INTRODUCTION: Williams syndrome (WS) is a rare neurodevelopmental disorder that is caused by a hemizygous deletion on chromosome 7q11.23. The interest of WS to neurocognitive scientists stems from the uneven profiles of cognitive abilities, with spatial cognition seriously impaired and language and face processing relatively proficient. We know relatively little about the visual search attention and executive function in children with WS.
OBJECTIVE: The objective of this study was to examine the nature of visual search attention and executive function in children with WS, compared with children with Down syndrome (DS), healthy chronological age–matched control subjects (CA), and healthy mental age–matched control subjects (MA).
METHODS: A total of 142 children were tested: 21 with WS, 25 with DS, 45 CA, and 41 MA. MA were matched to the children with WS and DS using the Peabody Picture Vocabulary Test. All participants were assessed on a set of computerized visual search tasks and Wilding Monster Sorting Test using a touch-screen.
RESULTS: The results showed that selective attention, switch, and sustained attention of children with WS all are less developed. Children with WS produced a large number of shape errors, and they also confused shape distractors with targets more than the other groups. Children with WS exhibited poorer executive performance as compared with both groups of typical children. They produced more repetitive errors than did children with DS.
CONCLUSIONS: These findings reveal distinct visual search deficits and atypically developing executive function in children with WS.
- Copyright © 2008 by the American Academy of Pediatrics