Department of Psychology, University Sapienza of Rome, Rome, Italy.
Res Dev Disabil. 2011 May-Jun;32(3):972-85. doi: 10.1016/j.ridd.2011.02.001. Epub 2011 Feb 24.
This study aimed to evaluate spatial function in subjects with Williams syndrome (WS) by using a large-scale task with multiple rewards and comparing the spatial abilities of WS subjects with those of mental age-matched control children. In the present spatial task, WS participants had to explore an open space to search nine rewards placed in buckets arranged according to three spatial configurations: a cross, a 3 × 3 matrix and a cluster composed by three groups of three buckets each. The findings demonstrate that WS individuals were impaired in efficiently exploring the environment and in building cognitive spatial maps. In exploring the three spatial configurations, they performed worse than control subjects on all parameters analyzed. In fact, WS individuals took more time to complete the task, made more errors, performed a reduced number of error-free trials, displayed lower search efficiency, exhibited shorter spatial spans, showed a higher number of no-visits and displayed marked tendencies to perseverate and to neglect some buckets. Furthermore, WS individuals showed disorganized explorative patterns in comparison to control children. WS influenced performances differentially as a specific effect of the susceptibility of the configurations to being explored in a principled way. In the cross configuration that had strong spatial constraints, both groups exhibited their worst performances. In the matrix configuration, the altered explorative strategies of the WS subjects primarily affected their central exploration. The performances in the cluster configuration indicated that chunking was a strategy of strength in both TD and WS groups. In conclusion, WS individuals' deficits exhibited in the present explorative test may be considered an index of their difficulties in spatial orientation and motion perception displayed in the real world. The marked impairment in spatial information processing is discussed in neuro-anatomical alterations reported in WS.
本研究旨在通过使用具有多种奖励的大规模任务来评估威廉姆斯综合征（WS）患者的空间功能，并将 WS 患者的空间能力与同龄心理匹配的对照组儿童进行比较。在目前的空间任务中，WS 参与者必须探索一个开放空间，以搜索放置在根据三个空间配置排列的桶中的九个奖励：十字形、3x3 矩阵和由三组每组三个桶组成的集群。研究结果表明，WS 个体在有效地探索环境和构建认知空间地图方面存在障碍。在探索这三种空间配置时，他们在所有分析的参数上都比对照组表现更差。事实上，WS 个体完成任务需要更多的时间，犯更多的错误，完成无错误的试次更少，搜索效率更低，空间跨度更短，无访问次数更多，并且表现出明显的坚持和忽视某些桶的倾向。此外，WS 个体的探索模式与对照组儿童相比表现出混乱。WS 对表现的影响是特定于配置易于以有原则的方式探索的敏感性的特定影响。在具有强烈空间约束的十字形配置中，两组都表现出最差的表现。在矩阵配置中，WS 患者改变的探索策略主要影响了他们的中心探索。集群配置中的表现表明，在 TD 和 WS 组中，分块都是一种优势策略。总之，WS 个体在本探索性测试中表现出的缺陷可被视为其在现实世界中空间定向和运动感知困难的指标。在 WS 中报告的神经解剖学改变中讨论了对空间信息处理的明显损害。
