Data_Sheet_1_Path Integration and Cognitive Mapping Capacities in Down and Williams Syndromes.docx

Williams (WS) and Down (DS) syndromes are neurodevelopmental disorders with distinct genetic origins and different spatial memory profiles. In real-world spatial memory tasks, where spatial information derived from all sensory modalities is available, individuals with DS demonstrate low-resolution spatial learning capacities consistent with their mental age, whereas individuals with WS are severely impaired. However, because WS is associated with severe visuo-constructive processing deficits, it is unclear whether their impairment is due to abnormal visual processing or whether it reflects an inability to build a cognitive map. Here, we tested whether blindfolded individuals with WS or DS, and typically developing (TD) children with similar mental ages, could use path integration to perform an egocentric homing task and return to a starting point. We then evaluated whether they could take shortcuts and navigate along never-traveled trajectories between four objects while blindfolded, thus demonstrating the ability to build a cognitive map. In the homing task, 96% of TD children, 84% of participants with DS and 44% of participants with WS were able to use path integration to return to their starting point consistently. In the cognitive mapping task, 64% of TD children and 74% of participants with DS were able to take shortcuts and use never-traveled trajectories, the hallmark of cognitive mapping ability. In contrast, only one of eighteen participants with WS demonstrated the ability to build a cognitive map. These findings are consistent with the view that hippocampus-dependent spatial learning is severely impacted in WS, whereas it is relatively preserved in DS.

威廉姆斯综合症（WS）和唐氏综合症（DS）均为具有独特遗传起源和不同空间记忆特征的神经发育障碍。在现实世界的空间记忆任务中，当所有感官模态的空间信息均可用时，DS患者表现出与智力年龄相一致的低分辨率空间学习能力，而WS患者则表现出严重损害。然而，由于WS与严重的视觉构建处理缺陷相关联，其损害是否源于异常的视觉处理，或是否反映了构建认知地图的能力不足，尚不明确。在本研究中，我们测试了WS或DS的盲人个体以及具有相似智力年龄的典型发展（TD）儿童是否能够利用路径积分来完成以自我为中心的归巢任务并返回起点。随后，我们评估了他们在盲状态下能否在四个物体之间采取捷径并沿从未走过的轨迹导航，从而证明其构建认知地图的能力。在归巢任务中，96%的TD儿童、84%的DS参与者和44%的WS参与者能够持续利用路径积分返回起点。在认知地图构建任务中，64%的TD儿童和74%的DS参与者能够采取捷径并使用从未走过的轨迹，这是认知地图构建能力的标志。相比之下，仅有一名WS参与者表现出构建认知地图的能力。这些发现与以下观点一致：WS患者的海马体依赖性空间学习能力受到严重影响，而DS患者的这一能力相对保留。