Jennifer Kramer MSc , Renée L. Roelofs PhD , Ellen Wingbermühle PhD , Sara Pieters PhD , Jos Egger PhD
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引用次数: 0
Abstract
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.
Plain language summary
This Netherlands study examined executive and social functioning in 26 children and adolescents (7-17 years) with Noonan syndromes compared to 25 typically developing peers. Youth with Noonan syndromes showed relatively lower performance on working memory and attention, and in daily functioning. Parents reported more executive function problems, characteristics of attention deficit with hyperactivity, and autism spectrum disorders in the group with Noonan syndromes. These findings contribute new knowledge of cognitive and behavioral features of Noonan syndromes and underline the importance of individualized neuropsychological assessment in this population, especially in the context of functional impairments, such as learning and social problems.