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Executive dysfunction in children with neurofibromatosis type 1: A study of action planning

Published online by Cambridge University Press:  04 August 2010

ARNAUD ROY ,
JEAN-LUC ROULIN ,
VALERIE CHARBONNIER ,
PHILIPPE ALLAIN ,
LUCIANO FASOTTI ,
SEBASTIEN BARBAROT ,
JEAN-FRANCOIS STALDER ,
ANNE TERRIEN  and
DIDIER LE GALL
ARNAUD ROY*
Affiliation:
Laboratory of Psychology, University of Angers, France Neurofibromatosis Clinic, University Hospital of Nantes, France Pediatric Unit for Learning Disabilities, University Hospital of Nantes, France
JEAN-LUC ROULIN
Affiliation:
Inter-University Laboratory of Psychology, University of Savoie, France
VALERIE CHARBONNIER
Affiliation:
Laboratory of Psychology, University of Angers, France Pediatric Unit for Learning Disabilities, University Hospital of Nantes, France
PHILIPPE ALLAIN
Affiliation:
Laboratory of Psychology, University of Angers, France Neuropsychology Unit, Department of Neurology, University Hospital of Angers, France
LUCIANO FASOTTI
Affiliation:
Radboud University Nijmegen, Donders Institute for Brain, Cognition and Behaviour, the Netherlands
SEBASTIEN BARBAROT
Affiliation:
Neurofibromatosis Clinic, University Hospital of Nantes, France
JEAN-FRANCOIS STALDER
Affiliation:
Neurofibromatosis Clinic, University Hospital of Nantes, France
ANNE TERRIEN
Affiliation:
Laboratory of Psychology, University of Angers, France Neuropsychology Unit, Department of Neurology, University Hospital of Angers, France
DIDIER LE GALL
Affiliation:
Laboratory of Psychology, University of Angers, France Neuropsychology Unit, Department of Neurology, University Hospital of Angers, France
*
*Correspondence and reprint requests to: Arnaud Roy, Université d’Angers, Faculté des Lettres, Langues et Sciences Humaines 11, boulevard Lavoisier, 49045 Angers Cedex 01 France; E-mail: arnaud.roy@univ-angers.fr
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Abstract

In this study, we tested the hypothesis that action planning is impaired in children with neurofibromatosis type 1 (NF1). Thirty-six children with NF1 were pair-matched to 36 healthy controls (HC) on age (range, 7–12 years), sex, and parental education level, and both groups were administered three action-planning tasks. To examine the relation of task performance to attention deficit hyperactivity disorder (ADHD), the NF1 group was divided into subsets of children who met or did not meet criteria for ADHD. Children with NF1 performed less well than HC on all planning tasks, and differences remained when controlling for IQ or a measure of visuospatial skill. Both the NF1 with ADHD subset and NF1 without ADHD subset performed more poorly than HC on two of the tasks, whereas only the NF1 with ADHD subset performed worse than HC on the third planning task. The results underscore the importance of evaluating executive function in children with NF1 and suggest that deficits in this domain may be only partially related to ADHD. Planning deficits in children with NF1 may be part of their cognitive phenotype. Identifying these deficits is relevant in determining factors contributing to learning problems and in developing appropriate interventions. (JINS, 2010, 16, 1056–1063.)

Keywords

Executive functionDysexecutive syndromeNeurodevelopmental diseaseLearning disabilitiesVisuospatial processingADHD
Type
Research Articles
Information
Journal of the International Neuropsychological Society , Volume 16 , Issue 6 , November 2010 , pp. 1056 - 1063
Copyright
Copyright © The International Neuropsychological Society 2010

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