Executive dysfunction in children with neurofibromatosis type 1: a study of action planning.
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.
Roy
Arnaud
A
Laboratory of Psychology, University of Angers, France. arnaud.roy@univangers.fr
Roulin
Jean-Luc
JL
Charbonnier
Valerie
V
Allain
Philippe
P
Fasotti
Luciano
L
Barbarot
Sebastien
S
Stalder
Jean-Francois
JF
Terrien
Anne
A
Le Gall
Didier
D
D000704:Analysis of Variance; D016022:Case-Control Studies; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D056344:Executive Function / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D009483:Neuropsychological Tests; D013028:Space Perception / Q000502:physiology; D014888:Wechsler Scales