Behavioural, academic and neuropsychological profile of normally gifted Neurofibromatosis type 1 children

• Published in: Journal of intellectual disability research Vol. 49; no. 1; pp. 33 - 46
• Main Authors: Descheemaeker, M.-J., Ghesquière, P., Symons, H., Fryns, J. P., Legius, E.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Ltd 01.01.2005; Blackwell Publishing; Wiley Subscription Services, Inc
• Subjects: Academic Achievement; Academic underachievement; Achievement; Anatomy; Attention Deficit Disorders; Attention Deficit Hyperactivity Disorder; Behavior disorders; Behavior Problems; Behavior Rating Scales; behavioural problems; Check Lists; Child; Child Behavior Disorders - epidemiology; Child, Gifted; Cognition Disorders - diagnosis; Cognition Disorders - epidemiology; Control Groups; Developmental Delays; Developmental disabilities; Dyslexia; Elementary school students; Elementary Schools; Executive Function; Female; Genetic Disorders; Humans; Intelligence Quotient; Learning Disabilities; Literacy; Male; Measures (Individuals); Neurofibromatosis 1 - epidemiology; Neurofibromatosis type 1; Neurology; neuropsychological functioning; Neuropsychological Tests; Neuropsychology; Nonverbal Learning; Parents; Perceptual Disorders - diagnosis; Perceptual Disorders - epidemiology; Profiles; Severity of Illness Index; Spelling; Underachievement; Verbal Ability
• ISSN: 0964-2633; 1365-2788
• DOI: 10.1111/j.1365-2788.2005.00660.x

In the present study the neuropsychological, academic and social‐emotional profiles were examined in Neurofibromatosis type 1 (NF1) children Subjects  17 NF1 children (ages 7–11) with NF1 without serious medical problems and with a full scale IQ (FSIQ) above 70. Methods  Wechsler Intelligence Scale for Children‐Revised (WISC‐R), academic tests and an exhaustive neuropsychological test battery were administered in all children. Parents and teachers filled out the Child Behavioural Checklist (CBCL) and Teacher Report Form (TRF), respectively, the NF1 children the Experienced Competence Scale for Children (ECSC). Results and discussion  Nearly 50% (8/17) of the children showed learning disabilities, when corrected for IQ in the academic evaluations. Isolated impaired literacy skills, particularly spelling problems, were most frequent (4/8), whereas a pure arithmetic learning disability was rare (1/8). Three children presented both learning disabilities. Results on academic and neuropsychological tests did not fit the well‐known types of learning disabilities – nonverbal learning disability (NLD) and dyslexia. Nearly all NF1 children showed visual perceptual and executive dysfunctions. In this study, teachers more frequently reported behavioural problems in NF1 children than parents, as opposed to literature data in a general population. The correspondence of the perception of internalizing problems between the children and teachers was greater than between children and their parents. No correlation was found between the performances on the WISC‐R, specific neuropsychological results, academic performances and behavioural problems. The Deficiency in Attention, Motor and Perception (DAMP) concept seems most appropriate in order to describe the neuropsychological deficits and their repercussions on behavioural and academic performances seen in NF1 children.