Abstract

Abstract Objective Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder affecting approximately 100,000 people in the U.S. It is commonly associated with neurofibromas, café-au-lait spots, and optic gliomas. It has also been linked to neurocognitive deficits, including learning disabilities and ADHD. This study examined relationships among executive functions (EF) and academic achievement in children diagnosed with NF1. Methods Participants included 29 children (ages 2–21; M = 9.28, SD = 5.50) diagnosed with NF1 referred for neuropsychological evaluation as part of standard clinical care. A battery of tests was administered, including measures of intelligence (Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition; Wechsler Intelligence Scale for Children, Fifth Edition; Wechsler Adult Intelligence Scale, Fourth Edition), attention (Conners’ Continuous Performance Test II), EF (Delis-Kaplan Executive Function System), academic achievement in reading and math (Wechsler Individual Achievement Test, Third Edition), and parent rating scales examining EF in the home environment (Behavior Rating Inventory of Executive Function, Second Edition). Results Relations between attention, EF, and academic achievement were examined using bivariate Pearson correlations. Lower scores on measures of EF (e.g., inhibition of prepotent responses) were linked to lower math achievement scores (r = .73, p < .05). Difficulties with sustained attention were associated with lower performance on measures of reading (r = −.60, p < .05) and math achievement (r = −.59, p < .05). Parent report of EF difficulties was correlated with lower reading scores (r = −.68, p < .05). Conclusions Results suggest that children with NF1 are at risk for deficits in EF and academic achievement. Problems in these two domains are likely to co-exist. Thus, interventions targeting these skills should be well integrated.

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