COLLABORATIVE RESEARCH: Spatial Cognition and Spatial Language in Williams Syndrome

本研究将探讨儿童和成人的空间认知和空间语言，使用罕见的遗传缺陷，威廉姆斯综合征的证据，阐明正常空间认知的性质。 患有威廉姆斯综合征的个体通常具有空间认知严重受损的认知特征以及相对较少的语言。 因此，这种综合征提供了一个独特的机会，了解空间认知和空间语言的性质，它们的发展，以及它们的相互作用。 本研究的目的是确定（a）空间崩溃是否是选择性的，只发生在某些空间能力上，而不发生在其他空间能力上，以及（B）空间语言是否可以在没有与正常空间认知相互作用的情况下正常地获得和使用。 “选择性”的问题将通过四个空间领域的实验来解决，对象表征，空间感知与空间中的视觉手动动作，导航和空间语言。 如果空间认知的某些方面得以保留，而其他方面则没有，这就与空间认知是专门化的以及不同类型的空间能力从不同起源发展的观点相一致。 相反，如果空间认知的所有方面都受到损害，这将与以下观点相一致：存在对各种空间认知产生同等影响的一般发展原则。 空间语言和非语言空间认知之间的“互动”问题将通过两种空间知识之间关系的实验来解决。 如果两者都受到同等程度的损害，这将表明大脑的高度互动组织，其中空间认知和空间语言紧密耦合。 相反，如果空间语言被选择性地保留下来，这将与高度的选择性相一致，并意味着语言发展不需要受到某些类型的认知障碍的影响。 总的来说，这些研究应该阐明正常空间认知的性质，确定发展和其故障是否发生沿着正常的认知结构，这将表明认知专业化。 他们应该通过确定在没有正常的非语言空间认知的情况下空间语言是否能够正常发展来阐明空间语言的性质，这表明两个系统之间具有高度的独立性。 他们应该通过确定一个系统的崩溃是否不可避免地迫使其他系统发生变化，或者发展崩溃是否可以选择性地适用于认知的子系统，来阐明发展过程。

This research will explore spatial cognition and spatial language in children and adults, using evidence from a rare genetic deficit, Williams syndrome, to shed light on the nature of normal spatial cognition. Individuals with Williams syndrome typically have a cognitive profile of profoundly impaired spatial cognition together with relatively spared language. This syndrome therefore provides a unique opportunity to understand the nature of spatial cognition and spatial language, their development, and their interaction. The goal of this research is to determine (a) whether spatial breakdown is selective, occurring for only certain spatial capacities but not others, and (b) whether spatial language can be acquired and used normally in the absence of interaction with normal spatial cognition. The question of "selectivity" will be addressed by experiments in four spatial domains, object representation, perception of space vs. visual-manual action in space, navigation, and spatial language. If some aspects of spatial cognition are spared, but others are not, this would be consistent with the idea that spatial cognition is specialized, and that different kinds of spatial capacities develop from different origins. In contrast, if all aspects of spatial cognition are impaired, this would be consistent with the idea that there are general principles of development that affect all kinds of spatial cognition equally. The question of "interaction" between spatial language and non-linguistic spatial cognition will be addressed by experiments on the relationship between the two kinds of spatial knowledge. If both are equally impaired, this would suggest a highly interactive organization of the brain in which spatial cognition and spatial language are tightly coupled. In contrast, if spatial language is selectively spared, this would be consistent with a high degree of selectivity, and would mean that language development need not be affected by certain kinds of cognitive impairment. As a whole, these studies should elucidate the nature of normal spatial cognition by determining whether development and its breakdown occur along the lines of normal cognitive architecture, which would suggest cognitive specialization. They should elucidate the nature of spatial language by determining whether it can develop normally in the absence of normal non-linguistic spatial cognition, which would suggest a high degree of independence between the two systems. And they should shed light on developmental processes, by determining whether breakdown in one system inevitably forces changes in other systems, or whether developmental breakdown can apply selectively across sub-systems of cognition.