Praxis skills and executive function in children with neurofibromatosis type 1

ABSTRACT

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.

KEYWORDS:

• Executive dysfunction
• learning disabilities
• motor coordination
• neurodevelopmental disorder
• praxis

Acknowledgments

The authors thank all the children and their parents who participated in this study. We are also grateful to our colleague Dr. Sharman Levinson for improving the English. We would also like to thank the French patients association “Association Neurofibromatosis & Recklinghausen” for its support and aid in the achievement of this work.