Abstract
Aim: To describe the cognitive development of children with neurofibromatosis type 1 (NF1) and plexiform neurofibromas, and identify predictors of cognitive development. Method: Participants included 88 children with NF1 and plexiform neurofibromas (50 males, 38 females, aged 6–18y, mean=12y, SD=3y 7mo) on a natural history study at the National Cancer Institute. Neuropsychological assessments (e.g. IQ, academic achievement, attention, and executive functioning) were administered three times over 6 years. Results: Relative to normative peers, the total sample of children with NF1 and plexiform neurofibromas demonstrated significantly lower scores in most cognitive domains and decreasing z-scores over time in math, writing, inhibitory control, and working memory. Children who had parents with (vs without) NF1 were more likely to experience decreased z-scores in performance IQ, reading, writing, attention, and working memory. Higher (vs lower) parental education was related to higher levels of IQ, math, reading, and cognitive flexibility and a slower decrease in math z-scores. Children’s sex and the number of NF1 disease-related complications were not related to most cognitive outcomes. Interpretation: Children with NF1 and plexiform neurofibromas are at high risk for cognitive difficulties and declining z-scores in various domains of cognitive functioning over time. The findings highlight the need for a better understanding of the within-group differences in these children and their need for individualized educational plans. What this paper adds: Math, writing, inhibitory control, and working memory scores decreased over time. The proportion of children with clinically significant cognitive deficits increased over time. Parental neurofibromatosis type 1 and low education were related to greater cognitive difficulties in children.
Original language | English |
---|---|
Pages (from-to) | 977-984 |
Number of pages | 8 |
Journal | Developmental Medicine and Child Neurology |
Volume | 62 |
Issue number | 8 |
DOIs | |
State | Published - Aug 1 2020 |
Bibliographical note
Publisher Copyright:Published 2020. This article is a U.S. Government work and is in the public domain in the USA.
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Developmental Neuroscience
- Clinical Neurology