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5 - Language development in children with Williams syndrome: New insights from cross-linguistic research

Published online by Cambridge University Press:  04 August 2010

By
Stavroula Stavrakaki
Edited by
Marcia A. Barnes
Marcia A. Barnes
Affiliation:
University of Texas Health Science Center, Houston
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Summary

Introduction

Williams syndrome (WS) is a genetically based neurodevelopmental disorder, which is caused by a microdeletion of chromosome 7, more specifically, at the region of chromosome 7q11.23 (Doll & Grzeschik, 2001; Ewart et al., 1993; Korenberg et al., 2000). Due to its uneven cognitive profile, WS has recently been the focus of scientific research in the field of developmental cognitive neuroscience (Clahsen & Almazan, 1998; Clahsen & Temple, 2003; Jordan et al., 2002; Thomas et al., 2001). Impaired visuo-spatial cognition, planning, and problem solving co-occur with relatively spared abilities in the domain of language, social cognition, and face processing (Bellugi et al., 1988; Karmiloff-Smith, 1998; Karmiloff-Smith et al., 1997; Mervis et al., 2000; Tager-Flusberg et al., 2003; Tager-Flusberg & Sullivan, 2000).

There is much controversy surrounding the status and development of the relatively spared cognitive abilities in WS, especially language. More specifically, some researchers argue that language development in WS (and other developmental disorders) reflects the abnormal development of the entire cognitive system (Karmiloff-Smith, 1998; Thomas et al., 2001; Thomas & Karmiloff-Smith, 2002). Consequently, the developmental pattern in WS is expected to be qualitatively different from typical development. If this is the case, then there should not be selective preservation of cognitive abilities in WS while the rest of the system develops abnormally. In other words, there should be no evidence for “residual normality” in WS (Thomas & Karmiloff-Smith, 2002).

Type
Chapter
Information
Genes, Brain and Development
The Neurocognition of Genetic Disorders
 , pp. 105 - 121
Publisher: Cambridge University Press
Print publication year: 2010

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