Predictors of cognitive development in children with neurofibromatosis type 1 and plexiform neurofibromas

Yang Hou, Taryn Allen, Pamela L. Wolters, Mary Anne Toledo-Tamula, Staci Martin, Andrea Baldwin, Stephanie Reda, Andy Gillespie, Anne Goodwin, Brigitte C. Widemann

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


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 languageEnglish
Pages (from-to)977-984
Number of pages8
JournalDevelopmental Medicine and Child Neurology
Issue number8
StatePublished - Aug 1 2020

Bibliographical note

Funding Information:
The authors thank the children and caregivers who participated in this study. We also thank all members of the Health Psychology and Neurobehavioral Research Group and the Neurofibromatosis Team of the Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute for their assistance with data collection and the care of patients on the NF1 Natural History study. This research was supported by the Intramural Research Program of the National Institutes of Health. In addition, this project has been funded in whole or in part with federal funds from the National Cancer Institute, National Institutes of Health under contract no. HHSN261200800001E. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government.

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


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