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Review
. 2013 Jun;23(3):414-22.
doi: 10.1016/j.conb.2012.12.006. Epub 2013 Jan 15.

The social phenotype of Williams syndrome

Anna Järvinen  1 , Julie R Korenberg, Ursula Bellugi
Affiliations
Review

The social phenotype of Williams syndrome

Anna Järvinen et al. Curr Opin Neurobiol. 2013 Jun.

Abstract

Williams syndrome (WS) offers an exciting model for social neuroscience because its genetic basis is well-defined, and the unique phenotype reflects dimensions of prosocial behaviors. WS is associated with a strong drive to approach strangers, a gregarious personality, heightened social engagement yet difficult peer interactions, high nonsocial anxiety, unusual bias toward positive affect, and diminished sensitivity to fear. New neurobiological evidence points toward alterations in structure, function, and connectivity of the social brain (amygdala, fusiform face area, orbital-frontal regions). Recent genetic studies implicate gene networks in the WS region with the dysregulation of prosocial neuropeptides. The study of WS has implications for understanding human social development, and may provide insight for translating genetic and neuroendocrine evidence into treatments for disorders of social behavior.

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Figures

Figure 1
Figure 1
Peaks and valleys of cognitive ability: dissociation of visual-spatial and language (social) functions in WS.
Figure 2
Figure 2
A preoccupation with a stranger (experimenter) by a child with WS interferes with task administration.
Figure 3
Figure 3
ERP signature indexing converging profiles of attention to faces in WS (increased) and autism (decreased) relative to typical development (TD).
Figure 4
Figure 4
Summary of the key dimensions of the social phenotype of WS.
See this image and copyright information in PMC

References

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