Department of Neurology, 2 Gibson, Hospital of the University of Pennsylvania, Philadelphia, PA 19104-4283, USA.
Nat Rev Neurol. 2010 Feb;6(2):88-97. doi: 10.1038/nrneurol.2009.216.
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.
原发性进行性失语症（PPA）是一种语言逐渐衰退的疾病，是额颞叶变性等神经退行性疾病的常见表现。目前已识别出 PPA 的三种变体：进行性非流利性失语症、语义性痴呆和失语法性进行性失语症。在针对神经退行性疾病的病因特异性治疗时代，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 Pick 体痴呆和其他tau 病、TDP-43 蛋白病以及阿尔茨海默病的促成作用。这些数据表明，特定的 PPA 变体与潜在的病理之间存在关联，尽管许多 PPA 病例与意外的病理有关。神经影像学和生物流体生物标志物现在已成为临床诊断的重要辅助手段。人们非常希望将生物标志物评估添加到仔细的临床检查中，以便在患者的一生中能够准确诊断与 PPA 相关的病理，并使这些发现成为该疾病谱中临床试验的基础。
