进行,预测 AD 病理学具有显著的意义。通过回顾 CBS-AD 病例报告和系列,并将其与 CBS-AD 基础的皮质基底节变性病理学病例进行对比,本文旨在研究可能预测 AD 病理学的因素。AD 病理学如何产生这种临床表型,而不是典型的阿尔茨海默病型痴呆,也将进行回顾。

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