Laboratory of Psychology, LPPL EA4638, University of Angers, Angers, France.
Department of Dermatology, Nantes University, CHU Nantes, Nantes, France.
J Clin Exp Neuropsychol. 2021 Mar;43(2):163-175. doi: 10.1080/13803395.2021.1893277. Epub 2021 Mar 8.
: Executive functions (EFs) impairment is common in children with neurofibromatosis type 1 (NF1), and could be a significant vulnerability associated with this medical disorder. However, we still know little about EFs in preschool NF1. Our study assessed EFs in NF1 children using performance-based tests and daily life questionnaires, which combined the views of parents and teachers. Seven classic experimental tasks were used to evaluate EFs in 33 NF1 children aged 3 to 5 years old, and BRIEF-P questionnaires were completed by their parents and teachers. These children's performance was compared with a control group of 52 healthy children matched in age, gender and socio-cultural status. NF1 children have significantly lower scores for 5 out of 7 executive tasks than control children and significantly higher levels of EF concerns in the parent and teacher BRIEF-P ratings. The correlations between performance-based tests and questionnaires are weak. Our results support an early executive dysfunction in NF1 children and call for early and systematic assessment of EFs. Both performance-based tests and questionnaires are complementary tools to investigate early EFs dysfunction in children with NF1.
执行功能（EFs）障碍在 1 型神经纤维瘤病（NF1）儿童中很常见，可能是与这种疾病相关的一个显著脆弱性因素。然而，我们对学龄前 NF1 儿童的 EFs 知之甚少。我们的研究使用基于表现的测试和日常生活问卷评估了 NF1 儿童的 EFs，这些问卷综合了家长和老师的观点。我们使用了 7 个经典的实验任务来评估 3 至 5 岁的 33 名 NF1 儿童的 EFs，并由他们的家长和老师完成 BRIEF-P 问卷。这些孩子的表现与 52 名年龄、性别和社会文化地位相匹配的健康儿童的对照组进行了比较。NF1 儿童在 7 项执行任务中的 5 项得分明显低于对照组儿童，在家长和教师 BRIEF-P 评分中 EF 问题的水平明显更高。基于表现的测试和问卷之间的相关性较弱。我们的研究结果支持 NF1 儿童存在早期执行功能障碍，并呼吁对 EFs 进行早期和系统的评估。基于表现的测试和问卷都是调查 NF1 儿童早期 EFs 功能障碍的互补工具。
