Language and Williams Syndrome: How Intact Is "Intact"?

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.

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

“…3 Language delay is usually present, but verbal IQ is generally, although not always, higher than performance IQ in WBS cases. [3][4][5][6][7][8] By contrast, although language delay is common to both disorders, subjects with autism tend to have higher performance IQs than verbal IQs and display excessive social versus non-social anxiety. However, difficulty reading emotions from the face and voice, an inability to get along with peers and poor social judgement are common features of both disorders.…”

Inflectional morphology in cri du chat syndrome – A case study

“…Problems with gender agreement in other atypical populations have been attributed to the fact that the inflected elements are distributed across several words within a noun phrase (cf. Karmiloff-Smith et al, 1997;Orgassa and Weerman, 2008), for example, den store bilen 'the big car', where the agreeing elements are the determiner den, the inflection -e on the adjective store and the inflection -en on bilen, all exponents of masculine gender in Norwegian. One challenge with gender agreement for individuals with limitations in verbalauditory processing is to extract and internalize general information about these distributed patterns from exemplars.…”

“…Nominal domain studies have shown that grammatical gender is a problem for children with SLI (Roulet-Amiot and Jakubowicz, 2006; Orgassa and Weerman, 2008) and with Williams syndrome (Karmiloff-Smith, Grant, Berthoud, Davies, Howlin, and Udwin, 1997). In summary, there are commonalities as well as differences in morphological impairment across types of impairment, across structures and across languages.…”

A Dual Comparative Approach: Integrating Lines of Evidence from Human Evolutionary Neuroanatomy and Neurodevelopmental Disorders

“…Individuals with Williams syndrome additionally display a relatively predictable and consistent cognitive and behavioral phenotype, including global reductions in total IQ, particularly with respect to visuospatial intelligence [Meyer-Lindenberg et al, 2006]. Curiously, individuals with Williams syndrome have been shown to demonstrate linguistic capacity characterized by elaborated vocabulary and unusually expressive use of phrases [Udwin and Yule, 2005; Reilly et al, 2004], but struggle with elements of syntax, grammatical structure, and embedded meaning [Karmiloff-Smith et al, 1997; Brock, 2007]. Among the most salient features of Williams syndrome, affected individuals tend to show an abnormally high drive to engage in social and affiliative behavior, and exhibit an unusual willingness to approach strangers [Doyle et al, 2004; Järvinen-Pasley et al, 2008].…”

“…Deficits in sentence morphology and syntax [Karmiloff-Smith et al, 1997] indicate difficulty in encoding rules of proper language use and grammatical structure [Thomas and Karmiloff-Smith, 2005; Clahsen, 1998]. Given the role of the striatum in recursive elements and rule-encoding in language use and acquisition [Teichmann et al, 2006] these processing abnormalities might be related to differences in striatal microstructure.…”