The broad goal of our research program is to understand the biological basis for language and other cognitive functions. In this project, Developmental Profile of Williams Syndrome Children, we address this important issue from a new perspective. Our studies under the Neurodevelopmental Research Center of adolescents with Williams Syndrome (WMS) point to a unique, behavioral profile in this rare metabolic disorder, in which there is a striking fractionation of higher cortical functions: linguistic abilities are selectively preserved in the face of severe general cognitive deficits. Our pilot studies of WMS younger children indicate a different cognitive phenotype showing extreme retardation in all developmental milestones, including language. Almost nothing is known about the initial capacity of WMS; we propose to investigate the development of language and cognition in WMS and matched Down Syndrome (DNS) children between 6 and 11 years of age, in order to provide critical tests of alternative theories of language, cognition and brain organization.
Our specific aims i nclude: The Decoupling of Language from Cognition in the Developing WMS Child. We examine language and cognitive correlates in the young child, to assess the hypothesis that particular linguistic abilities are dependent on the development of specific cognitive functions, or alternatively, that the two domains are dissociable in development. Differential Impairment of Components of Language. Syntactic functions in older WMS are remarkably spared, but semantic organization appears to be deviant; WMS show a proclivity for atypical words. We investigate the basis for this dissociation in the early stages of lexical and grammatical development in WMS. Dissociations Within Domains of Cognition. Even within the domain of spatial cognition, there are clusters of deficits and preservations which may be specific to WMS: markedly impaired visuospatial skills but a remarkable capacity for facial recognition as an island of sparing. WMS may result in selective attention to details of a configuration at the expense of a whole, while DNS results in an opposite profile, leading to hypotheses concerning the basis for the dissociation. Neural Substrate for WMS. These studies will be carried out in conjunction with Neurophysiological and Neuroanatomical studies of brain structure and function, to address specific hypotheses about the neural substrate for this unusual neuropsychological profile. These studies should contribute to our understanding of brain organization for language and cognitive functioning using a specific neurodevelopmental disorder as a model. WMS presents a rare dissociation of language from other cognitive capacities, providing an unusual opportunity to explore some of the central issues of developmental cognitive neuroscience.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Specialized Center (P50)
Project #
5P50NS022343-10
Application #
3738381
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
10
Fiscal Year
1995
Total Cost
Indirect Cost
Name
University of California San Diego
Department
Type
DUNS #
077758407
City
La Jolla
State
CA
Country
United States
Zip Code
92093
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Fan, Chun Chieh; Brown, Timothy T; Bartsch, Hauke et al. (2017) Williams syndrome-specific neuroanatomical profile and its associations with behavioral features. Neuroimage Clin 15:343-347
Ng, Rowena; Brown, Timothy T; Järvinen, Anna M et al. (2016) Structural integrity of the limbic-prefrontal connection: Neuropathological correlates of anxiety in Williams syndrome. Soc Neurosci 11:187-92
Ng, Rowena; Brown, Timothy T; Erhart, Matthew et al. (2016) Morphological differences in the mirror neuron system in Williams syndrome. Soc Neurosci 11:277-88
Lai, Philip T; Reilly, Judy S (2015) Language and affective facial expression in children with perinatal stroke. Brain Lang 147:85-95
Webb, Sara Jane; Bernier, Raphael; Henderson, Heather A et al. (2015) Guidelines and best practices for electrophysiological data collection, analysis and reporting in autism. J Autism Dev Disord 45:425-43
Yousefian, Omid; Ballantyne, Angela O; Doo, Alex et al. (2015) Clock drawing in children with perinatal stroke. Pediatr Neurol 52:592-8
Polse, Lara R; Reilly, Judy S (2015) Orthographic and semantic processing in young readers. J Res Read 38:47-72

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