4.4 Article

Hypersociability in Williams syndrome

期刊

JOURNAL OF COGNITIVE NEUROSCIENCE
卷 12, 期 -, 页码 30-46

出版社

MIT PRESS
DOI: 10.1162/089892900561968

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资金

  1. NICHD NIH HHS [P01 HD33113] Funding Source: Medline
  2. NIDCD NIH HHS [P50 DC01289] Funding Source: Medline
  3. NINDS NIH HHS [P50 NS22343] Funding Source: Medline
  4. EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH & HUMAN DEVELOPMENT [P01HD033113] Funding Source: NIH RePORTER
  5. NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [P50NS022343] Funding Source: NIH RePORTER
  6. NATIONAL INSTITUTE ON DEAFNESS AND OTHER COMMUNICATION DISORDERS [P50DC001289] Funding Source: NIH RePORTER

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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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