Neurobiology of Language Department, Max Planck Institute for Psycholinguistics, Nijmegen, The Netherlands.
Department of Cognitive Neuroscience, Faculty of Psychology and Neuroscience, Maastricht University, Maastricht, The Netherlands.
J Inherit Metab Dis. 2024 Jul;47(4):690-702. doi: 10.1002/jimd.12740. Epub 2024 Apr 10.
Classical galactosaemia (CG) is a hereditary disease in galactose metabolism that despite dietary treatment is characterized by a wide range of cognitive deficits, among which is language production. CG brain functioning has been studied with several neuroimaging techniques, which revealed both structural and functional atypicalities. In the present study, for the first time, we compared the oscillatory dynamics, especially the power spectrum and time-frequency representations (TFR), in the electroencephalography (EEG) of CG patients and healthy controls while they were performing a language production task. Twenty-one CG patients and 19 healthy controls described animated scenes, either in full sentences or in words, indicating two levels of complexity in syntactic planning. Based on previous work on the P300 event related potential (ERP) and its relation with theta frequency, we hypothesized that the oscillatory activity of patients and controls would differ in theta power and TFR. With regard to behavior, reaction times showed that patients are slower, reflecting the language deficit. In the power spectrum, we observed significant higher power in patients in delta (1-3 Hz), theta (4-7 Hz), beta (15-30 Hz) and gamma (30-70 Hz) frequencies, but not in alpha (8-12 Hz), suggesting an atypical oscillatory profile. The time-frequency analysis revealed significantly weaker event-related theta synchronization (ERS) and alpha desynchronization (ERD) in patients in the sentence condition. The data support the hypothesis that CG language difficulties relate to theta-alpha brain oscillations.
经典型半乳糖血症(CG)是一种遗传性的半乳糖代谢疾病,尽管进行了饮食治疗,但仍表现出广泛的认知缺陷,其中包括语言产生。已经使用多种神经影像学技术研究了 CG 大脑的功能,这些技术揭示了结构和功能的异常。在本研究中,我们首次比较了 CG 患者和健康对照组在执行语言产生任务时的脑电图(EEG)中的振荡动力学,特别是功率谱和时频表示(TFR)。21 名 CG 患者和 19 名健康对照组以完整句子或单词的形式描述动画场景,这表明句法计划有两个复杂程度。基于先前关于 P300 事件相关电位(ERP)及其与θ频率的关系的工作,我们假设患者和对照组的振荡活动在θ功率和 TFR 方面会有所不同。就行为而言,反应时间表明患者较慢,反映了语言缺陷。在功率谱中,我们观察到患者在 delta(1-3 Hz)、theta(4-7 Hz)、beta(15-30 Hz)和 gamma(30-70 Hz)频率下的功率显著更高,但在 alpha(8-12 Hz)频率下没有,这表明存在非典型的振荡模式。时频分析显示,在句子条件下,患者的事件相关 theta 同步(ERS)和 alpha 去同步(ERD)明显较弱。数据支持 CG 语言困难与θ-α脑振荡有关的假设。