DEVELOPMENTAL PROFILES OF WILLIAMS SYNDROME CHILDREN

威廉氏综合征（WMS）是一种基于基因的综合征，在成熟状态下导致认知能力的高峰和低谷。我们调查了WMS儿童中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，并且只有在语法出现时，WMS才显示出比DNS越来越大的优势。我们将研究在线和离线的措施，语言和其他领域之间的集中和分离，以及话语功能的发展。a)话语中语言与情感的交集。我们调查了发育中的WMS儿童的超情感和语言，这可能使他们与其他中心人群区别开来。b) WMS中语言与空间表征的分离。由于严重的空间认知缺陷，WMS儿童提供了一个特殊的机会来探索语言和空间表征之间的潜力。对比发展：WMS中空间加工与人脸加工的分离。WMS导致空间建构的高度特异性损伤。相比之下，面部加工在不同范式中表现出优势。因此，WMS儿童为研究视觉领域内功能的发育可分离性提供了有力的工具。发育中WMS儿童的脑组织。我们将研究WMS中语言和空间功能的发育方面可能由神经系统介导的假设，这些神经系统在主要方面与正常人群不同。通过电生理和功能性MRI研究，我们还假设在WMS中，与腹侧视觉处理流相反，背侧可能存在不同的功能障碍。比较发展中的WMS儿童和其他中心人群的概况可以提供对高级皮质功能的遗传基础问题的见解。

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.