Pinto-Grau Marta, Donohoe Bronagh, O'Connor Sarah, Murphy Lisa, Costello Emmet, Heverin Mark, Vajda Alice, Hardiman Orla, Pender Niall
Academic Unit of Neurology (MP-G, BD, SO, LM, EC, MH, AV, OH, NP), Trinity Biomedical Sciences Institute, Trinity College Dublin; and Department of Psychology (MP-G, BD, SO, LM, EC, NP), Beaumont Hospital, Dublin, Ireland.
Neurol Clin Pract. 2021 Oct;11(5):e634-e644. doi: 10.1212/CPJ.0000000000001006.
To investigate the incidence and nature of language change and its relationship to executive dysfunction in a population-based incident amyotrophic lateral sclerosis (ALS) sample, with the hypothesis that patterns of frontotemporal involvement in early ALS extend beyond areas of executive control to regions associated with language processing.
One hundred seventeen population-based incident ALS cases without dementia and 100 controls matched by age, sex, and education were included in the study. A detailed assessment of language processing including lexical processing, word spelling, word reading, word naming, semantic processing, and syntactic/grammatical processing was undertaken. Executive domains of phonemic verbal fluency, working memory, problem-solving, cognitive flexibility, and social cognition were also evaluated.
Language processing was impaired in this incident cohort of individuals with ALS, with deficits in the domains of word naming, orthographic processing, and syntactic/grammatical processing. Conversely, phonological lexical processing and semantic processing were spared. Although executive dysfunction accounted in part for impairments in grammatical and orthographic lexical processing, word spelling, reading, and naming, primary language deficits were also present.
Language impairment is characteristic of ALS at early stages of the disease and can develop independently of executive dysfunction, reflecting selective patterns of frontotemporal involvement at disease onset. Language change is therefore an important component of the frontotemporal syndrome associated with ALS.
在一个基于人群的肌萎缩侧索硬化症（ALS）新发病例样本中，研究语言变化的发生率、性质及其与执行功能障碍的关系，假设早期ALS中额颞叶受累模式超出执行控制区域，延伸至与语言处理相关的区域。
本研究纳入了117例无痴呆的基于人群的ALS新发病例以及100例年龄、性别和教育程度相匹配的对照。对语言处理进行了详细评估，包括词汇处理、单词拼写、单词阅读、单词命名、语义处理和句法/语法处理。还评估了音素言语流畅性、工作记忆、问题解决、认知灵活性和社会认知等执行领域。
在这个ALS新发病例队列中，语言处理存在受损情况，在单词命名、正字法处理和句法/语法处理领域存在缺陷。相反，语音词汇处理和语义处理未受影响。尽管执行功能障碍部分导致了语法和正字法词汇处理、单词拼写、阅读和命名方面的损伤，但主要的语言缺陷也存在。
语言障碍是ALS疾病早期的特征，并且可以独立于执行功能障碍而出现，反映了疾病发作时额颞叶受累的选择性模式。因此，语言变化是与ALS相关的额颞叶综合征的一个重要组成部分。
