Bruno Jennifer Lynn, Hosseini S M Hadi, Saggar Manish, Quintin Eve-Marie, Raman Mira Michelle, Reiss Allan L
Department of Psychiatry, Center for Interdisciplinary Brain Sciences Research, Stanford, CA 94305-5795, USA.
School and Applied Child Psychology Program, McGill University, Montreal, QC, CanadaH3A 1Y2.
Cereb Cortex. 2017 Mar 1;27(3):2249-2259. doi: 10.1093/cercor/bhw055.
Fragile X syndrome (FXS), the most common inherited cause of intellectual disability and autism spectrum disorder, is associated with significant behavioral, social, and neurocognitive deficits. Understanding structural brain network topology in FXS provides an important link between neurobiological and behavioral/cognitive symptoms of this disorder. We investigated the connectome via whole-brain structural networks created from group-level morphological correlations. Participants included 100 individuals: 50 with FXS and 50 with typical development, age 11-23 years. Results indicated alterations in topological properties of structural brain networks in individuals with FXS. Significantly reduced small-world index indicates a shift in the balance between network segregation and integration and significantly reduced clustering coefficient suggests that reduced local segregation shifted this balance. Caudate and amygdala were less interactive in the FXS network further highlighting the importance of subcortical region alterations in the neurobiological signature of FXS. Modularity analysis indicates that FXS and typically developing groups' networks decompose into different sets of interconnected sub networks, potentially indicative of aberrant local interconnectivity in individuals with FXS. These findings advance our understanding of the effects of fragile X mental retardation protein on large-scale brain networks and could be used to develop a connectome-level biological signature for FXS.
脆性X综合征(FXS)是智力残疾和自闭症谱系障碍最常见的遗传病因,与显著的行为、社交和神经认知缺陷有关。了解FXS患者的脑结构网络拓扑结构,为该疾病的神经生物学症状与行为/认知症状之间提供了重要联系。我们通过基于群体水平形态学相关性构建的全脑结构网络来研究脑连接组。研究对象包括100名个体:50名FXS患者和50名发育正常的个体,年龄在11至23岁之间。结果表明,FXS患者脑结构网络的拓扑性质存在改变。小世界指数显著降低表明网络隔离与整合之间的平衡发生了变化,聚类系数显著降低表明局部隔离的减少改变了这种平衡。在FXS网络中,尾状核和杏仁核的交互作用较少,这进一步凸显了皮质下区域改变在FXS神经生物学特征中的重要性。模块分析表明,FXS组和发育正常组的网络分解为不同的相互连接的子网集,这可能表明FXS患者存在异常的局部连接性。这些发现增进了我们对脆性X智力低下蛋白对大规模脑网络影响的理解,并可用于开发FXS的连接组水平生物学特征。