Vanderbilt Kennedy Center for Research on Human Development, Nashville, TN, USA.
Department of Hearing and Speech Sciences, Vanderbilt University Medical Center, Nashville, TN, USA.
J Intellect Disabil Res. 2019 Mar;63(3):244-254. doi: 10.1111/jir.12570. Epub 2018 Nov 23.
The combination of intellectual, communicative and motor deficits limits the use of standardised behavioural assessments in individuals with Angelman syndrome (AS). The current study aimed to objectively evaluate the extent of social-emotional processing in AS using auditory event-related potentials (ERPs) during passive exposure to spoken stimuli.
Auditory ERP responses were recorded in 13 nonverbal individuals with the deletion subtype of AS, age 4-45 years, during the name recognition paradigm, in which their own names and names of close others (relative or friend) were presented among novel names. No behavioural responses were required.
Contrary to findings in typical children and adults, there was no significant evidence of differential neural response to known vs. novel names in participants with AS. Nevertheless, greater amplitude differences between known and unknown names demonstrated the predicted association with better interpersonal relationships and receptive communication abilities.
These findings indicate good tolerability of ERP procedures (85% success rate). The lack of own name differentiation is consistent with increased incidence of the autism-related symptoms in AS. Strong associations between the caregiver reports of adaptive functioning and neural indices of known name recognition support the utility of brain-based measures for objectively evaluating cognitive and affective processes in nonverbal persons with neurodevelopmental disorders.
智力、沟通和运动缺陷的结合限制了在患有 Angelman 综合征（AS）的个体中使用标准化行为评估。本研究旨在使用听觉事件相关电位（ERP）在被动暴露于口语刺激时客观地评估 AS 患者的社会情感处理程度。
在名称识别范式中记录了 13 名患有缺失型 AS 的非言语个体的听觉 ERP 反应，年龄在 4-45 岁之间，在此期间，他们自己的名字和亲近他人（亲戚或朋友）的名字与新名字一起呈现。不需要行为反应。
与典型儿童和成人的发现相反，AS 参与者对已知和未知名称的神经反应没有明显差异的证据。尽管如此，已知和未知名称之间的振幅差异越大，与更好的人际关系和接受性沟通能力的预期关联就越大。
这些发现表明 ERP 程序具有良好的耐受性（成功率为 85%）。缺乏自己名字的差异与 AS 中自闭症相关症状的发生率增加一致。照顾者对适应功能的报告与已知名称识别的神经指数之间的强烈关联支持使用基于大脑的措施客观评估神经发育障碍的非言语个体的认知和情感过程。
