Neuropsychological evaluation and parental assessment of behavioral and motor difficulties in children with neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem disorder, with large inter and intrafamilial clinical variability and uncertain prognosis. In children with NF1 cognitive disorders, learning difficulties and behavioral problems are common. The present study aims to establish the n...

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Publicado en:Research in Developmental Disabilities Vol. 48; pp. 220 - 231
Autores principales: Coutinho, V., Kemlin, I., Dorison, N., Billette de Villemeur, T., Rodriguez, D., Dellatolas, G.
Formato: research Journal Article
Publicado: Pergamon Press - An Imprint of Elsevier Science Jan2016
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Jan2016
      vid: 48
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      pub: Pergamon Press - An Imprint of Elsevier Science
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        atl: Neuropsychological evaluation and parental assessment of behavioral and motor difficulties in children with neurofibromatosis type 1.
      aug:
        au:
          Coutinho, V.
          Kemlin, I.
          Dorison, N.
          Billette de Villemeur, T.
          Rodriguez, D.
          Dellatolas, G.
        affil: APHP, GHUEP, Hôpital Trousseau, Service de Neuropédiatrie, 26, avenue Dr. Netter, 75012 Paris, France
      sug:
        subj:
          Learning Disorders Diagnosis
          Neurofibromatosis 1 Psychosocial Factors
          Motor Skills
          Learning Disorders Psychosocial Factors
          Neurofibromatosis 1 Diagnosis
          Neurofibromatosis 1 Physiopathology
          Behavioral Symptoms Diagnosis
          Parents Psychosocial Factors
          Neuropsychological Tests
          Child
          Reproducibility of Results
          Female
          Adolescence
          Male
          Human
          Child: 6-12 years
          Adolescent: 13-18 years
          Female
          Male
      ab: Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem disorder, with large inter and intrafamilial clinical variability and uncertain prognosis. In children with NF1 cognitive disorders, learning difficulties and behavioral problems are common. The present study aims to establish the neuropsychological and behavioral profiles of 78 patients with NF1, aged between 5 and 18 years, and to examine the relationship between these profiles and the transmission of NF1 (sporadic vs. familial), clinical manifestations, and environmental factors. We used several questionnaires completed by parents and neuropsychological tests. The results confirmed specific neuropsychological disabilities in children with NF1, especially involving visuospatial and fine motor skills, learning difficulties and behavioral problems. Cognitive difficulties were significantly more frequent in patients with familial than in those with sporadic NF1. All parental questionnaires were correlated with each other, but parental reports were not associated with FSIQ, SES, school status, and clinical manifestations of the disease. Neuropsychological tests were poorly related to parental reports of cognitive and behavioral difficulties.
      pubtype: Academic Journal
      doctype:
        research
        Journal Article
      ougenre: Article
    language: English
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