Abstract

Individuals with Williams syndrome (WS) demonstrate an abnormally positive social bias. However, the neural substrates of this hypersociability, i.e., positive attribution bias and increased drive toward social interaction, have not fully been elucidated. Methods: We performed an event-related functional magnetic resonance imaging study while individuals with WS and typically developing controls (TD) matched positive and negative emotional faces. WS compared to TD showed reduced right amygdala activation during presentation of negative faces, as in the previous literature. In addition, WS showed a unique pattern of right orbitofrontal cortex activation. While TD showed medial orbitofrontal cortex activation in response to positive, and lateral orbitofrontal cortex activation to negative, WS showed the opposite pattern. In light of the general notion of a medial/lateral gradient of reward/punishment processing in the orbitofrontal cortex, these findings provide an additional biological explanation for, or correlate of positive attribution bias and hypersociability in WS.

Highlights

  • Williams syndrome (WS) is a neurodevelopmental disorder characterized by a hemizygous microdeletion of approximately 20 genes, contiguous with the elastin gene on chromosome 7 (Francke 1999)

  • In the present study we investigated further the possible role of orbitofrontal cortex (OFC) in WS during positive and negative emotional valence face processing

  • There was no significant difference in age (p= 0.79) but there was a significant difference between groups in intelligence (FSIQ, VIQ, PIQ all p’s

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Summary

Introduction

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a hemizygous microdeletion of approximately 20 genes, contiguous with the elastin gene on chromosome 7 (locus 7q11.2) (Francke 1999).

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