Ninety-two children with velocardiofacial syndrome (VCFS), a genetic disorder caused by a microdeletion of chromosome 22q11.2 and an age, race, and gender-ratio comparable sample of 59 control participants were included in the project. Participants received an MRI as well as a comprehensive neuropsychological battery; the primary outcome measure in the current report is the Rey-Osterrieth Complex Figure (ROCF). Children with VCFS performed less well on the ROCF and have lower whole brain volume compared to controls. After controlling for whole brain volume differences, children with VCFS have bilaterally less parietal lobe gray and white matter yet more frontal lobe white matter. Brain–behavior relationships include: (a) for both groups, parietal volumes (both gray and white matter) predicted ROCF Copy Organization performance and frontal volumes (both gray and white matter) predicted ROCF Copy Accuracy performance; (b) for controls, frontal white matter also predicted ROCF Copy Organization performance; (c) ROCF Recall Organization performance was best predicted by frontal gray matter volume only in our controls; ROCF Recall Accuracy performance was best predicted by frontal gray matter volume in both groups; and (d) in children with VCFS, performance on the ROCF-Copy Structural Elements Accuracy scale was predicted by right hemisphere white matter volume. Our hypotheses were also retested using IQ-matched and whole brain volume-matched subsamples. Identical results were obtained in these analyses. Assumptions about the organization of and the localization of the brain structures that subserve specific cognitive functions in the typically developing brain may not apply in the abnormally developing brain.

中文翻译：

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Rey-Osterrieth 复杂图形的表现与有和没有 Velocardiofacial 综合征儿童的区域脑容量之间的关联

该项目包括 92 名患有 velocardiofacial 综合征 (VCFS) 的儿童，这是一种由染色体 22q11.2 微缺失引起的遗传疾病，以及 59 名对照参与者的年龄、种族和性别比例可比样本。参与者接受了 MRI 以及全面的神经心理学测试；本报告中的主要结果衡量指标是 Rey-Osterrieth 复杂图 (ROCF)。与对照组相比，VCFS 儿童在 ROCF 上的表现较差，全脑容量较低。在控制全脑容量差异后，VCFS 儿童双侧顶叶灰质和白质较少，而额叶白质较多。大脑-行为关系包括： (a) 对于两组，顶叶体积（灰质和白质）预测 ROCF 复制组织性能，额叶体积（灰质和白质）预测 ROCF 复制精度性能；(b) 对于对照，额叶白质也预测了 ROCF Copy Organization 的表现；(c) ROCF 召回组织性能仅在我们的控制中通过额叶灰质体积进行最佳预测；ROCF Recall Accuracy 性能最好由两组的额叶灰质体积预测；(d) 在患有 VCFS 的儿童中，ROCF-Copy Structural Elements Accuracy 量表的表现由右半球白质体积预测。我们的假设还使用 IQ 匹配和全脑容量匹配的子样本进行了重新测试。在这些分析中获得了相同的结果。