de la Sablonnière Justine, Tastevin Maud, Lavoie Monica, Laforce Robert
Clinique Interdisciplinaire de Mémoire, Département des Sciences Neurologiques du CHU de Québec, Faculté de Médecine, Université Laval, Quebec City, QC G1J 1Z4, Canada.
Brain Sci. 2021 Sep 14;11(9):1209. doi: 10.3390/brainsci11091209.
Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases presenting with insidious and relentless language impairment. Three main PPA variants have been described: the non-fluent/agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). At the time of diagnosis, patients and their families' main question pertains to prognosis and evolution, but very few data exist to support clinicians' claims. The objective of this study was to review the current literature on the longitudinal changes in cognition, behaviours, and functional abilities in the three main PPA variants. A comprehensive review was undertaken via a search on PUBMED and EMBASE. Two authors independently reviewed a total of 65 full-text records for eligibility. A total of 14 group studies and one meta-analysis were included. Among these, eight studies included all three PPA variants. Eight studies were prospective, and the follow-up duration was between one and five years. Overall, svPPA patients showed more behavioural disturbances both at baseline and over the course of the disease. Patients with lvPPA showed a worse cognitive decline, especially in episodic memory, and faster progression to dementia. Finally, patients with nfvPPA showed the most significant losses in language production and functional abilities. Data regarding the prodromal and last stages of PPA are still missing and studies with a longer follow-up observation period are needed.
原发性进行性失语症（PPA）是一组以隐匿性和持续性语言障碍为特征的神经退行性疾病。已描述了三种主要的PPA变异型：非流利/语法缺失型变异（nfvPPA）、语义型变异（svPPA）和音韵性变异（lvPPA）。在诊断时，患者及其家属的主要问题涉及预后和病情发展，但几乎没有数据支持临床医生的说法。本研究的目的是回顾当前关于三种主要PPA变异型认知、行为和功能能力纵向变化的文献。通过在PUBMED和EMBASE上进行检索进行了全面综述。两位作者独立审查了总共65篇全文记录的合格性。共纳入14项群组研究和1项荟萃分析。其中，8项研究纳入了所有三种PPA变异型。8项研究为前瞻性研究，随访时间为1至5年。总体而言，svPPA患者在基线期和疾病过程中均表现出更多行为障碍。lvPPA患者的认知衰退更严重，尤其是情景记忆方面，且向痴呆进展更快。最后，nfvPPA患者在语言表达和功能能力方面的损失最为显著。关于PPA前驱期和终末期的数据仍然缺失，需要进行随访观察期更长的研究。
