ABSTRACT

Girls with Fragile-X-Syndrome (FXS) present high levels of social anxiety, social avoidance, extreme shyness, tendency to social isolation, poor eye contact, learning difficulties, and depression. The aims of the present study, which is based on a group of young females with FXS are: 1) to analyze the possible associations between emotion recognition, theory of mind, and social anxiety, and adaptive behavior, and emotional state; 2) to study the relationship between intelligence quotient (IQ) and adaptive behavior; and 3) to assess whether social anxiety is more prevalent in girls with FXS. The study has 40 female participants aged between 7 and 16 years (26 positive full mutation FXS and 14 as a control group). A neuropsychological assessment was conducted using the following tests: WISC-V, NEPSY-II, SENA, ADHD Rating Scale, BAS, and ABAS-II. In comparison with the control group, the group with FXS presented a greater association between IQ and self-direction ability, and between emotion recognition and leadership. The FXS group presented higher levels of social anxiety and shyness. In the group of girls with FXS, IQ may have prognostic value for both self-direction ability and social adaptation level.

摘要

患有脆性 X 综合征 (FXS) 的女孩表现出高度的社交焦虑、社交回避、极度害羞、社交孤立倾向、眼神接触不良、学习困难和抑郁。本研究的目的是基于一组患有 FXS 的年轻女性：1) 分析情绪识别、心理理论和社交焦虑、适应性行为和情绪状态之间可能的关联；2）研究智商（IQ）与适应行为的关系；3) 评估社交焦虑是否在患有 FXS 的女孩中更为普遍。该研究有 40 名年龄在 7 至 16 岁之间的女性参与者（26 名阳性全突变 FXS 和 14 名作为对照组）。使用以下测试进行神经心理学评估：WISC-V、NEPSY-II、SENA、ADHD 评定量表、BAS 和 ABAS-II。与对照组相比，FXS 组在智商和自我导向能力之间，以及在情绪识别和领导力之间表现出更大的关联。FXS 组表现出更高水平的社交焦虑和害羞。在患有 FXS 的女孩组中，智商可能对自我导向能力和社会适应水平具有预后价值。