Everyday executive functions in children with neurofibromatosis type 1: Data from nine institutions.
Year: 2026
Type: article
Study design: Observational Study
Abstract: Objective: Executive function (EF) difficulties are prevalent in neurogenetic syndromes such as neurofibromatosis type 1 (NF1), the most common autosomal dominant condition associated with nervous system tumors. However, age-related differences in EF problems in children with NF1 remains poorly understood. This study aimed to examine how EF problems in daily life vary across ages throughout childhood and adolescence and how these age patterns vary across demographic and NF1 disease-related characteristics.Method: This study integrated data of 1,294 observations from 1,025 children with NF1 (mean age = 9.77 years, SD = 4.06, range = 3-18) from nine institutions in the United States and Australia. EF was reported by parents using the Behavior Rating Inventory of Executive Function. Time-varying effect modeling was used to estimate curvilinear EF trends.Results: Children with NF1 demonstrated elevated EF problems compared to the normative mean from early childhood through late adolescence, with deviations generally largest from mid-childhood through mid-adolescence. At the most vulnerable age period, medium to large EF gaps were observed across five core domains (Cohen’s d = 0.73-1.42). The exact age patterns varied across EF domains, and by children’s sex, parental education, and NF1 inheritance.Conclusions: Leveraging the largest EF dataset of children with NF1 to date, this study precisely estimated EF problems across developmental periods and highlighted the heterogeneity in EF age patterns. These age-specific estimates are crucial for establishing EF developmental norms that guide clinical practice and patient management strategies. The findings can inform the design of individualized intervention and prevention strategies tailored to various developmental periods and diverse demographic and disease-related characteristics for children with NF1.
Source: Neuropsychology
DOI: 10.1037/neu0001087
Institutions: Army Medical University, Florida State University, National Cancer Institute, Center for Cancer Research, Murdoch Children's Research Institute, University of Wisconsin–Milwaukee, Children's National, George Washington University, Children's Hospital Colorado, University of Colorado Anschutz, Children's Hospital at Westmead, Westmead Hospital, Kennedy Krieger Institute, Center for Autism and Related Disorders, University of California, Los Angeles, The University of Texas System
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W7154608407