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From Mind to Mouth: Event Related Potentials of Sentence Production in Classic Galactosemia

机译:从头脑到嘴巴:经典半乳糖血症中与事件相关的句子产生潜能

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摘要

Patients with classic galactosemia, an inborn error of metabolism, have speech and language production impairments. Past research primarily focused on speech (motor) problems, but these cannot solely explain the language impairments. Which specific deficits contribute to the impairments in language production is not yet known. Deficits in semantic and syntactic planning are plausible and require further investigation. In the present study, we examined syntactic encoding while patients and matched controls overtly described scenes of moving objects using either separate words (minimal syntactic planning) or sentences (sentence-level syntactic planning). The design of the paradigm also allowed tapping into local noun phrase- and more global sentence-level syntactic planning. Simultaneously, we recorded event-related potentials (ERPs). The patients needed more time to prepare and finish the utterances and made more errors. The patient ERPs had a very similar morphology to that of healthy controls, indicating overall comparable neural processing. Most importantly, the ERPs diverged from those of controls in several functionally informative time windows, ranging from very early (90–150 ms post scene onset) to relatively late (1820–2020 ms post scene onset). These time windows can be associated with different linguistic encoding stages. The ERP results form the first neuroscientific evidence for language production impairments in patients with galactosemia in lexical and syntactic planning stages, i.e., prior to the linguistic output phase. These findings hence shed new light on the language impairments in this disease.
机译:患有典型的半乳糖血症,先天性代谢错误的患者会出现言语和语言障碍。过去的研究主要集中在言语(运动)问题上,但是这些不能仅解释语言障碍。尚不清楚哪些具体的缺陷会导致语言能力的下降。语义和句法计划上的缺陷是合理的,需要进一步研究。在本研究中,我们检查了句法编码,同时使用单独的单词(最小句法计划)或句子(句子级句法计划)对患者和匹配的控件公开描述了移动对象的场景。该范式的设计还允许使用本地名词短语和更全局的句子级句法计划。同时,我们记录了事件相关电位(ERP)。患者需要更多的时间来准备和完成话语,并且犯了更多的错误。病人的ERPs与健康对照组的形态非常相似,表明神经处理总体可比。最重要的是,ERP在几个功能丰富的时间窗口中与控件的ERP有所不同,范围从很早(场景发生后90-150 ms)到相对较晚(场景发生后1820-2020 ms)。这些时间窗口可以与不同的语言编码阶段相关联。 ERP结果形成了在词法和句法计划阶段(即在语言输出阶段之前)的半乳糖血症患者语言产生障碍的首个神经科学证据。因此,这些发现为这种疾病的语言障碍提供了新的启示。

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