Social Cognition in Female Adolescents with Fragile X Syndrome

描述(由申请人提供)：拟议项目的目的是描述患有FXS的女性青少年在社会认知测试中的表现，这些测试对执行功能(EFS)的需求有所不同。这项研究的动机是越来越多的证据表明，患有FXS的人在社会认知和EFS方面存在特定的损害，超出了智力残疾的范围。这些缺陷与FXS青少年及其家庭的主要社会残疾和照顾者负担有关，并可能是其基础，这方面的知识对临床干预和表型发育模式都有意义。我们开发了社会认知任务，直接操纵EFS的两个方面--工作记忆和抑制控制。这些任务需要在线社会信息处理，并且是与青少年合作开发的，以反映他们的日常社会生活；因此，它们对于评估这个年龄段的社会认知具有面子和生态有效性。这些任务将被用来表征智商在75或以上的FXS青少年的社会认知表现。分数将与自我和父母对社会行为的感知进行比较，以探索社会认知任务分数与实验室以外结果的关系。社会认知技能在适合同伴的社会行为中起着关键作用，也可能是获得特定文化社会知识所必需的。因此，患有发育性社会缺陷的青少年不仅可能遭受身体结构和功能方面的损害，而且还可能在社会活动和参与社会生活方面受到限制。这反过来可能会对主观幸福感和健康产生各种负面影响。因此，对青少年社会认知的研究不仅具有重要的理论意义，而且具有重要的公共卫生意义。

DESCRIPTION (provided by applicant): The purpose of the proposed project is to characterize the performance of female adolescents with FXS on tests of social cognition that vary in their demand on executive functions (EFs). The study was motivated by the growing evidence of specific impairments in both social cognition and EFs in individuals with FXS, beyond what can be accounted for by intellectual disability. These deficits are associated with, and may underlie, major social disability and caregiver burden for adolescents with FXS and their families, and knowledge in this area has implications for both clinical intervention and models of phenotype development. We have developed social cognition tasks that directly manipulate two aspects of EFs, working memory and inhibitory control. The tasks require on-line social information processing and were developed in collaboration with adolescents to reflect their daily social lives; thus they have face and ecological validity for the evaluation of social cognition in this age group. The tasks will be used to characterize social cognition performance in adolescents with FXS who have IQs of 75 or above. Scores will be compared to self- and parent perceptions of social behavior, to explore the relation of social cognition task scores to outcomes beyond the laboratory. Social cognitive skills play a critical role in peer-appropriate social behavior, and also may be required for the acquisition of culture-specific social knowledge. Thus, adolescents with developmental social deficits may experience not only impairments at the level of body structures and functions, but also limitations in social activities and restricted participation in social life. This may in turn produce a variety of negative effects on subjective well-being and health. Thus, the study of adolescent social cognition is important not only for theoretical reasons but as a public health issue.