The interplay between anxiety and social functioning in Williams syndrome.

The developmental disorder Williams syndrome (WS) has been associated with an atypical social profile of hyper-sociability and heightened social sensitivity across the developmental spectrum. In addition, previous research suggests that both children and adults with WS have a predisposition towards anxiety. The current research aimed to explore the profiles of social behaviour and anxiety across a broad age range of individuals with the disorder (n = 59, ages 6-36 years). We used insights from parental reports on two frequently used measures, the Spence Children's Anxiety Scale (SCAS-P) and the Social Responsiveness Scale (SRS). Severity of anxiety was correlated with a greater degree of social dysfunction as measured by the SRS in this group. We split the group according to high or low anxiety as measured by the SCAS-P and explored the profile of social skills for the two groups. Individuals high and low in anxiety differed in their social abilities. The results emphasise the need to address anxiety issues in this disorder and to consider how components of anxiety might relate to other features of the disorder.

Heterogeneity of social approach behaviour in Williams syndrome: the role of response inhibition.

The developmental disorder of Williams syndrome (WS) is associated with an overfriendly personality type, including an increased tendency to approach strangers. This atypical social approach behaviour (SAB) has been linked to two potential theories: the amygdala hypothesis and the frontal lobe hypothesis. The current study aimed to investigate heterogeneity of SAB in WS by exploring whether subgroups of SAB profiles could be identified using cluster analytic techniques. Twenty-five children with WS aged 6-15 years completed three behavioural tasks tapping (i) social approach behaviour, (ii) emotion recognition ability and (iii) response inhibition. Cluster analyses revealed preliminary evidence of WS subgroups based on SAB profiles and indicated that response inhibition ability was the key differentiating variable between SAB cluster profiles. The findings provide tentative support for the frontal lobe hypothesis of SAB in WS and highlight the importance of investigating SAB at a heterogeneous level.

Brief report: exploring the relationship between sensory processing and repetitive behaviours in Williams Syndrome.

This study explored the relationship between sensory processing abnormalities and repetitive behaviours in children with Williams Syndrome (WS; n = 21). This is a novel investigation bringing together two clinical phenomena for the first time in this neuro-developmental disorder. Parents completed the Sensory Profile (Short Form; Dunn in The sensory profile manual. San Antonio: The Psychological Corporation, 1999) and the Repetitive Behaviour Questionnaire (Turner 1995). A significant correlation was evident between the total scores on each of these measures; suggesting that children with WS who exhibit increased sensory processing abnormalities also display a higher number of repetitive behaviours. Further exploratory analyses of subscales of the measures indicated potentially important relationships that suggest a role for arousal regulation in the relationship between sensory processing abnormalities and repetitive behaviours in WS.

Anxiety and repetitive behaviours in autism spectrum disorders and williams syndrome: a cross-syndrome comparison.

Children with autism spectrum disorder or Williams syndrome are vulnerable to anxiety. The factors that contribute to this risk remain unclear. This study compared anxiety in autism spectrum disorder and Williams syndrome and examined the relationship between repetitive behaviours and anxiety. Thirty-four children with autism and twenty children with Williams syndrome were assessed with measures of anxiety and repetitive behaviours. Children with autism had higher levels of anxiety. Within the autism sample higher levels of repetitive behaviours were associated with more anxiety. This was not replicated in the Williams syndrome sample, indicating a differential role for restricted and repetitive behaviours in relation to anxiety. Understanding the links between repetitive behaviours and anxiety is essential for effective intervention.