J. Cogn. Neurosci.
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(Journal of Cognitive Neuroscience. 2000;12:S30-S46.)
© 2000 The MIT Press

Article

Hypersociability in Williams Syndrome

Wendy Jones, Ursula Bellugi, Zona Lai, Michael Chiles, Judy Reilly, Alan Lincoln and Ralph Adolphs

The Salk Institute for Biological Studies
San Diego State University
California School of Professional Psychology
University of Iowa

Corresponding author. The Laboratory for Cognitive Neuroscience, The Salk Institute for Biological Studies, 10010 North Torrey Pines Road, La Jolla, CA 92037, USA. Tel.: +1-619-453-4100 ext. 1416; fax: +1-619-452-7052

Studies of abnormal populations provide a rare opportunity for examining relationships between cognition, genotype and brain neurobiology, permitting comparisons across these different levels of analysis. In our studies, we investigate individuals with a rare, genetically based disorder called Williams syndrome (WMS) to draw links among these levels. A critical component of such a cross-domain undertaking is the clear delineation of the phenotype of the disorder in question. Of special interest in this paper is a relatively unexplored unusual social phenotype in WMS that includes an overfriendly and engaging personality. Four studies measuring distinct aspects of hypersocial behavior in WMS are presented, each probing specific aspects in WMS infants, toddlers, school age children, and adults. The abnormal profile of excessively social behavior represents an important component of the phenotype that may distinguish WMS from other developmental disorders. Furthermore, the studies show that the profile is observed across a wide range of ages, and emerges consistently across multiple experimental paradigms. These studies of hypersocial behavior in WMS promise to provide the groundwork for crossdisciplinary analyses of gene–brain–behavior relationships.


Key Words: Hypersociability • Williams syndrome • Gene-brain-behavior relationship






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