Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College Dublin, 152-160 Pearse Street, Dublin 2, Ireland.
Department of Psychology, Beaumont Hospital, Dublin, Ireland.
Neuropsychol Rev. 2018 Jun;28(2):251-268. doi: 10.1007/s11065-018-9375-7. Epub 2018 Apr 28.
Amyotrophic Lateral Sclerosis is a neurodegenerative disorder characterized primarily by motor network disruption. Extra-motor manifestations including executive functions, social cognition, and behavioral changes are now well recognized as important features of ALS, and are associated with frontotemporal and frontostriatal network disruption. However, the presence and characterization of language changes has received less attention. This systematic review characterizes the profile of reported language dysfunction in ALS. PRISMA guidelines were implemented to carry out and report the review. Current evidence suggests that areas of neuroanatomical disruption in ALS spread to language centers such as posterior, inferior frontal and superior temporal areas leading to deficits in word retrieval, syntactic and grammatical processing, and spelling. However, the majority of studies of language in ALS have been limited by the recruitment of small clinic-based prevalent samples and important questions remain regarding the incidence and progression of language impairment in ALS. Further studies from population-based incident cohorts will help to determine the range of language deficits in ALS, and how these relate to previously defined executive and behavioral sub-phenotypes.
肌萎缩侧索硬化症是一种主要以运动网络紊乱为特征的神经退行性疾病。现在已经明确认识到，运动外表现包括执行功能、社会认知和行为改变是 ALS 的重要特征，与额颞叶和额纹状体网络紊乱有关。然而，语言变化的存在和特征尚未得到足够重视。本系统综述对 ALS 中报道的语言功能障碍的特征进行了描述。采用 PRISMA 指南进行并报告了此次综述。目前的证据表明，ALS 中神经解剖学紊乱的区域会扩散到语言中枢，如后、下额和上颞区，导致词汇检索、句法和语法处理以及拼写方面的缺陷。然而，ALS 语言研究的大多数研究都受到小范围临床流行样本招募的限制，关于 ALS 中语言障碍的发生率和进展仍存在重要问题。来自基于人群的发病队列的进一步研究将有助于确定 ALS 中语言缺陷的范围，以及这些缺陷与之前定义的执行和行为亚型的关系。
