title: Deficit in phonological processes: a characteristic of the neuropsychological profile of children with NF1.
abstract: Learning disabilities are one of the most frequent complications of neurofibromatosis type 1 (NF1) in children. Studies of the effects of the neurocognitive deficit on academic performance are relatively rare, owing to the small size of the populations concerned. However, research is needed to develop effective rehabilitation programs. In the present study, we explored the impact of a possible phonological deficit on the reading abilities of children with NF1. A multicenter, cross-sectional study was conducted in France on two groups of 75 children with or without NF1 aged 8-12 years, matched for age, sex, handedness, and reading level. All participants underwent a neuropsychological evaluation to assess their general cognitive level, reading skills, phonological processes, visuoperceptual abilities, and attentional capacity. Phonological skills were assessed by means of two phonological awareness tasks and one short-term memory task. In the group of children with NF1, 41% had reading difficulties. Phonological processes were impaired in this group, compared with the children without NF1. Similar differences were found for a phoneme deletion task after adjustment for reading difficulties, IQ level, and visuoperceptual abilities. Phonological awareness, but not phonological short-term memory, was impaired in children with NF1, and not just those whose reading was impaired. Results suggest that children with NF1 have a phonological awareness deficit, whatever their reading level. Identification of reduced phonological skills may warrant the implementation of a specific rehabilitation program before early reading difficulties emerge.
authors.affiliation: a ToNIC, Toulouse NeuroImaging Center , Université de Toulouse, Inserm , UPS , France.|b Children's Hospital , Purpan University Hospital , Toulouse , France.
authors.forename: Yves
authors.identifier: 0000-0001-7800-4392
authors.initials: Y
authors.lastname: Chaix
authors.affiliation: c Epidemiology Department , Toulouse University Hospital , Toulouse , France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Lauwers-Cancès
authors.affiliation: b Children's Hospital , Purpan University Hospital , Toulouse , France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Faure-Marie
authors.affiliation: c Epidemiology Department , Toulouse University Hospital , Toulouse , France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Gentil
authors.affiliation: b Children's Hospital , Purpan University Hospital , Toulouse , France.|d Pediatric Clinical Research Unit , Toulouse Clinical Investigation Center, Children's Hospital, Purpan University Hospital , Toulouse , France.
authors.forename: Sandrine
authors.initials: S
authors.lastname: Lelong
authors.affiliation: e Neuropediatrics & Disabilities Unit, Gatien de Clocheville Children's Hospital , Tours University Hospital , Tours , France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Schweitzer
authors.affiliation: f Pediatric Neurology Department & Neurofibromatosis Referral Center , Armand Trousseau Hospital, East Paris University Hospital , Paris , France.|g University of Paris VI Pierre & Marie Curie, Sorbonne Universities , Paris , France.|h "Neuroprotection of the Developing Brain" Joint Research Unit (U1141) , INSERM , Paris , France.
authors.forename: Diana
authors.initials: D
authors.lastname: Rodriguez
authors.affiliation: b Children's Hospital , Purpan University Hospital , Toulouse , France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Iannuzzi
authors.affiliation: f Pediatric Neurology Department & Neurofibromatosis Referral Center , Armand Trousseau Hospital, East Paris University Hospital , Paris , France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Kemlin
authors.affiliation: f Pediatric Neurology Department & Neurofibromatosis Referral Center , Armand Trousseau Hospital, East Paris University Hospital , Paris , France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Dorison
authors.affiliation: i Neuropediatric Department & Language Disorders Referral Center , Montpellier Regional University Hospital , Montpellier , France.|j Faculty of Medicine , University of Montpellier , Montpellier , France.|k "Physiology & Experimental Medicine" Research Unit (U1046 INSERM & UMR 9214 CNRS) , University of Montpellier , Montpellier , France.
authors.forename: François
authors.initials: F
authors.lastname: Rivier
authors.affiliation: i Neuropediatric Department & Language Disorders Referral Center , Montpellier Regional University Hospital , Montpellier , France.
authors.forename: Maryline
authors.initials: M
authors.lastname: Carniero
authors.affiliation: i Neuropediatric Department & Language Disorders Referral Center , Montpellier Regional University Hospital , Montpellier , France.
authors.forename: Elodie
authors.initials: E
authors.lastname: Preclaire
authors.affiliation: l Dermatology Clinic , Hôtel-Dieu University Hospital , Nantes , France.
authors.forename: Sébastien
authors.identifier: 0000-0002-6629-9100
authors.initials: S
authors.lastname: Barbarot
authors.affiliation: m Department of Pediatric Neurology , Woman Mother Child Hospital , Bron , France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Lion-François
authors.affiliation: n "Brain & Imaging" Joint Research Unit (UMR 930), Bretonneau Hospital , Tours Regional University Hospital , Tours , France.|o University of Tours François Rabelais , Tours , France.
authors.forename: Pierre
authors.identifier: 0000-0002-4152-9731
authors.initials: P
authors.lastname: Castelnau
mesh_terms: D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007859:Learning Disabilities; D008297:Male; D009456:Neurofibromatosis 1 / Q000473:pathology / Q000523:psychology*; D009483:Neuropsychological Tests / Q000592:standards*; D010700:Phonetics
keywords: Child; NF1; neuropsychology; phonological processes; reading disorders