Abstract

Individuals with an extra X chromosome (Klinefelter syndrome) are at risk for problems in social functioning and have an increased vulnerability for autism traits. In the search for underlying mechanisms driving this increased risk, this study focused on social attention, affective arousal and empathy. Seventeen adults with XXY and 20 non-clinical controls participated in this study. Eyetracking was used to investigate social attention, as expressed in visual scanning patterns in response to the viewing of empathy evoking video clips. Skin conductance levels, reflecting affective arousal, were recorded continuously during the clips as well. Empathic skills, i.e. participants' understanding of own and others' emotions in response to the clips was also assessed. Results showed reduced empathic understanding, decreased visual fixation to the eye region, but increased affective arousal in individuals with Klinefelter syndrome. We conclude that individuals with XXY tend to avoid the eye region. Considering the increased affective arousal, we speculate that this attentional deployment strategy may not be sufficient to successfully downregulate affective hyper-responsivity. As increased affective arousal was related to reduced empathic ability, we hypothesize that own affective responses to social cues play an important role in difficulties in understanding the feelings and intentions of others. This knowledge may help in the identification of risk factors for psychopathology and targets for treatment.

Highlights

  • Klinefelter syndrome (KS) is a chromosome aneuploidy characterized by an additional X chromosome in boys and men (47, XXY), with a prevalence of 1:500 to 1:1000 male births [1]

  • Considering that individuals with autism spectrum disorders (ASD) often show a reduced tendency to focus on the eyes of others, which has been related to impairments in understanding the emotions of others and increased social anxiety [30,31], a study on attention to such social cues in individuals with Klinefelter syndrome is warranted

  • In this study we investigated empathy, social attention, and affective arousal in response to social stimuli in individuals with Klinefelter syndrome (KS)

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Summary

Introduction

Klinefelter syndrome (KS) is a chromosome aneuploidy characterized by an additional X chromosome in boys and men (47, XXY), with a prevalence of 1:500 to 1:1000 male births [1]. In earlier studies using selfreport measures, men with KS reported to be more affectively aroused than controls in response to emotion inducing events [7], and reported to experience higher levels of distress than controls during social interactions, for example when dealing with criticism or when starting a conversation with others [13] These studies on social and emotional processing call for further investigation, considering social dysfunctioning and vulnerability for autism symptoms in individuals with Klinefelter syndrome. In this study we focused on social attention and emotion processing in KS using a neurocognitive approach To this end, we investigated physiological arousal (i.e., skin conductance level) and visual scanning patterns whilst viewing empathy evoking video clips. These implicit measures of social attention (fixation patterns) and emotional reactivity (arousal) were related to more explicit measures, i.e. the understanding of emotions of others and one’s own emotions in response to the video clips

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