Literature DB >> 10953232

II. Hypersociability in Williams Syndrome.

W Jones1, U Bellugi, Z Lai, M Chiles, J Reilly, A Lincoln, R Adolphs.   

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 groundwork for crossdisciplinary analyses of gene-brain-behavior relationships.

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Year:  2000        PMID: 10953232     DOI: 10.1162/089892900561968

Source DB:  PubMed          Journal:  J Cogn Neurosci        ISSN: 0898-929X            Impact factor:   3.225


  117 in total

1.  Preliminary evidence of abnormal white matter related to the fusiform gyrus in Williams syndrome: a diffusion tensor imaging tractography study.

Authors:  B W Haas; F Hoeft; N Barnea-Goraly; G Golarai; U Bellugi; A L Reiss
Journal:  Genes Brain Behav       Date:  2011-10-19       Impact factor: 3.449

Review 2.  Cognitive and behavioral characteristics of children with Williams syndrome: implications for intervention approaches.

Authors:  Carolyn B Mervis; Angela E John
Journal:  Am J Med Genet C Semin Med Genet       Date:  2010-05-15       Impact factor: 3.908

3.  Autistic symptomatology, face processing abilities, and eye fixation patterns.

Authors:  Jennifer C Kirchner; Alexander Hatri; Hauke R Heekeren; Isabel Dziobek
Journal:  J Autism Dev Disord       Date:  2011-02

Review 4.  Genomic copy number variation in disorders of cognitive development.

Authors:  Eric M Morrow
Journal:  J Am Acad Child Adolesc Psychiatry       Date:  2010-11       Impact factor: 8.829

5.  Genetic influences on sociability: heightened amygdala reactivity and event-related responses to positive social stimuli in Williams syndrome.

Authors:  Brian W Haas; Debra Mills; Anna Yam; Fumiko Hoeft; Ursula Bellugi; Allan Reiss
Journal:  J Neurosci       Date:  2009-01-28       Impact factor: 6.167

6.  Theory of mind in Williams syndrome assessed using a nonverbal task.

Authors:  Melanie A Porter; Max Coltheart; Robyn Langdon
Journal:  J Autism Dev Disord       Date:  2008-05

7.  Comparison of TFII-I gene family members deleted in Williams-Beuren syndrome.

Authors:  Timothy A Hinsley; Pamela Cunliffe; Hannah J Tipney; Andrew Brass; May Tassabehji
Journal:  Protein Sci       Date:  2004-10       Impact factor: 6.725

8.  Viewing social scenes: a visual scan-path study comparing fragile X syndrome and Williams syndrome.

Authors:  Tracey A Williams; Melanie A Porter; Robyn Langdon
Journal:  J Autism Dev Disord       Date:  2013-08

9.  Toddlers with Williams syndrome process upright but not inverted faces holistically.

Authors:  Cara H Cashon; Oh-Ryeong Ha; Christopher A DeNicola; Carolyn B Mervis
Journal:  J Autism Dev Disord       Date:  2013-11

10.  A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes.

Authors:  Hayley Crawford; Joanna Moss; Laura Groves; Robyn Dowlen; Lisa Nelson; Donna Reid; Chris Oliver
Journal:  J Autism Dev Disord       Date:  2020-01
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