DEVELOPMENTAL PROFILES OF WILLIAMS SYNDROME CHILDREN

威廉氏综合征（WMS）是一种基于遗传的综合征，其导致成熟状态下认知能力的高峰和低谷的认知概况。我们调查了WMS儿童这种不寻常的特征的发展，以便为语言，认知和大脑组织的替代理论提供线索。WMS中语言与一般认知发展的对比。重要的是，在第一个单词的发展中，WMS和唐氏综合症（DNS）儿童同样延迟，只有当语法出现时，WMS才表现出比DNS更大的优势。我们将考察在线和离线测量语言和其他领域之间的关注和分离，以及话语功能的发展。（1）话语中语言与情感的交叉。我们调查发展中的WMS儿童的情感亢进和语言，这可能使他们有别于其他中心人群。B）WMS中语言与空间表征的分离。由于他们严重的空间认知缺陷，WMS儿童提供了一个特殊的机会，探索语言和空间表征之间的潜力。对比发展：WMS中空间与面孔处理的分离。WMS导致空间结构的高度特异性损伤。相比之下，面对处理显示跨范式的力量。因此，WMS儿童为研究视觉领域内功能的发育分离性提供了一个强大的工具。脑组织在发展中的儿童与WMS。 我们将研究的假设，发展方面的语言和空间功能的WMS可能介导的神经系统，在主要方面不同于正常人群。我们还假设，可能有差异功能障碍，在背侧，而不是在WMS的视觉处理腹侧流，解决小瓶电生理和功能性MRI研究。发展中的WMS儿童和其他中心人群的配置文件的比较，可以提供更高的皮质功能的遗传基础的问题的见解。

William's syndrome (WMS) is a genetically based syndrome that results in a cognitive profile of peaks and valleys of cognitive abilities in the mature state. We investigate the development of this unusual profile in WMS Children in order to provide clues to alternative theories of language, cognition, and brain organization. Contrasting Development of Language and General Cognition in WMS. Importantly, in the development of first words, WMS and Down syndrome (DNS) children are equally delayed, and it is only as grammar emerges that WMS appear to show an increasing advantage over DNS. We will examine both on- line and off-line measures focusing and dissociations between language and other domains, as well as the development of discourse functions. a) The Intersection of Language and Affect in Discourse. We investigate hyperaffectivity and language in developing WMS children, which may set them apart from other Center populations. b) The Dissociation between Language and Spatial Representation in WMS. Because of their severe spatial cognitive deficit, WMS children provide a special opportunity to probe potential between language and spatial representations. Contrasting Development: The Dissociation between Space and Face Processing in WMS. WMS results in a highly specific impairment in spatial construction. In contrast, face processing shows strength across paradigms. Thus, WMS children provide a powerful vehicle for investigating the developmental separability of functions within the visual domain. Brain Organization in the Developing Child with WMS. We will examine the hypothesis that developmental aspects of language and spatial functions in WMS may be mediated by neural systems that differ in major ways from those in normal populations. We also hypothesize that there may be differential dysfunction in the dorsal as opposed to the ventral stream of visual processing in WMS, addressed vial electrophysiological and functional MRI studies. Comparison of the profiles of developing WMS Children and other Center populations can provide insights into issues of the genetic bases of higher cortical functions.