Primary progressive aphasia: clinicopathological correlations.

Primary progressive aphasia: clinicopathological correlations.
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DOI:
10.1038/nrneurol.2009.216
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发表时间:
2010-02
影响因子:
38.1
通讯作者:
Grossman, Murray
Grossman, Murray
中科院分区:
医学1区
文献类型:
--
作者:
Grossman, Murray

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原发性进行性失语症(PPA)是一种语言能力下降的疾病,是神经退行性疾病如额颞叶变性的常见表现。PPA有三种变体:进行性非流利性失语症、语意性痴呆和语意缺失性进行性失语症。在对神经退行性疾病进行病因特异性治疗的时代,确定PPA的组织病理学基础至关重要。PPA的临床病理相关性强调了匹克体和其他tau病变、TDP-43蛋白病变和阿尔茨海默病的痴呆的促进作用。这些数据表明,尽管PPA的许多病例与意想不到的病理有关,但PPA的特定变体与潜在病理之间存在关联。神经成像和生物流体生物标志物正在成为临床诊断的重要辅助手段。在仔细的临床检查中增加生物标志物评估,将有很大的希望能够在患者的生命中准确诊断与PPA相关的病理,并且这些发现将作为该疾病谱的临床试验的基础。
Primary progressive aphasia (PPA) is a disorder of declining language that is a frequent presentation of neurodegenerative diseases such as frontotemporal lobar degeneration. Three variants of PPA are recognized: progressive nonfluent aphasia, semantic dementia, and logopenic progressive aphasia. In an era of etiology-specific treatments for neurodegenerative conditions, determining the histopathological basis of PPA is crucial. Clinicopathological correlations in PPA emphasize the contributory role of dementia with Pick bodies and other tauopathies, TDP-43 proteinopathies, and Alzheimer disease. These data suggest an association between a specific PPA variant and an underlying pathology, although many cases of PPA are associated with an unexpected pathology. Neuroimaging and biofluid biomarkers are now emerging as important adjuncts to clinical diagnosis. There is great hope that the addition of biomarker assessments to careful clinical examination will enable accurate diagnosis of the pathology associated with PPA during a patient’s life, and that such findings will serve as the basis for clinical trials in this spectrum of disease.
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