DEVELOPMENTAL PROFILES OF WILLIAMS SYNDROME CHILDREN

我们研究的长期目标是了解 语言和其他认知功能的发展。 我们一直 调查威廉姆斯综合征，一种罕见的遗传性疾病， 导致智力迟钝。 我们发现，这种综合征还 导致认知功能的特定分离，无论是在内部还是 跨领域;（a）巨大的认知缺陷，但相当大的节省 （B）在空间上的极端障碍 但面部处理能力很强。 此外，我们的研究发现， 威廉姆斯综合征在大脑皮层上留下了独特的形态学印记， 大脑，表现在神经生理学和神经解剖学研究结果。 语言和其他认知领域发展的对比。 在 在这里提出的研究中，我们将研究 5-12岁威廉姆斯综合征儿童语言和其他认知领域 年 目的是描述和比较 个体认知域及其潜在的神经基础。 研究 威廉姆斯综合征儿童的发展轨迹可以提供一个 认知领域与脑关系的新视角 organization. 虽然语言相对较少（特别是 句法）在青少年中，他们的语言并非没有异常 (e.g.,不寻常的语义组织的迹象）。 也有 持续存在的形态学错误。 在发展中，也有 异常：威廉姆斯综合征幼儿表现出巨大的延迟， 语言里程碑 我们目前的发现是至少在某些方面 威廉姆斯综合征患者的语言能力直到10岁才真正开始活跃 几岁左右。 研究的目的之一是探索 这种选择性恢复的性质，并找到延迟之间的联系， 以及后来出现的异常现象。 交叉口 发展中的话语情感与语言。 威廉姆斯综合征 青少年大量使用情感语言手段， 话语，并表现出对社会交往的浓厚兴趣。 初步 对威廉姆斯综合征儿童的观察表明， 在语言出现之前，可能会出现一个轮廓。 我们将调查 语言和情感在这种综合症发展中的联系。 威廉姆斯综合征患者的视觉认知： 发展 威廉姆斯综合征导致高度特异性分离 在空间认知中，人们有选择地关注物体的细节， 以牺牲整体为代价。 相比之下， 在不同的实验模式中显示出了显著的保存性。 因此，在本发明中， 威廉姆斯综合征可以为研究 内部以及跨部门的功能的发展可分离性， 认知领域 发展中的威廉姆斯综合征的脑组织 孩子 我们对儿童和青少年威廉姆斯综合征的研究 表明语言和其他认知功能的各个方面可能 由神经系统介导，这些神经系统在主要方面不同于在 正常人群。 我们将研究大脑组织， 行为和神经生理学的综合测量。 研究 这种罕见疾病的发展与其他疾病形成了重大对比。 在中心研究的人群。 这些研究还将提供关键的 为家庭、医生和教育工作者提供信息。

The long-term goal of our research is to understand the biological bases of development of language and other cognitive functions. We have been investigating Williams syndrome, a rare genetically-based disorder that results in mental retardation. We have found that the syndrome also results in specific dissociations in cognitive functions, both within and across domains; (a) massive cognitive deficits but considerable sparing of language in adolescents and adults; and (b) extreme disorders in spatial cognition but excellent face processing. Moreover, our studies are finding that Williams syndrome leaves a distinctive morphological stamp on the brain, manifest in both neurophysiological and neuroanatomical findings. Contrasts in the Development of Language and Other Cognitive Domains. In the studies proposed here, we will examine developmental trajectories of language and other cognitive domains in Williams syndrome children age 5-12 years. The objective is to describe and compare the development of individual cognitive domains and their underlying neural bases. The study of developmental trajectories in Williams syndrome children can provide a new perspective on the relationships between domains of cognition and brain organization. While there is relative sparing of language (particularly syntax) ion the adolescents,, their language is not without anomalies (e.g., signs of unusual semantic organization). There are also morphological errors that persist. Developmentally, there are also anomalies: the Williams syndrome toddlers exhibit massive delays in language milestones. Our finding so far is that at least certain aspects of language in Williams syndrome do not really start flourishing until 10 years of age or so. One of the objectives of the research is to explore the nature of this selective recovery and to find links between the delay in onset in language and the anomalies that later surface. Intersection of Affect and Language in Discourse in Development. Williams syndrome adolescents make abundant usage of affective linguistic devices in discourse and display strong interest in social interaction. Preliminary observations of Williams syndrome children suggest that this affective profile may appear prior to the emergence of language. We will investigate the links between language and affect in development in this syndrome. Visual Based Cognition in Williams Syndrome: Peaks & Valleys in Development. Williams syndrome results in a highly specific dissociation in spatial cognition in which there is selective attention to details of a configuration at the expense of the whole. In contrast, face processing shows remarkable preservation across experimental paradigms. Thus, Williams syndrome can provide a powerful vehicle for investigating the developmental separability of functions within, as well as across, cognitive domains. Brain Organization in the Developing Williams Syndrome Child. Our research with Williams syndrome children and adolescents suggests that aspects of language and other cognitive functions may be mediated by neural systems that differ in major ways from those seen in normal populations. We will investigate the brain organization through combined measures of behavior and neurophysiology. The study of development in this rare disorder provides major contrasts with other populations studied in the Center. These studies will also provide crucial information for families, physicians and educators.