Language and Williams syndrome: how intact is "intact"?
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.
Karmiloff-Smith
A
A
MRC-CDU, London, U.K. annette@cdu.ucl.ac.uk
Grant
J
J
Berthoud
I
I
Davies
M
M
Howlin
P
P
Udwin
O
O
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis / Q000523:psychology; D007805:Language Development Disorders / Q000175:diagnosis* / Q000523:psychology; D007807:Language Tests; D008297:Male; D010364:Pattern Recognition, Visual; D012016:Reference Values; D012660:Semantics; D014825:Vocabulary; D018980:Williams Syndrome / Q000175:diagnosis* / Q000523:psychology