Department of Functioning Science, Institute for Developmental Research, Aichi Human Service Center Aichi, Japan.
Department of Pediatrics, Central Hospital, Aichi Human Service Center Aichi, Japan.
Front Hum Neurosci. 2013 Dec 11;7:856. doi: 10.3389/fnhum.2013.00856. eCollection 2013.
Williams syndrome (WS) is a genetic disorder caused by the partial deletion of chromosome 7. Individuals with WS have atypical cognitive abilities, such as hypersociability and compromised visuospatial cognition, although the mechanisms underlying these deficits, as well as the relationship between them, remain unclear. Here, we assessed performance in mental rotation (MR) and level 2 visual perspective taking (VPT2) tasks in individuals with and without WS. Individuals with WS obtained lower scores in the VPT2 task than in the MR task. These individuals also performed poorly on both the MR and VPT2 tasks compared with members of a control group. For the individuals in the control group, performance scores improved during development for both tasks, while the scores of those in the WS group improved only in the MR task, and not the VPT2 task. Therefore, we conducted a second experiment to explore the specific cognitive challenges faced by people with WS in the VPT2 task. In addition to asking participants to change their physical location (self-motion), we also asked them to adopt a third-person perspective by imagining that they had moved to a specified location (self-motion imagery). This enabled us to assess their ability to simulate the movement of their own bodies. The performance in the control group improved in both the self-motion and self-motion imagery tasks and both performances were correlated with verbal mental age. However, we did not find any developmental changes in performance for either task in the WS group. Performance scores for the self-motion imagery task in the WS group were low, similar to the scores observed for the VPT2 in this population. These results suggest that MR and VPT2 tasks involve different processes, and that these processes develop differently in people with WS. Moreover, difficulty completing VPT2 tasks may be partly because of an inability of people with WS to accurately simulate mental body motion.
威廉姆斯综合征（WS）是一种由染色体 7 部分缺失引起的遗传疾病。WS 患者具有非典型的认知能力，例如社交过度和视觉空间认知受损，尽管这些缺陷的机制以及它们之间的关系仍不清楚。在这里，我们评估了 WS 患者和非 WS 患者在心理旋转（MR）和二级视觉透视任务（VPT2）中的表现。WS 患者在 VPT2 任务中的得分低于 MR 任务。与对照组相比，这些患者在 MR 和 VPT2 任务中的表现都很差。对于对照组中的个体，两种任务的表现得分都随着发育而提高，而 WS 组的得分仅在 MR 任务中提高，而在 VPT2 任务中则没有提高。因此，我们进行了第二项实验，以探索 WS 患者在 VPT2 任务中面临的特定认知挑战。除了要求参与者改变他们的身体位置（自我运动）之外，我们还要求他们通过想象自己已经移动到指定位置（自我运动想象）来采用第三人称视角。这使我们能够评估他们模拟自己身体运动的能力。对照组在自我运动和自我运动想象任务中的表现都有所提高，并且两种表现都与言语心理年龄相关。然而，我们没有发现 WS 组在这两种任务中的任何表现都没有发生发育变化。WS 组的自我运动想象任务表现得分较低，与该人群在 VPT2 中观察到的得分相似。这些结果表明，MR 和 VPT2 任务涉及不同的过程，这些过程在 WS 患者中的发展方式不同。此外，完成 VPT2 任务的困难可能部分是因为 WS 患者无法准确模拟心理身体运动。
