Literature DB >> 9180000

Language and Williams syndrome: how intact is "intact"?

A Karmiloff-Smith1, J Grant, I Berthoud, M Davies, P Howlin, O Udwin.   

Abstract

It has been claimed that Williams syndrome (WS), a rare neurodevelopmental disorder, is characterized by serious cognitive deficits alongside intact language. The syndrome is often used as a prime example of the modularity of an innate faculty for morphosyntactic rules. We challenge this claim and hypothesize that morphosyntax, although surprisingly good given WS level of mental retardation, is by no means intact. We make an initial test of this hypothesis through an analysis of the receptive language of a group of English-speaking WS individuals on a standardized morphosyntactic test. We then present an experimental study of expressive language that examines grammatical gender assignment in French-speaking WS patients. Despite a Verbal Mental Age selected to be higher than the chronological age of the young control group, these people with WS continue even in adulthood to show clear-cut deficits in their production of an aspect of morphosyntax that normal children acquire effortlessly very early. The results of the 2 studies, one focusing on receptive language and the other on expressive language, challenge the notion that comprehension and use of morphosyntactic rules in WS individuals are intact. The Within-domain dissociations regarding the use of grammatical gender assignment across several sentence clements and their difficulties in understanding embedded sentences-two quintessentially linguistic skills-suggest that we must rethink the notion of spared, modular, language capacities in Williams syndrome. We conclude that WS language follows a different path to normal acquisition and may turn out to be more like second language learning.

Entities:  

Mesh:

Year:  1997        PMID: 9180000

Source DB:  PubMed          Journal:  Child Dev        ISSN: 0009-3920


  32 in total

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Review 3.  Cognitive and behavioral characteristics of children with Williams syndrome: implications for intervention approaches.

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5.  An atypical deletion of the Williams-Beuren syndrome interval implicates genes associated with defective visuospatial processing and autism.

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Review 6.  Language phenotypes and intervention planning: bridging research and practice.

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7.  MRI assessment of superior temporal gyrus in Williams syndrome.

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Journal:  Cogn Behav Neurol       Date:  2008-09       Impact factor: 1.600

8.  Using novel control groups to dissect the amygdala's role in Williams syndrome.

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9.  The two-word stage: motivated by linguistic or cognitive constraints?

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Review 10.  Space and language in Williams syndrome: insights from typical development.

Authors:  Barbara Landau; Katrina Ferrara
Journal:  Wiley Interdiscip Rev Cogn Sci       Date:  2013-09-30
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