Summary
background The 22q11.2 deletion syndrome (22q11.2ds) has a highly variable phenotype with a multitude of somatic and psychiatric features. Little is known about the adaptive skills of adolescents with 22q11.2ds.
aim To investigate adaptive functioning, intelligence and behavioural problems and their interrelationship in adolescents with 22q11.2ds.
method We interviewed the parents of 37 adolescents with 22q11.2ds using the Vineland Adaptive Behavior Scales. We assessed the intelligence of the adolescents by means of the Wechsler Intelligence Scales. Parents, adolescents and teachers were required to complete the Achenbach behavioural questionnaire.
results We found that adolescents with 22q11.2ds had impaired adaptive skills; these skills were significantly more impaired than the adolescents’ overall intelligence, i.e. their I.Q. Socialisation was a relatively well-developed domain compared to daily living skills. All respondents reported that the number of internalising problems exceeded the number of externalising problems. There was no correlation between adaptive functioning and behavioural problems, age or gender.
conclusion The evaluation of adaptive skills in these adolescents is a first step on the road to the development of measures aimed at improving their functioning in society.