Hypersociability in Williams syndrome

被引:345
作者
Jones, W
Bellugi, U
Lai, Z
Chiles, M
Reilly, J
Lincoln, A
Adolphs, R
机构
[1] Salk Inst Biol Studies, Cognit Neurosci Lab, La Jolla, CA 92037 USA
[2] San Diego State Univ, San Diego, CA 92182 USA
[3] Calif Sch Profess Psychol, San Francisco, CA 94109 USA
[4] Univ Iowa, Iowa City, IA 52242 USA
关键词
D O I
10.1162/089892900561968
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
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.
引用
收藏
页码:30 / 46
页数:17
相关论文
共 43 条
[1]   The human amygdala in social judgment [J].
Adolphs, R ;
Tranel, D ;
Damasio, AR .
NATURE, 1998, 393 (6684) :470-474
[2]  
[Anonymous], ESSAYS VERBAL VISUAL
[3]  
[Anonymous], 1995, CHILDES PROJECT TOOL
[4]  
BAMBERG M, 1996, INTERACTION SOCIAL C, P329
[5]  
Bayley N., 1993, Bayley scales of infant and toddler development, VSecond
[6]  
Bayley N, 1969, BAYLEY SCALES INFANT
[7]   Towards the neural basis for hypersociability in a genetic syndrome [J].
Bellugi, U ;
Adolphs, R ;
Cassady, C ;
Chiles, M .
NEUROREPORT, 1999, 10 (08) :1653-1657
[8]   Bridging cognition, the brain and molecular genetics: evidence from Williams syndrome [J].
Bellugi, U ;
Lichtenberger, L ;
Mills, D ;
Galaburda, A ;
Korenberg, JR .
TRENDS IN NEUROSCIENCES, 1999, 22 (05) :197-207
[9]  
Bellugi U, 1999, DEV COGN NEUROSCI, P111
[10]  
BELLUGI U, 1998, CND9801 U CAL SAN DI