Sensitivity of the autonomic nervous system to visual and auditory affect across social and non-social domains in Williams syndrome

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.

Social Cognition in Williams Syndrome: Face Tuning

“…Williams–Beuren syndrome individuals possess a hypersocial personality profile, commonly referred to as “cocktail party” style, that is manifested as a friendly, but often exaggeratedly friendly, appetitive drive for social interaction with other people and social closeness, with enhanced emotionality and face processing, and a fondness for music ( Jones et al, 2000 ; Tager-Flusberg et al, 2003 ; Järvinen et al, 2013 ). The pattern of the autonomic nervous system response in WS is complex, with increased arousal and lack of habituation to faces ( Järvinen et al, 2012 ). The friendly attitude of those with WS tends to extend to unfamiliar people, leading parents of children with WS to worry about their children’s abnormal tendency to seek out and engage strangers ( Gagliardi et al, 2003 ; Järvinen et al, 2015 ).…”

Social functioning and autonomic nervous system sensitivity across vocal and musical emotion in Williams syndrome and autism spectrum disorder

“…Individuals with Williams syndrome (WS) and autism spectrum disorder (ASD) typically experience difficulties in interpreting others' emotional nonverbal displays, which have been linked to abnormalities in social behavior (Braverman, Fein, Lucci, & Waterhouse, 1989;J€ arvinen-Pasley et al, 2010a). WS stems from a hemi-deletion of 25-28 genes on chromosome 7 (Ewart et al, 1993;Hillier et al, 2003), and results in increased social drive, low social anxiety, and bias toward social over nonsocial information (J€ arvinen, Korenberg, & Bellugi, 2013;J€ arvinen-Pasley et al, 2008a;Martens, Wilson, & Reutens, 2008;Riby & Hancock, 2008, 2009.…”

Anxiety and autonomic response to social-affective stimuli in individuals with Williams syndrome

“…Namely, unique to individuals with WS, greater anxiety was associated with diminished autonomic arousal to angry social stimuli. These findings provide initial clues linking the social and anxiety phenotypes of WS, suggesting that aberrations in the ANS that have been shown to underlie anomalous emotion perception (Järvinen et al, 2012) and social dysfunction ) may also be linked to increased anxiety (Ng et al, 2014) in this population. *Note.…”

“…Mean inter-beat interval (IBI) was calculated from the raw ECG signal to assess differences in heartbeat across social-affective conditions relative to baseline level. For more detailed information regarding psychophysiological recordings, please see Järvinen et al (2012;2015a-d), as ANS analyses were replicated.…”

“…Recent evidence indicates that individuals with WS exhibit atypical autonomic nervous system (ANS) functioning in response to emotional stimuli (Järvinen et al, 2012Plesa Skwerer et al, 2009;Riby et al, 2012; for a review, see . Autonomic reactivity, as indexed by measures such as electrodermal (EDA) responses and heart rate, reflect sympathetic activation for fight-or-flight behaviours when in fear, and/or parasympathetic activity for resting responses when relaxed (Herman et al, 2005).…”