Emerging Executive Functioning:
Relations to Behavioral Difficulties in
Young Children with Williams
Syndrome
-
1
University of Wisconsin-Milwaukee, United States
Background:Williams syndrome is a genetically-based neurodevelopmental disorder, characterized by mild to moderate intellectual ability. Research about the behavioral phenotype of children with Williams syndrome has found that children often show socio-communicative deficits that overlap with those seen in children with autism spectrum disorders (Klein-Tasman et al., 2007). They also show heightened levels of inattention and hyperactivity, with symptoms meeting criteria for ADHD at a rate higher than generally seen for children with intellectual disabilities (Leyfer et al., 2006).Although executive functions, complex cognitive processes associated with pre-frontal cortical functioning, show robust correlations to symptoms of ASD and ADHD in the literature (Pennington & Ozonoff, 1996), their role in the WS behavioral phenotype has yet to be explored. The current study examines emerging executive functions and their relation to parent reports of attention problems and social responsiveness in young children with WS.
Methods: Participants were 27 children with Williams syndrome (9 male, 18 female),ranging from 4-7 years (M= 69.87 months,SD=11.89). Each child was administered a brief measure of verbal and nonverbal reasoning (KBIT-II), as well as a battery of developmentally-appropriate executive function measures: A-not-B, Delayed Alternation, NEPSY Statue, Dimensional Change Cart Sort (DCCS). Parents of each participant were also asked to complete the Social Responsiveness Scale (SRS), a measure of social-cognitive and socio-communicative difficulties characteristic of ASD, and the Conners Parent Rating Scales-Revised (Conners), a measure of behavioral symptoms associated with ADHD.
Results: On measures with age-based norms (A-not-B and Statue), participants demonstrated borderline to mild impairment. Passing scores on the DCCS Post-switch phase were also well below age-expected levels. With the exception of Delayed Alternation, performance on executive function tasks was significantly correlated with age. No correlations with verbal or nonverbal standard scores were found. After controlling for age, DCCS performance correlated with parent ratings on the Conners ADHD Index (r = -.409, p <.05), SRS Social Communication (r = -.441, p <.05), and SRS Autistic Mannerisms (r = -.444, p <.05). Total raw scores for Statue correlated significantly with SRS Social Motivation (r = -.460, p <.05).
Discussion: To date, research has largely focused on documenting the occurrence of atypical behaviors that characterize individuals with Williams syndrome, with few investigations exploring dysfunctional brainbased processes that may contribute to their presence. The current study provides an analysis of emerging executive functions in Williams syndrome, and findings suggest that pre-frontal dysfunction may contribute to problem behaviors often characteristic of children in this population. Further implications regarding the relationship between executive function deficits and problem behavior in this population will be discussed.
Conference:
12th International Professional Conference on Williams Syndrome, Garden Grove,CA, United States, 13 Jul - 14 Jul, 2008.
Presentation Type:
Oral Presentation
Topic:
SESSION 5: Anxiety and Other Psychiatric Problems
Citation:
Tasman
BK and
Gallo
FJ
(2009). Emerging Executive Functioning:
Relations to Behavioral Difficulties in
Young Children with Williams
Syndrome.
Conference Abstract:
12th International Professional Conference on Williams Syndrome.
doi: 10.3389/conf.neuro.09.2009.07.016
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Received:
30 Apr 2009;
Published Online:
30 Apr 2009.
*
Correspondence:
B. K Tasman, University of Wisconsin-Milwaukee, Wisconsin, United States, karen.berman@nih.gov