Social skills of children with 22q11 Deletion Syndrome: A social cognitive neuroscience approach

Karen Kiley-Brabeck, Fordham University


The 22q11 DS is the most common genetic deletion syndrome. The deletion is believed to result in the dysregulation of the dopamine, GABA, and glutamate systems which, in turn, affects the prefrontal cortex, associated circuitry, and cognitive abilities governed by these brain regions. Research suggests that 22q11 DS children experience attention and executive function deficits; clinical observation also suggests impaired social abilities. This study sought to empirically identify if 22q11 DS children experience social skills, executive function, and/or executive attention impairments, and how these skills may be associated. A social cognitive neuroscience approach was used that attempted to relate these three areas of functioning (neurochemical, neurocognitive, and social). ^ The study was conducted as part of a longitudinal project at Rockefeller University. Fifty-one 22q11 DS children and 30 control participants took part in the study. Parents completed the Social Skills Rating System (SSRS) and Behavior Rating Inventory of Executive Function (BRIEF). The children were administered two computerized tests, the Wisconsin Card Sorting Test: 64 - Computer Version (WCST: 64-CV) and the Attention Network Test for Children (ANT). ^ When compared with control participants, 22q11 DS children were found to: (1) have more impaired social skills, (2) be more likely to score in the clinically impaired range on the BRIEF global and subscale measures, and (3) have more perseverative responses and errors on the WCST: 64-CV. No significant group differences were found on the ANT; however a group x age interaction was detected. Correlational analysis revealed a significant, negative association between the BRIEF and SSRS total scores. A simple regression found that the BRIEF Global Composite score accounted for 42.4% of the variance in affected children's social skills. ^ Secondary analyses revealed significant associations between SSRS and BRIEF subscale scores. A multiple regression revealed that emotional control, planning, and monitoring skills were significant predictors of Total Social Skills Scores in affected children. A significant group x socio-economic status interaction was also found. ^ This study suggests that social and executive function skills are associated in 22q11 DS children. This has direct implications for remediation efforts and supports the validity of utilizing a social cognitive neuroscience approach. ^

Subject Area

Psychology, Developmental|Psychology, Clinical|Psychology, Physiological

Recommended Citation

Karen Kiley-Brabeck, "Social skills of children with 22q11 Deletion Syndrome: A social cognitive neuroscience approach" (January 1, 2004). ETD Collection for Fordham University. Paper AAI3116864.