DEVELOPMENTAL PROFILES OF WILLIAMS SYNDROME CHILDREN

威廉姆斯综合症儿童的发育概况

• 批准号: 6618949
• 负责人: URSULA BELLUGI
• 金额: $ 13.47万
• 依托单位: UNIVERSITY OF CALIFORNIA, SAN DIEGO
• 依托单位国家: 美国
• 财政年份: 2002
• 资助国家: 美国
• 起止时间: 2002-08-01 至 2003-07-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6618949
• 关键词: Downs syndrome; Williams syndrome; behavioral /social science research tag; brain mapping; child psychology; cognition disorders; evoked potentials; face expression; functional magnetic resonance imaging; human subject; language development; language disorders; longitudinal human study; memory; middle childhood (6-11); neural information processing; neural plasticity; neuropsychological tests; neuropsychology; patient oriented research; psycholinguistics; psychometrics; social behavior; space perception; syntax; visual perception

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.

威廉综合征（WMS）是一种基于遗传的综合征，可导致成熟状态下认知能力的峰值和山谷的认知特征。我们调查了WMS儿童中这种不寻常的概况的发展，以便为语言，认知和大脑组织的替代理论提供线索。对比WMS中语言和一般认知的发展。重要的是，在第一词的发展中，WMS和唐氏综合症（DNS）儿童同样延迟了，只有语法出现，WMS似乎比DNS表现出越来越多的优势。我们将研究语言与其他领域之间的聚焦和离线措施，以及话语功能的发展。 a）语言的交集并在话语中影响。我们研究了发展WMS儿童的影响力和语言，这可能使他们与其他中心人群区分开来。 b）WMS中语言和空间表示之间的解离。由于其严重的空间认知赤字，WMS儿童为语言和空间表示之间的潜力提供了特殊的机会。对比的发展：WMS中空间和面部处理之间的解离。 WMS导致空间结构的高度特异性损害。相比之下，面部处理显示了跨范式的强度。因此，WMS儿童提供了一种强大的工具，用于研究视觉域内功能的发展性可分离性。患有WMS的发展中心的大脑组织。 我们将研究以下假设：WMS中语言和空间功能的发展方面可能是由神经系统介导的，这些神经系统与正常人群的主要方式不同。我们还假设背侧可能存在差异功能障碍，而不是WMS中视觉处理的腹侧功能障碍，涉及小瓶电生理学和功能性MRI研究。比较开发WMS儿童和其他中心种群的特征可以提供有关较高皮质功能遗传基础问题的见解。