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

Jennifer Kramer MSc , Renée L. Roelofs PhD , Ellen Wingbermühle PhD , Sara Pieters PhD , Jos Egger PhD
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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.
努南综合症儿童和青少年的执行和社会功能:认知和行为
目的本研究旨在研究Noonan综合征儿童和青少年的执行和社会功能,这有助于了解该人群的认知和行为特征以及可能的治疗方案。方法选取26例患有努南综合征(包括努南综合征、努南综合征合并多发痣、努南样综合征伴毛发生长期疏松)的儿童和青少年(平均年龄11.92岁,SD = 2.64)和25例发育典型的儿童和青少年(平均年龄10.32岁,SD = 2.75)作为研究对象。使用多变量方差分析比较这些组中儿童和青少年的执行和社会功能的认知和行为测量。此外,还研究了执行功能和社会功能之间的关系。结果显示,在工作记忆和注意力方面存在显著的组间差异,即使在控制结晶智力的情况下,对照组的表现也优于努南综合征儿童和青少年。在行为层面上,与对照组相比,患有努南综合征的儿童和青少年在日常生活中经历了更多的执行功能问题、更多的注意力缺陷/多动障碍和自闭症谱系障碍的特征,即使在控制结晶智力的情况下也是如此。执行功能的行为测量与自闭症谱系障碍的特征呈正相关。结论工作记忆和注意力障碍是儿童和青少年努南综合征的主要认知特征。这些困难与父母报告的执行功能问题、注意力缺陷/多动障碍的特征和自闭症谱系障碍一起出现。荷兰的这项研究检查了26名患有努南综合症的儿童和青少年(7-17岁)的执行和社会功能,并与25名正常发展的同龄人进行了比较。患有努南综合症的青少年在工作记忆、注意力和日常功能方面表现相对较差。在努南综合症组中,父母报告了更多的执行功能问题,多动症的注意缺陷特征和自闭症谱系障碍。这些发现为努南综合征的认知和行为特征提供了新的知识,并强调了在这一人群中进行个性化神经心理学评估的重要性,特别是在学习和社会问题等功能障碍的背景下。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
JAACAP open
JAACAP open Psychiatry and Mental Health
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