Preliteracy impairments in children with neurofibromatosis type 1

Dev Med Child Neurol. 2018 Jul;60(7):703-710. doi: 10.1111/dmcn.13768. Epub 2018 Apr 18.

Abstract

Aim: This cross-sectional study aimed to examine the preliteracy abilities of young children with neurofibromatosis type 1 (NF1) and to identify which of these abilities best predicted conventional literacy (spelling).

Method: Forty-two children with NF1 (23 males, 19 females; mean age [SD] 5y 6mo [6mo]) were compared with 32 unaffected children (15 males, 17 females; mean age [SD] 5y 4mo [6mo]). All children completed a comprehensive cognitive assessment including measures of phonological processing (phonological awareness, phonological memory, rapid automatic naming) and letter-sound knowledge.

Results: Children with NF1 performed significantly poorer than the comparison group across all cognitive and preliteracy domains, with specific weaknesses evident in phonological awareness (F1,68 =14.13, p<0.001, partial η2 =0.17), phonological memory (F1,68 =13.87, p<0.001, partial η2 =0.17), and letter-sound knowledge (F1,71 =5.65, p=0.020, partial η2 =0.07). Within the group with NF1 group, over a third of children demonstrated impairment in at least one phonological processing domain and the risk of phonological impairment was 5.60 times that of unaffected children. Children's letter-sound knowledge was the strongest predictor of conventional literacy (spelling).

Interpretation: This study establishes that preliteracy deficits are present and detectable in young children with NF1. As a result of the high incidence of preliteracy impairment, we recommend screening phonological awareness and letter-sound knowledge to identify risk of future learning disorders.

What this paper adds: Young children with neurofibromatosis type 1 are at elevated risk of preliteracy deficits. The most affected domains are phonological awareness and phonological memory. Letter-sound knowledge is the strongest predictor of conventional literacy (spelling).

Publication types

  • Multicenter Study

MeSH terms

  • Analysis of Variance
  • Awareness
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis
  • Cognition Disorders / etiology*
  • Comprehension
  • Cross-Sectional Studies
  • Developmental Disabilities / etiology*
  • Dyslexia / diagnosis
  • Dyslexia / etiology*
  • Female
  • Humans
  • Longitudinal Studies
  • Male
  • Neurofibromatosis 1 / complications*
  • Neuropsychological Tests