Towards the neural basis for hypersociability in a genetic syndrome
Abstract
Williams syndrome (WMS), a rare disorder with a distinctive profile of medical, psychological, neurophysiological and neuroanatomical characteristics, results from hemizygous deletion of about 20 genes. The phenotype exhibits specific dissociations in higher cognitive functions: general cognitive deficits but spared linguistic abilities; extreme spatial cognitive deficits, but intact face processing. Of special interest is an unusual social phenotype in WMS: an overly friendly, engaging personality and excessive sociability with strangers. In this first experimental study of social behavior in WMS, we report that WMS subjects show an abnormal positive bias in their social judgments of unfamiliar individuals, consistent with their behavior in real life. Our findings contribute to an understanding of the neural and genetic bases of human social behavior.
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WS is a neurodevelopmental condition caused by a hemizygous microdeletion on chromosome 7q11.23, and is characterized by hypersociality and being overly empathic.
“is a neurodevelopmental condition caused by a hemizygous microdeletion on chromosome 7q11.23, and is characterized by hypersociality and being overly empathic [684,685].”
From Reward circuitry dysfunction in psychiatric and neurodevelopmental disorders and genetic syndromes: animal models and clinical findings (2012) · cites this paper as [684]
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References 5 · Cited by 207
- Authors
- Bellugi, U., Adolphs, R., Cassady, C., & Chiles, M.
- Affiliations
- Salk Institute for Biological Studies (US)
- University of Iowa (US)
- Venue
- NeuroReport
- Published
- 1999-06
- Volume
- 10
- Issue
- 8
- Pages
- 1653-1657
- ISSN
- 0959-4965 (ISSN-L)
- OpenAlex
- W2123747365
- Cited by
- 207
- References
- 5
- Citations by year
- 2024: 1 · 2023: 1 · 2022: 4 · 2021: 1 · 2020: 7 · 2019: 3 · 2018: 7 · 2017: 7 · 2016: 8 · 2015: 9 · 2014: 12 · 2013: 13 · 2012: 11
- FWCI
- 1.18
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neuroreport (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (1p01dc001289-01)
- U.S. Public Health Service
- National Institute on Deafness and Other Communication Disorders (P01 DC01289)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (P01HD033113)
- Topics
- Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cognitive Neuroscience › Face Recognition and Perception
- Keywords
- Williams syndrome, hypersociability, social judgment, social behavior, social cognition, neural basis of social behavior
- MeSH terms
- Adult, Female, Humans, Male, Neuropsychological Tests, Social Behavior, Williams Syndrome
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