زبان داستانی در سندرم ویلیامز و ارتباط عصب روانشناسی آن

The cognitive profile of individuals with Williams' Syndrome (WS) shows peaks and troughs, with fairly good linguistic performance and a well described weakness in visual-spatial abilities. This study aims to describe in detail the narrative abilities of a group of 9 WS participants who underwent careful cognitive evaluation to assess their visual-spatial abilities, sustained attention, phonological short-term memory and lexical as well as grammatical skills in tests of expressive and receptive language. Furthermore, they performed a picture-description task in order to elicit more fluid and communicative speech samples, which were then compared with those provided by a group of 29 children with typically developing language (TD) matched for mental age. The WS participants showed visual-spatial deficits but scored within the normal range, according to their mental age, in the linguistic assessment. For the narrative task, they showed good phonological, lexical and syntactic skills, but their story descriptions were less effective than those produced by the TD group on measures assessing global coherence and lexical informativeness, showing dissociation between macro and microlinguistic abilities. These impairments were not correlated to the visual-spatial disturbances. These data suggest that the domain of discourse processing is a relative weakness in WS individuals.