Abstract
One of the most compelling features of Williams syndrome (WS) is the widely reported excessive sociability, accompanied by a relative proficiency in expressive language, which stands in stark contrast with significant intellectual and nonverbal impairments. It has been proposed that the unique language skills observed in WS are implicated in the strong drive to interact and communicate with others, which has been widely documented in WS. Nevertheless, this proposition has yet to be empirically examined. The present study aimed at investigating the relationship between a brain index of language processing and judgments of approachability of faces, as a proxy for sociability, in individuals with WS as contrasted to typical controls. Results revealed a significant and substantial association between the two in the WS, but not in the control group, supporting the hitherto untested notion that language use in WS might be uniquely related to their excessive social drive.
Highlights
Williams syndrome (WS) is a rare neurodevelopmental disorder caused by a hemizygous deletion of 25–30 genes on chromosome band 7q11.23 (Ewart et al 1993) and associated with atypical social and cognitive profiles
This study represents a first known-to-us attempt to examine the relationship between language and sociability in a syndrome characterized by unusual social interest and surprisingly proficient expressive language, the latter standing out in a cognitive profile characterized by overall intellectual disability and profound impairments in nonverbal, visuospatial functioning
The primary intent of the study was to assess the association between an electrophysiological correlate of language comprehension and approachability ratings, as a proxy for sociability, in individuals with WS as compared with TD controls, operating under the primary hypothesis that a relative proficiency in linguistic skills characterizing WS might be related to their strong drive to interact and communicate with others
Summary
Williams syndrome (WS) is a rare neurodevelopmental disorder caused by a hemizygous deletion of 25–30 genes on chromosome band 7q11.23 (Ewart et al 1993) and associated with atypical social and cognitive profiles. Albeit relatively little systematically investigated aspect of the WS behavioral profile is “sociability,” a hallmark feature described as involving indiscriminate friendliness, enhanced empathy, and loquaciousness and socially engaging language (Doyle et al 2004; Järvinen-Pasley et al 2008; Jones et al 2000; Mervis and Klein-Tasman 2000). The attraction to other people and interest in social interactions are evident even among infants and toddlers (cf Järvinen-Pasley et al 2008; Mervis et al 2003) and persist into adulthood. Overall, notwithstanding the complex cognitive and behavioral profile of WS, excessive sociability and a keen interest in interacting with people are considered to be the most robust behavioral characteristics of WS (cf Järvinen-Pasley et al 2008)
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