Cracking under pressure: Cognitive load influences performance in youth with NF1.
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OBJECTIVES: To extend the current understanding of executive function (EF) deficits in youth with neurofibromatosis type 1 by investigating the impact of cognitive load on performance compared to typically developing children. METHODS: In this prospective multicenter study, 42 children with neurofibromatosis type 1 (NF1) (ages 7-18) completed neuropsychological assessments of intellect and executive functioning. Age- and sex-matched controls (n = 42) were drawn from the normative database for the tasks of executive control (TEC). Multivariate and supplementary univariate analyses examined group differences and task effects (inhibitory control and working memory demand). Associations between TEC performance and parent-reported executive dysfunction (BRIEF) were also explored. RESULTS: Both groups showed reduced accuracy and speed with increased inhibitory demand and made fewer errors with increased working memory demand. However, children with NF1 were significantly less accurate and consistent across tasks, particularly under higher cognitive load, while controls improved or maintained performance. Significant group × cognitive load interactions were observed, and laboratory-based deficits in NF1 were associated with parent-reported executive dysfunction. CONCLUSIONS: Children with NF1 experience unique and multidimensional decrements in EF performance in response to increased cognitive load, unlike typically developing peers. These deficits appear to be clinically relevant. Targeting working memory and inhibitory control may reduce susceptibility to cognitive overload and improve outcomes for children with NF1.