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1.
J Child Lang ; 40(1): 266-89, 2013 Jan.
Article in English | MEDLINE | ID: mdl-22883814

ABSTRACT

Research on language in individuals with Williams syndrome (WS) has been fueled by persistent theoretical controversies for two decades. These shifted from initial focus on dissociations between language and cognition functions, to examining the paradox of socio-communicative impairments despite high sociability and relatively proficient expressive language. We investigated possible sources of communicative difficulties in WS in a collaborative referential communication game. Five- to thirteen-year-old children with WS were compared to verbal mental age- and to chronological age-matched typically developing children in their ability to consider different types of information to select a speaker's intended referent from an array of items. Significant group differences in attention deployment to object locations, and in the number and types of clarification requests, indicated the use of less efficient and less mature strategies for reference resolution in WS than expected based on mental age, despite learning effects similar to those of the comparison groups, shown as the game progressed.


Subject(s)
Communication , Williams Syndrome/psychology , Attention , Child , Child, Preschool , Cognition , Female , Games, Experimental , Humans , Language , Male , Speech
2.
J Neurodev Disord ; 3(4): 325-34, 2011 Dec.
Article in English | MEDLINE | ID: mdl-22081426

ABSTRACT

People with Williams syndrome (WS) have been consistently described as showing heightened sociability, gregariousness, and interest in people, in conjunction with an uneven cognitive profile and mild to moderate intellectual or learning disability. To explore the mechanisms underlying this unusual social-behavioral phenotype, we investigated whether individuals with WS show an atypical appraisal style and autonomic responsiveness to emotionally laden images with social or nonsocial content. Adolescents and adults with WS were compared to chronological age-matched and nonverbal mental age-matched groups in their responses to positive and negative images with or without social content, using measures of self-selected viewing time (SSVT), autonomic arousal reflected in pupil dilation measures, and likeability ratings. The participants with WS looked significantly longer at the social images compared to images without social content and had reduced arousal to the negative social images compared to the control groups. In contrast to the comparison groups, the explicit ratings of likeability in the WS group did not correlate with their SSVT; instead, they reflected an appraisal style of more extreme ratings. This distinctive pattern of viewing interest, likeability ratings, and autonomic arousal to images with social content in the WS group suggests that their heightened social drive may be related to atypical functioning of reward-related brain systems reflected in SSVT and autonomic reactivity measures, but not in explicit ratings.

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