Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging

Aim To characterize the adaptive behavior profile of children with neurofibromatosis type 1 (NF1) and determine its relationship to neuropsychological functioning and non‐neoplastic T2‐weighted hyperintense brain lesions on brain magnetic resonance imaging (MRI). Method In this cross‐sectional study...

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Bibliographic Details
Published in:Developmental medicine and child neurology 2019-08, Vol.61 (8), p.972-978
Main Authors: Eby, Noah S, Griffith, Jennifer L, Gutmann, David H, Morris, Stephanie M
Format: Article
Language:English
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Summary:Aim To characterize the adaptive behavior profile of children with neurofibromatosis type 1 (NF1) and determine its relationship to neuropsychological functioning and non‐neoplastic T2‐weighted hyperintense brain lesions on brain magnetic resonance imaging (MRI). Method In this cross‐sectional study, we retrospectively reviewed neuropsychological reports from 104 children with NF1 (56 males, 48 females; mean age 10y 4mo; standard deviation [SD] 3y 4mo; range 3y 5mo–17y 6mo), and extracted data from a range of cognitive and behavioral measures, including the Adaptive Behavior Assessment System (ABAS). Brain MRI was retrospectively reviewed in 42 individuals. Results Adaptive Behavior Assessment System scores were continuously distributed and pathologically shifted by 0.79 to 1.26SD across Conceptual, Social, and Practical domains, and 46.5% of individuals had a composite score in the borderline or impaired range. Impairment in adaptive functioning was correlated with deficits in executive function (r=–9.543, p
ISSN:0012-1622
1469-8749
DOI:10.1111/dmcn.14144