Fragile X syndrome (FXS) is a genetic condition associated with increased risk for social anxiety and avoidance. Using functional near-infrared spectroscopy (fNIRS), we previously demonstrated aberrant neural activity responding to faces in young girls with FXS cross-sectionally. Here, we tested the hypothesis that abnormalities in neural activation and sensitization would increase with age in 65 girls with FXS (ages 6–16 years) relative to an age-matched control group of 52 girls who had comparable cognitive function and clinical symptoms. fNIRS data were collected at 2 time points (mean [SD] = 2.8 [0.6] years apart) during a face processing task. Linear mixed-effect models examined longitudinal neural profiles in girls with FXS and control participants. Correlational analysis was performed to examine associations between neural sensitization (increasing neural response to repeated stimuli) and clinical ratings. In the FXS group, 24 participants had 1 fNIRS scan, and 32 had 2 scans. In the control group, 28 participants had 1 fNIRS scan, and 22 had 2 scans. Brain activations in the superior frontal gyrus were higher in girls with FXS than control participants at both time points. Neural sensitization also increased in girls with FXS at a higher rate than control participants in the superior frontal gyrus when responding to upright faces. For the FXS group, sensitization in the superior frontal gyrus positively correlated with longitudinal increases in anxiety and social avoidance scores. Girls with FXS show increasingly abnormal neural activation and sensitization responding to faces over time. Aberrant neural sensitization in girls with FXS is associated with longitudinal changes in anxiety and social skills.

中文翻译：

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脆性 X 综合征面部处理过程中功能性神经激活和敏化的纵向变化


脆性 X 综合征 (FXS) 是一种与社交焦虑和回避风险增加相关的遗传性疾病。使用功能性近红外光谱 (fNIRS)，我们之前证明了患有 FXS 的年轻女孩对面部做出反应的异常神经活动。在这里，我们测试了这样的假设：相对于由 52 名具有相似认知功能和临床症状的年龄匹配女孩组成的年龄匹配对照组，65 名 FXS 女孩（6-16 岁）的神经激活和敏化异常会随着年龄的增长而增加。在人脸处理任务期间，在 2 个时间点（平均 [SD] = 2.8 [0.6] 年）收集 fNIRS 数据。线性混合效应模型检查了患有 FXS 的女孩和对照组参与者的纵向神经特征。进行相关分析以检查神经敏化（增加对重复刺激的神经反应）和临床评级之间的关联。在 FXS 组中，24 名参与者进行了 1 次 fNIRS 扫描，32 名参与者进行了 2 次扫描。在对照组中，28 名参与者进行了 1 次 fNIRS 扫描，22 名参与者进行了 2 次扫描。在这两个时间点，患有 FXS 的女孩额上回的大脑激活均高于对照组参与者。当对直立面部做出反应时，患有 FXS 的女孩额上回的神经敏感度也比对照组参与者更高。对于 FXS 组，额上回的敏化与焦虑和社交回避得分的纵向增加呈正相关。随着时间的推移，患有 FXS 的女孩表现出越来越异常的神经激活和对面孔的敏感反应。患有 FXS 的女孩异常的神经敏化与焦虑和社交技能的纵向变化有关。