Williams syndrome hypersociability: A neuropsychological study of the amygdala and prefrontal cortex hypotheses

Abstract

Individuals with Williams syndrome display indiscriminate approach towards strangers. Neuroimaging studies conducted so far have linked this social profile to structural and/or functional abnormalities in WS amygdala and prefrontal cortex. In this study, the neuropsychological hypotheses of amygdala and prefrontal cortex involvement in WS hypersociability was explored using three behavioral tasks--facial emotional recognition task, a social approach task and a go no/go task. Thus, a group 15 individuals with Williams syndrome was compared to two groups of normal developing individuals--a group of 15 individuals matched for chronological age (CA) and 15 individuals matched for mental age (MA), and sex. Individuals with WS present a specific impairment in recognizing negative facial expressions and do not display impairments in response inhibition when compared with typically developing groups. Although these findings partially support the amygdala contribution to WS hypersociability, we found that general cognitive functioning predicted this performance. Additionally, individuals with WS did not differ from both CA and MA groups in the recognition of angry facial expressions, a finding suggesting that they are actually able to identify stimuli associated with social threat. Overall, the results seem to indicate that this social profile must be understood within a developmental framework.

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

“…The results support previous findings with regard to social approach behaviour in WS (Bellugi et al, 1999;Martens et al, 2009;Capitao et al, 2011) …”

Continuous Cognitive Dynamics of the Evaluation of Trustworthiness in Williams Syndrome

“…These results may be associated with the fact that the facial expression on the trustworthy faces showed no teeth and no definitive smile, and therefore may have appeared more neutral than happy (see Figure 1D). Previous results have shown that individuals with WS rate neutral faces as “medium approachable” (Capitão et al, 2011). These findings might also reflect the previous finding that individuals with WS have more difficulty than controls in interpreting facial expressions (Gagliardi et al, 2003; Plesa-Skwerer et al, 2006; Porter et al, 2007; Capitão et al, 2011), and have atypical eye scanpath patterns when viewing both positive and negative facial expressions (Porter et al, 2010).…”

“…These variations resulted in a total of 80 facial stimuli, creating a 2-by-2 design (trustworthiness by extremity). These stimuli were chosen because they lack many irrelevant features of more naturalistic face images (e.g., hair or accessories) that can distract individuals with WS and interfere with their attention to facial expressions (Martens et al, 2009; Capitão et al, 2011). …”

Editorial: Cognitive science approach to developmental disorders: From “discrete diagnostic” to “dimensional”

“…While people with ASD have difficulties with communication, people with Williams syndrome (WS) exhibit hypersociability (Bellugi, Adolphs, Cassady, & Chiles, 1999;Capitao, Sampaio, Fernandez, Sousa, Pinheiro, & Gibcalves, 2011). These conditions have opposite characteristic traits of communication (Asada & Itakura, 2012).…”