Rogalski E J, Mesulam M M
Cognitive Neurology and Alzheimers Disease Center, Northwestern University Feinberg School of Medicine, 320 E. Superior Street, Searle 11-579, Chicago, IL 60611, USA.
Curr Alzheimer Res. 2009 Aug;6(4):331-6. doi: 10.2174/156720509788929264.
Primary Progressive Aphasia (PPA) is a neurodegenerative syndrome characterized by a gradual dissolution of language, but relative sparing of other cognitive domains during the initial stages of the disease. Research has led to substantial progress in understanding the clinical characteristics, genetics, and neuropathology of this syndrome. This article reviews the clinical criteria for diagnosing PPA, discusses the utility of defining the mild cognitive impairment (MCI) stage of PPA, and highlights some of the more recent research advances particularly in the area of pathology and genetics.
原发性进行性失语(PPA)是一种神经退行性综合征,其特征是语言功能逐渐衰退,但在疾病初期其他认知领域相对保留。研究在理解该综合征的临床特征、遗传学和神经病理学方面取得了重大进展。本文回顾了诊断PPA的临床标准,讨论了定义PPA轻度认知障碍(MCI)阶段的实用性,并重点介绍了一些最新的研究进展,特别是在病理学和遗传学领域。