Theory of Mind in Williams Syndrome Assessed Using a Nonverbal Task

Porter, Melanie; Coltheart, Max; Langdon, Robyn

doi:10.1007/s10803-007-0447-4

Cited by 56 publications

(51 citation statements)

References 25 publications

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“…These findings are, to some extent, in line with those of a recent study using a TOM visual task and showing impaired understanding of false-beliefs, but MAappropriate performance on social script and mechanical stories, in a subgroup of individuals with WS (Porter et al 2008). However, findings of this study contrast with those of previous studies showing that adults with WS are relatively proficient at reading mental state information from the eyes region, i.e.…”

Section: Discussionsupporting

confidence: 87%

Verbal Peaks and Visual Valleys in Theory of Mind Ability in Williams Syndrome

Santos

Deruelle

2008

J Autism Dev Disord

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Research on theory of mind (TOM) has provided a major contribution to the understanding of developmental disorders characterized by atypical social behaviour. Yet, there is still little consensus relative to TOM abilities in Williams syndrome (WS). This study used visual and verbal tasks to investigate attribution of intentions in individuals with WS relative to mental age-matched typically developing individuals. Results showed that individuals with WS perform as accurately as controls on the verbal but not on the visual task. Such modality differences did not affect WS group's performance on a control condition not requiring TOM neither were found for the control group. These results suggest the existence of a verbal peak and a visual valley in TOM ability in WS.

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Section: Discussionsupporting

confidence: 87%

Verbal Peaks and Visual Valleys in Theory of Mind Ability in Williams Syndrome

Santos

Deruelle

2008

J Autism Dev Disord

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“…WBS individuals are also very friendly, gregarious and empathic, with relatively good emotion recognition abilities. In contrast to their good social perceptual skills, their social reasoning capabilities, such as theory of mind are impaired [5], [6]. They display poor impulse control (behavioural disinhibition), a deficit which may underlie their apparent overfriendly personalities and tendency to approach strangers [7], [8].…”

Section: Introductionmentioning

confidence: 99%

A Role for Transcription Factor GTF2IRD2 in Executive Function in Williams-Beuren Syndrome

et al. 2012

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Executive functions are amongst the most heritable cognitive traits with twin studies indicating a strong genetic origin. However genes associated with this domain are unknown. Our research into the neurodevelopmental disorder Williams-Beuren syndrome (WBS) has identified a gene within the causative recurrent 1.5/1.6 Mb heterozygous microdeletion on chromosome 7q11.23, which may be involved in executive functioning. Comparative genome array screening of 55 WBS patients revealed a larger ∼1.8 Mb microdeletion in 18% of cases, which results in the loss of an additional gene, the transcription factor GTF2IRD2. The GTF gene family of transcription factors (GTF2I, GTF2IRD1 and GTF2IRD2) are all highly expressed in the brain, and GTF2I and GTF2IRD1 are involved in the pathogenesis of the cognitive and behavioural phenotypes associated with WBS. A multi-level analysis of cognitive, behavioural and psychological functioning in WBS patients showed that those with slightly larger deletions encompassing GTF2IRD2 were significantly more cognitively impaired in the areas of spatial functioning, social reasoning, and cognitive flexibility (a form of executive functioning). They also displayed significantly more obsessions and externalizing behaviours, a likely manifestation of poor cognitive flexibility and executive dysfunction. We provide the first evidence for a role for GTF2IRD2 in higher-level (executive functioning) abilities and highlight the importance of integrating detailed molecular characterisation of patients with comprehensive neuropsychological profiling to uncover additional genotype-phenotype correlations. The identification of specific genes which contribute to executive function has important neuropsychological implications in the treatment of patients with conditions like WBS, and will allow further studies into their mechanism of action.

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“…Although there is substantial agreement in the field regarding the “hypersociability” of individuals with WS, the neurocognitive mechanisms underlying this striking profile have remained elusive. To date, most experimental studies tapping the social phenotype of WS have focused on explicit responses to stimuli, such as accuracy in face recognition (Bellugi et al 1994; Rose et al 2007; Tager-Flusberg et al 2003), identifying emotional expressions (Gagliardi et al 2003; Plesa Skwerer et al 2006a, b; Porter et al 2007), and direction of gaze (Mobbs et al 2004) or performance on tasks probing social cognition (John and Mervis 2010; Porter et al 2008; Tager-Flusberg and Sullivan 2000). …”

mentioning

confidence: 99%

A multimeasure approach to investigating affective appraisal of social information in Williams syndrome

Skwerer

Ammerman

André

et al. 2011

J Neurodevelop Disord

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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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Theory of Mind in Williams Syndrome Assessed Using a Nonverbal Task

Cited by 56 publications

References 25 publications

Verbal Peaks and Visual Valleys in Theory of Mind Ability in Williams Syndrome

Verbal Peaks and Visual Valleys in Theory of Mind Ability in Williams Syndrome

A Role for Transcription Factor GTF2IRD2 in Executive Function in Williams-Beuren Syndrome

A multimeasure approach to investigating affective appraisal of social information in Williams syndrome

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