Difficultés motrices, cognitives et comportementales chez les enfants et adolescents atteints de neurofibromatose de type 1 (maladie de von Recklinghausen)

Abstract : Cognitive and behavioral difficulties are common in children with Neurofibromatosis type 1 (NF1), however findings concerning the specific neuropsychological and behavioral profile as well as the association of these difficulties with clinical manifestations and brain imagery abnormalities are often contradictory. After a literature review, the present study: (i) describes behavioral, cognitive, and motor difficulties in 78 patients with NF1, aged 5 to 18 years, using parental questionnaires (quality of life, impact of illness, parental difficulties, Conners, BRIEF, CBCL), and tests of intellectual efficiency and specific neuropsychological functions; (ii) examines the relationships between clinical, behavioral, neuropsychological and imaging findings (presence or absence of "Unidentified Bright Objects" UBOs, characteristic feature of NF1). Learning disabilities, despite relatively good report of quality of life, attention disorders and child anxiety were the main parental concerns. All parental questionnaires were strongly inter-correlated, and associated with an overall positive or negative parental attitude during the interview with the psychologist. Parental concerns were only weakly related to neuropsychological tests. The presence of specific cognitive difficulties, particularly in visuospatial and fine motor skills, was confirmed. Imaging data were not associated with neuropsychological scores. Cognitive difficulties were more important in familial than sporadic forms.
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Virginie Coutinho. Difficultés motrices, cognitives et comportementales chez les enfants et adolescents atteints de neurofibromatose de type 1 (maladie de von Recklinghausen). Psychologie. Université Sorbonne Paris Cité, 2015. Français. ⟨NNT : 2015USPCB185⟩. ⟨tel-01860627⟩

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