PDF(Sci-hub)
Abstract：
Systematically review the abnormalities in event related potential (ERP) recorded in Rett Syndrome (RTT) patients and animals in search of translational biomarkers of deficits related to the particular neurophysiological processes of known genetic origin (MECP2 mutations).
Pubmed, ISI Web of Knowledge and BIORXIV were searched for the relevant articles according to PRISMA standards.
ERP components are generally delayed across all sensory modalities both in RTT patients and its animal model, while findings on ERPs amplitude strongly depend on stimulus properties and presentation rate. Studies on RTT animal models uncovered the abnormalities in the excitatory and inhibitory transmission as critical mechanisms underlying the ERPs changes, but showed that even similar ERP alterations in auditory and visual domains have a diverse neural basis. A range of novel approaches has been developed in animal studies bringing along the meaningful neurophysiological interpretation of ERP measures in RTT patients.
While there is a clear evidence for sensory ERPs abnormalities in RTT, to further advance the field there is a need in a large-scale ERP studies with the functionally-relevant experimental paradigms.
The review provides insights into domain-specific neural basis of the ERP abnormalities and promotes clinical application of the ERP measures as the non-invasive functional biomarkers of RTT pathophysiology.
Pubmed, ISI Web of Knowledge and BIORXIV were searched for the relevant articles according to PRISMA standards.
ERP components are generally delayed across all sensory modalities both in RTT patients and its animal model, while findings on ERPs amplitude strongly depend on stimulus properties and presentation rate. Studies on RTT animal models uncovered the abnormalities in the excitatory and inhibitory transmission as critical mechanisms underlying the ERPs changes, but showed that even similar ERP alterations in auditory and visual domains have a diverse neural basis. A range of novel approaches has been developed in animal studies bringing along the meaningful neurophysiological interpretation of ERP measures in RTT patients.
While there is a clear evidence for sensory ERPs abnormalities in RTT, to further advance the field there is a need in a large-scale ERP studies with the functionally-relevant experimental paradigms.
The review provides insights into domain-specific neural basis of the ERP abnormalities and promotes clinical application of the ERP measures as the non-invasive functional biomarkers of RTT pathophysiology.
摘要：
系统地回顾Rett综合征（RTT）患者和动物中记录的事件相关电位（ERP）异常，以寻找与已知遗传起源（MECP2突变）的特定神经生理过程相关的缺陷的翻译生物标志物。
已发布，根据PRISMA标准搜索了ISIWebofKnowledge和BIORXIV的相关文章。
在RTT患者及其动物模型中，ERP组件在所有感觉模式中通常都会延迟，而ERPs振幅的发现强烈依赖于刺激特性和呈现率。对RTT动物模型的研究揭示了兴奋性和抑制性传递的异常是ERP变化的关键机制。但表明，即使在听觉和视觉领域类似的ERP改变也具有不同的神经基础。在动物研究中已经开发了一系列新颖的方法，从而对RTT患者的ERP措施进行了有意义的神经生理学解释。
虽然有明确的证据表明RTT中的感觉ERP异常，为了进一步推进该领域，需要使用功能相关的实验范式进行大规模的ERP研究。
这篇综述提供了对ERP异常的领域特异性神经基础的见解，并促进了ERP措施作为RTT病理生理学的非侵入性功能生物标志物的临床应用。
已发布，根据PRISMA标准搜索了ISIWebofKnowledge和BIORXIV的相关文章。
在RTT患者及其动物模型中，ERP组件在所有感觉模式中通常都会延迟，而ERPs振幅的发现强烈依赖于刺激特性和呈现率。对RTT动物模型的研究揭示了兴奋性和抑制性传递的异常是ERP变化的关键机制。但表明，即使在听觉和视觉领域类似的ERP改变也具有不同的神经基础。在动物研究中已经开发了一系列新颖的方法，从而对RTT患者的ERP措施进行了有意义的神经生理学解释。
虽然有明确的证据表明RTT中的感觉ERP异常，为了进一步推进该领域，需要使用功能相关的实验范式进行大规模的ERP研究。
这篇综述提供了对ERP异常的领域特异性神经基础的见解，并促进了ERP措施作为RTT病理生理学的非侵入性功能生物标志物的临床应用。
