Williams syndrome is a rare congenital neurodevelopmental disorder which is characterized at the psychological level by an unusual cognitive phenotype. Individuals with Williams syndrome are typically affectionate, very interested in people, and have relatively good language and face-processing skills, despite low IQ scores. This profile has led some researchers to propose that Williams syndrome involves a sparing in theory of mind abilities. The research program will explore theory of mind and related social-cognitive abilities in well- defined groups of children and adolescents with Williams syndrome. The subjects with Williams syndrome will be compared to two sets of mentally retarded/low IQ (MR) and normal controls: one set matched on overall mental age, the other set matched on language abilities. The main hypothesis is that the subjects with Williams syndrome will perform significantly better than the matched MR subjects on tasks tapping basic theory of mind abilities, including false belief (Experiments 1, 2), knowledge and intention (Experiment 4), the use of mental states to explain behavior (Experiment 3), discrimination of facial expressions of affect (Experiment 6), and visual perspective taking (Experiment 5). Subjects with Williams syndrome are also predicted to perform significantly better than the MR controls on a second-order theory of mind task (Experiment 7). In contrast, subjects with WIlliams syndrome are predicted to perform at the same level as the MR controls, and significantly worse than the normally developing controls, on tasks tapping higher-order social-cognitive constructs that build on a basic theory of mind but which also entail additional aspects of conceptual change. This prediction will be tested in tasks tapping knowledge of lies and jokes (Experiment 8), the concepts of trait (Experiment 9) and empathy (Experiment 11), moral judgment (Experiment 10), concepts of personal and social relations (Experiment 12), and self understanding (Experiment 13). The findings from this research will have important theoretical implications for conceptualizations of the cognitive and neural mechanisms that underlie social cognition, and will advance the understanding of social development in individuals who have Williams syndrome.

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Research Project (R01)
Project #
5R01HD033470-03
Application #
2523084
Study Section
Special Emphasis Panel (ZRG2-BEM (02))
Project Start
1995-09-29
Project End
2000-04-30
Budget Start
1997-05-01
Budget End
1998-04-30
Support Year
3
Fiscal Year
1997
Total Cost
Indirect Cost
Name
Eunice Kennedy Shriver Center Mtl Retardatn
Department
Type
DUNS #
City
Waltham
State
MA
Country
United States
Zip Code
02254
Skwerer, Daniela Plesa; Ammerman, Emily; Tager-Flusberg, Helen (2013) Do you have a question for me? How children with Williams syndrome respond to ambiguous referential communication during a joint activity. J Child Lang 40:266-89
Plesa Skwerer, D; Borum, L; Verbalis, A et al. (2009) Autonomic responses to dynamic displays of facial expressions in adolescents and adults with Williams syndrome. Soc Cogn Affect Neurosci 4:93-100
Plesa-Skwerer, Daniela; Sullivan, Kate; Joffre, Kristen et al. (2004) Self concept in people with Williams syndrome and Prader-Willi syndrome. Res Dev Disabil 25:119-38
Tager-Flusberg, Helen; Plesa-Skwerer, Daniela; Faja, Susan et al. (2003) People with Williams syndrome process faces holistically. Cognition 89:11-24
Sullivan, Kate; Winner, Ellen; Tager-Flusberg, Helen (2003) Can adolescents with Williams syndrome tell the difference between lies and jokes? Dev Neuropsychol 23:85-103
Levy, Yonata; Smith, Jason; Tager-Flusberg, Helen (2003) Word reading and reading-related skills in adolescents with Williams syndrome. J Child Psychol Psychiatry 44:576-87
Tager-Flusberg, H; Sullivan, K (2000) A componential view of theory of mind: evidence from Williams syndrome. Cognition 76:59-90
Sullivan, K; Tager-Flusberg, H (1999) Second-order belief attribution in Williams syndrome: intact or impaired? Am J Ment Retard 104:523-32
Tager-Flusberg, H; Boshart, J; Baron-Cohen, S (1998) Reading the windows to the soul: evidence of domain-specific sparing in Williams syndrome. J Cogn Neurosci 10:631-9