Abstract
Although individuals with Williams syndrome (WS) typically demonstrate an increased appetitive social drive, their social profile is characterized by dissociations, including socially fearless behavior coupled with anxiousness, and distinct patterns of “peaks and valleys” of ability. The aim of this study was to compare the processing of social and non-social visually and aurally presented affective stimuli, at the levels of behavior and autonomic nervous system (ANS) responsivity, in individuals with WS contrasted with a typically developing (TD) group, with the view of elucidating the highly sociable and emotionally sensitive predisposition noted in WS. Behavioral findings supported previous studies of enhanced competence in processing social over non-social stimuli by individuals with WS; however, the patterns of ANS functioning underlying the behavioral performance revealed a surprising profile previously undocumented in WS. Specifically, increased heart rate (HR) reactivity, and a failure for electrodermal activity to habituate were found in individuals with WS contrasted with the TD group, predominantly in response to visual social affective stimuli. Within the auditory domain, greater arousal linked to variation in heart beat period was observed in relation to music stimuli in individuals with WS. Taken together, the findings suggest that the pattern of ANS response in WS is more complex than previously noted, with increased arousal to face and music stimuli potentially underpinning the heightened behavioral emotionality to such stimuli. The lack of habituation may underlie the increased affiliation and attraction to faces characterizing individuals with WS. Future research directions are suggested.
Highlights
Understanding the underpinnings of human social behavior is of relevance to both typical development as well as neurodevelopmental disorders
The main behavioral result showed that while individuals with Williams syndrome (WS) performed at a comparable level to the typically developing (TD) comparison group in processing emotional faces, they were significantly poorer at identifying affect in non-social images
This result is in line with earlier studies (e.g., Järvinen-Pasley et al, 2010a,b) suggesting that WS is characterized by a bias toward social information manifesting as superior processing of social over nonsocial stimuli
Summary
Understanding the underpinnings of human social behavior is of relevance to both typical development as well as neurodevelopmental disorders. Within this realm, the unusual social phenotype associated with Williams syndrome (WS) has brought this neurogenetic condition to the forefront of interest within the neuroscience community. The social affective characteristics have variably been described in WS for a decade, the role of the underlying autonomic nervous system (ANS) function in the fascinating social phenotype remains largely unknown. This question is of significance for several reasons. Individuals with WS have been described as demonstrating heightened emotional reactivity at the behavioral level (e.g., Tager-Flusberg and Sullivan, 2000; Levitin et al, 2004), and it is currently not understood whether this unusual sensitivity may play a role in regulating the reward www.frontiersin.org
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