II. Hypersociability in Williams Syndrome
Abstract
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 ground-work for crossdisciplinary analyses of gene-brain-behavior relationships.
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Other than Williams syndrome, indiscriminate behavior in young children is almost always associated with a history of severe neglect.
“Other than this genetic disorder, indiscriminate behavior in young children is almost always associated with a history of severe neglect.”
From Deviations from the expectable environment in early childhood and emerging psychopathology (2015) · cites this paper as Jones et al, 2000
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Self-citation
- Authors
- Jones, W., Bellugi, U., Lai, Z., Chiles, M., Reilly, J., Lincoln, A., & Adolphs, R.
- Affiliations
- Salk Institute for Biological Studies (US)
- San Diego State University (US)
- Alliant University (US)
- University of Iowa (US)
- Venue
- Journal of Cognitive Neuroscience
- Publisher
- MIT Press
- Published
- 2000-03-01
- Volume
- 12
- Issue
- Supplement 1
- Pages
- 30-46
- ISSN
- 0898-929X (ISSN-L)
- 1530-8898
- OpenAlex
- W2109056388
- Cited by
- 503
- References
- 37
- Citations by year
- 2026: 6 · 2025: 4 · 2024: 11 · 2023: 12 · 2022: 19 · 2021: 19 · 2020: 12 · 2019: 16 · 2018: 15 · 2017: 27 · 2016: 25 · 2015: 20 · 2014: 19 · 2013: 30 · 2012: 24
- FWCI
- 2.14
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of Cognitive Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health
- National Institute on Deafness and Other Communication Disorders (P50-DC01289, P50DC001289)
- National Institute of Neurological Disorders and Stroke (P50 NS 22343, P50NS022343)
- National Institute of Child Health and Human Development
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (P01 HD 33113, 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 › Autism Spectrum Disorder Research
- Keywords
- Williams syndrome, hypersociability, social phenotype, gene-brain-behavior relationship, genotypes, cognitive phenotypes, developmental disorders, personality, infants and toddlers, elementary school students, adults
- MeSH terms
- Adolescent, Adult, Anxiety, Separation, Autistic Disorder, Child, Child, Preschool, Down Syndrome, Female, Humans, Infant, Interview, Psychological, Language Development, Male, Psychological Tests, Social Behavior, Social Perception, Williams Syndrome
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