This study investigates the relationship between anxiety symptoms and adaptive function in children with chromosome 22q11.2 deletion syndrome (22q11.2DS). Seventy-eight children between 7 and 14 years of age with 22q11.2DS and 36 typically developing (TD) children without known genetic syndromes participated in a larger study of neurocognition. Parents completed questionnaires about their child's anxiety symptoms (Behavior Assessment System for Children, 2nd edition [BASC-2] and Spence Children's Anxiety Scale [SCAS]) and adaptive functioning (BASC-2 and Adaptive Behavior Assessment System, 2nd edition). Within the 22q11.2DS group, different DSM-IV anxiety domains were also analyzed using SCAS subscales. Based on parent report, 19% of children with 22q11.2DS had a prior diagnosis of an anxiety disorder versus 58% with at least 1 elevated anxiety score (BASC-2 or SCAS). Mean BASC-2 anxiety scores were significantly higher in 22q11.2DS (55.6 ± 12.5) than in TD children (48.3 ± 10; p = .003), and a greater percentage of children with 22q11.2DS (37%) had an elevated BASC-2 anxiety scores compared with TD children (14%; p = .01). Higher anxiety scores were related to lower adaptive function (r = -.27; p = .015), but there was no relationship between Wechsler Intelligence Scale for Children, 4th edition Full Scale Intelligence Quotient and BASC-2 adaptive skills (r = -.06; p = .6) in the 22q11.2DS group. For the individual SCAS anxiety subscales, panic-agoraphobia (r = -.38; p = .03), physical injury (r = -.34; p = .05), and obsessive-compulsive disorder (r = -.47; p = .005) were significantly negatively related to adaptive function in 22q11.2DS. Despite the known risk, anxiety is underidentified in children with 22q11.2DS. The presence of anxiety symptoms, but not intelligence levels, in children with 22q11.2DS negatively correlated with adaptive function and impacts everyday living skills.
