Remigereau Chrystelle, Roy Arnaud, Costini Orianne, Barbarot Sébastien, Bru Marie, Le Gall Didier
a Laboratory of Psychology LPPL (EA 4638), UBL , Brittany-Loire University , Angers , France.
b Reference Center for Learning Disabilities , University Hospital of Nantes , Nantes , France.
Appl Neuropsychol Child. 2018 Jul-Sep;7(3):224-234. doi: 10.1080/21622965.2017.1295856. Epub 2017 Mar 15.
This study aimed at examining motor and ideomotor praxis skills in children with neurofibromatosis type 1 (NF1). The impact of executive dysfunction, frequently described in children with NF1, on the expression of praxis impairments was also studied. Eighteen children with NF1 were included and matched with 20 control children for age (7-14 years), sex, laterality, and parental education level. Both groups of children underwent an assessment based on cognitive models of apraxia including visuomotor tasks, executive tests, and everyday life questionnaires. The group of children with NF1 showed a trend to weaker performances on motor and ideomotor praxis than the control group, only on the finger use condition (ps < .05; with a moderate to large effect size), but not regarding manual use condition (ps > .08). Moreover, these praxis difficulties disappeared when executive dysfunctions (planning and inhibition) were controlled. These findings support the negative impact of executive dysfunctions on praxis skills in children with NF1. The identification of praxis and executive function disorders as well as their interaction is important for differentiating primary praxic disorder from a cognitive deficit that may be expressed in gesture. Clinically, this distinction is essential to optimize targeted and effective rehabilitative interventions.
本研究旨在检测1型神经纤维瘤病（NF1）患儿的运动和观念运动运用技能。同时还研究了NF1患儿中经常出现的执行功能障碍对运用障碍表现的影响。研究纳入了18名NF1患儿，并根据年龄（7 - 14岁）、性别、利手和父母教育水平与20名对照儿童进行匹配。两组儿童均接受了基于失用症认知模型的评估，包括视运动任务、执行测试和日常生活问卷。NF1患儿组在运动和观念运动运用方面的表现仅在手指使用条件下（p值<0.05；效应量为中等至大）比对照组有表现较弱的趋势，但在手动使用条件下无此趋势（p值>0.08）。此外，当控制执行功能障碍（计划和抑制）时，这些运用困难消失了。这些发现支持了执行功能障碍对NF1患儿运用技能的负面影响。识别运用和执行功能障碍及其相互作用对于区分原发性运用障碍与可能在手势中表现出的认知缺陷很重要。临床上，这种区分对于优化有针对性的有效康复干预至关重要。
