Executive and Social Functioning in Children and Adolescents With Noonan Syndromes: Cognition and Behavior.

OBJECTIVE

The current study aims to examine executive and social functioning in children and adolescents with Noonan syndromes, which contributes to the understanding of the cognitive and behavioral profile of this population and possible treatment options.

METHOD: A total of 26 children and adolescents with Noonan syndromes (including Noonan syndrome, Noonan syndrome with multiple lentigines, and Noonan-like syndrome with loose anagen hair; mean age = 11.92 years, SD = 2.64) and 25 typically developing children and adolescents (mean age = 10.32 years, SD = 2.75) participated in this study. Cognitive and behavioral measures of executive and social functioning of children and adolescents in these groups were compared using multivariate analyses of variance. Moreover, the relationship between executive and social functioning was examined.

RESULTS

Results showed significant group differences on working memory and attention, with controls outperforming children and adolescents with Noonan syndromes, even when controlling for crystallized intelligence. At a behavioral level, children and adolescents with Noonan syndromes experienced more executive function problems and more characteristics of attention-deficit/hyperactivity disorder and autism spectrum disorders in daily life than controls, even when controlling for crystallized intelligence. Positive relationships were found between behavioral measures of executive functions and characteristics of autism spectrum disorders.

CONCLUSION

Difficulties in working memory and attention seem to be key cognitive features in children and adolescents with Noonan syndromes. These difficulties occur alongside parental reports of executive function problems, characteristics of attention-deficit/hyperactivity disorder, and autism spectrum disorders.