Macoir Joël, Lavoie Monica, Laforce Robert, Brambati Simona M, Wilson Maximiliano A
1 Département de réadaptation, Faculté de médecine, Université Laval, Québec, Canada.
2 Centre de recherche de l'Institut universitaire en santé mentale de Québec, Québec, Canada.
J Geriatr Psychiatry Neurol. 2017 May;30(3):151-161. doi: 10.1177/0891988717700507. Epub 2017 Mar 30.
Primary progressive aphasia (PPA) is a heterogeneous neurodegenerative condition in which the most prominent clinical feature is language difficulties. Other cognitive domains have been described to remain unaffected at the early stages of the disease and, therefore, excluded from diagnostic criteria. However, we show in this article that executive function (EF) disorders may be present in the 3 variants (nonfluent/agrammatic, logopenic, and semantic) of PPA. We also illustrate changes in language and EF by means of a 3-year behavioral and neuroimaging longitudinal study of a patient suffering from the semantic variant of PPA. This review provides an update on current knowledge of PPA, suggesting that dysexecutive symptoms may be encountered in the 3 PPA variants, in their early phases and/or in more advanced stages, when atrophy extends to adjacent brain areas.
原发性进行性失语(PPA)是一种异质性神经退行性疾病,其最突出的临床特征是语言障碍。据描述,在疾病早期其他认知领域未受影响,因此被排除在诊断标准之外。然而,我们在本文中表明,执行功能(EF)障碍可能存在于PPA的3种变体(非流利/语法缺失型、音韵性失语型和语义性失语型)中。我们还通过对一名患有语义性失语型PPA患者进行的为期3年的行为和神经影像学纵向研究,阐述了语言和执行功能的变化。本综述提供了关于PPA当前知识的更新,表明在PPA的3种变体的早期阶段和/或更晚期阶段,当萎缩扩展到相邻脑区时,可能会出现执行功能障碍症状。