Altered neural oscillations in classical galactosaemia during sentence production

IF 4.2 2区 医学 Q1 ENDOCRINOLOGY & METABOLISM
Sara Mazzini, Sai Yadnik, Inge Timmers, Estela Rubio-Gozalbo, Bernadette M. Jansma
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Abstract

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.

Abstract Image

经典半乳糖血症患者在造句过程中的神经振荡发生变化
典型半乳糖血症(CG)是一种半乳糖代谢遗传性疾病,尽管可以通过饮食治疗,但仍存在多种认知障碍,其中包括语言能力。研究人员利用多种神经影像学技术对古柯碱血症患者的大脑功能进行了研究,发现了结构和功能上的不典型性。在本研究中,我们首次比较了 CG 患者和健康对照组在完成语言生产任务时脑电图(EEG)中的振荡动态,尤其是功率谱和时频表征(TFR)。21 名 CG 患者和 19 名健康对照者用完整的句子或单词描述了动画场景,这表明句法规划有两种复杂程度。基于之前对 P300 事件相关电位(ERP)及其与θ频率关系的研究,我们假设患者和对照组的振荡活动在θ功率和TFR方面存在差异。在行为方面,患者的反应时间较慢,这反映了他们的语言障碍。在功率谱方面,我们观察到患者在δ(1-3 赫兹)、θ(4-7 赫兹)、β(15-30 赫兹)和γ(30-70 赫兹)频率上的功率明显较高,而在α(8-12 赫兹)频率上则没有,这表明患者的振荡特征不典型。时频分析显示,句子条件下患者的事件相关θ同步(ERS)和α非同步(ERD)明显较弱。这些数据支持了 CG 语言障碍与大脑θ-α振荡有关的假设。
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来源期刊
Journal of Inherited Metabolic Disease
Journal of Inherited Metabolic Disease 医学-内分泌学与代谢
CiteScore
9.50
自引率
7.10%
发文量
117
审稿时长
4-8 weeks
期刊介绍: The Journal of Inherited Metabolic Disease (JIMD) is the official journal of the Society for the Study of Inborn Errors of Metabolism (SSIEM). By enhancing communication between workers in the field throughout the world, the JIMD aims to improve the management and understanding of inherited metabolic disorders. It publishes results of original research and new or important observations pertaining to any aspect of inherited metabolic disease in humans and higher animals. This includes clinical (medical, dental and veterinary), biochemical, genetic (including cytogenetic, molecular and population genetic), experimental (including cell biological), methodological, theoretical, epidemiological, ethical and counselling aspects. The JIMD also reviews important new developments or controversial issues relating to metabolic disorders and publishes reviews and short reports arising from the Society''s annual symposia. A distinction is made between peer-reviewed scientific material that is selected because of its significance for other professionals in the field and non-peer- reviewed material that aims to be important, controversial, interesting or entertaining (“Extras”).
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