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.
Highlights
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
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 self-selected viewing time (SSVT) and autonomic reactivity measures, but not in explicit ratings
Several studies have used functional magnetic resonance imaging to examine the neural mechanisms underlying the atypical social profile in WS. These studies focused on the amygdala and its regulatory links to other cortical regions implicated in the processing of socially relevant information and in monitoring social behavior (Haas et al 2009; Meyer-Lindenberg et al 2005; MeyerLindenberg et al 2006; Paul et al 2009)
Summary
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. Path analyses of the activation patterns in the two groups showed altered amygdala–prefrontal connectivity in WS, suggesting that abnormalities in the regulatory interactions between the prefrontal cortex and the amygdala could explain the pattern of decreased social fear and increased nonsocial fear reported in people with WS (Meyer-Lindenberg et al 2005; Muñoz et al 2010) This interpretation of decreased responsiveness to or recognition of social threat in WS has been supported by other types of evidence showing autonomic hypoarousal to negative facial expressions (Plesa Skwerer et al 2009) or unusually high ratings of unfamiliar faces on approachability and trustworthiness dimensions (Bellugi et al 1999; Martens et al 2009; Porter et al 2007). In contrast to these autonomic reactivity differences, the WS group did not differ from the LID group in their ability to label the facial emotional expressions, though, as in several other studies (Gagliardi et al 2003; Plesa Skwerer et al 2006a, b; Porter et al 2007), their performance on this explicit task was impaired in comparison to normal controls
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