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Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging
Noah S. Eby, Jennifer L. Griffith, David H. Gutmann, Stephanie M. Morris
Bursky Center for Human Immunology & Immunotherapy Programs (CHiiPs)
Center of Regenerative Medicine
Roy and Diana Vagelos Division of Biology & Biomedical Sciences (DBBS)
Institute of Clinical and Translational Sciences
Neurofibromatosis (NF) Center
Siteman Cancer Center
Division of Pediatric and Developmental Neurology
Section of Neurocritical Care
DBBS - Neurosciences
Section of General Pediatric Neurology
DBBS - Developmental, Regenerative and Stem Cell Biology
DBBS - Molecular Genetics and Genomics
DBBS - Cancer Biology
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<0.001), externalizing problems (r=−0.366, p<0.001), and attention (r=−9.467, p=0.001). Cluster analysis revealed three distinct phenotypic subgroups, one of which exhibited normal cognitive ability, but impaired adaptive functioning, with persistent deficits in executive function, behavioral problems, and attention-deficit/hyperactivity disorder symptomatology. There was no relationship between ABAS scores and the number or location of unidentified bright objects. Interpretation: Adaptive functioning deficits are common among children with NF1 and are associated with impairment in other cognitive/behavioral domains, independent of general cognitive ability. What this paper adds: Deficits in adaptive behavior are common in children with neurofibromatosis type 1 (NF1). Poor adaptive functioning is associated with impairments in executive function, externalizing behaviors, and attention, regardless of cognitive ability. The presence or location of unidentified bright objects do not predict adaptive behavior skills in children with NF1.
Developmental Medicine and Child Neurology
https://doi.org/10.1111/dmcn.14144
10.1111/dmcn.14144
Dive into the research topics of 'Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging'. Together they form a unique fingerprint.
Neurofibromatosis 1 Medicine & Life Sciences 100%
Psychological Adaptation Medicine & Life Sciences 94%
Cognition Medicine & Life Sciences 64%
Magnetic Resonance Imaging Medicine & Life Sciences 54%
Executive Function Medicine & Life Sciences 42%
Aptitude Medicine & Life Sciences 27%
Brain Medicine & Life Sciences 20%
Eby, N. S., Griffith, J. L., Gutmann, D. H., & Morris, S. M. (2019). Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging. Developmental Medicine and Child Neurology, 61(8), 972-978. https://doi.org/10.1111/dmcn.14144
Eby, Noah S. ; Griffith, Jennifer L. ; Gutmann, David H. et al. / Adaptive functioning in children with neurofibromatosis type 1 : relationship to cognition, behavior, and magnetic resonance imaging. In: Developmental Medicine and Child Neurology. 2019 ; Vol. 61, No. 8. pp. 972-978.
@article{557fff33abd3482ea32543555ac9aa9d,
title = "Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging",
abstract = "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<0.001), externalizing problems (r=−0.366, p<0.001), and attention (r=−9.467, p=0.001). Cluster analysis revealed three distinct phenotypic subgroups, one of which exhibited normal cognitive ability, but impaired adaptive functioning, with persistent deficits in executive function, behavioral problems, and attention-deficit/hyperactivity disorder symptomatology. There was no relationship between ABAS scores and the number or location of unidentified bright objects. Interpretation: Adaptive functioning deficits are common among children with NF1 and are associated with impairment in other cognitive/behavioral domains, independent of general cognitive ability. What this paper adds: Deficits in adaptive behavior are common in children with neurofibromatosis type 1 (NF1). Poor adaptive functioning is associated with impairments in executive function, externalizing behaviors, and attention, regardless of cognitive ability. The presence or location of unidentified bright objects do not predict adaptive behavior skills in children with NF1.",
author = "Eby, {Noah S.} and Griffith, {Jennifer L.} and Gutmann, {David H.} and Morris, {Stephanie M.}",
note = "Publisher Copyright: {\textcopyright} 2019 Mac Keith Press",
doi = "10.1111/dmcn.14144",
journal = "Developmental Medicine and Child Neurology",
Eby, NS, Griffith, JL, Gutmann, DH & Morris, SM 2019, 'Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging', Developmental Medicine and Child Neurology, vol. 61, no. 8, pp. 972-978. https://doi.org/10.1111/dmcn.14144
Adaptive functioning in children with neurofibromatosis type 1 : relationship to cognition, behavior, and magnetic resonance imaging. / Eby, Noah S.; Griffith, Jennifer L.; Gutmann, David H. et al.
In: Developmental Medicine and Child Neurology, Vol. 61, No. 8, 08.2019, p. 972-978.
T1 - Adaptive functioning in children with neurofibromatosis type 1
T2 - relationship to cognition, behavior, and magnetic resonance imaging
AU - Eby, Noah S.
AU - Griffith, Jennifer L.
AU - Gutmann, David H.
AU - Morris, Stephanie M.
N1 - Publisher Copyright: © 2019 Mac Keith Press
N2 - 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<0.001), externalizing problems (r=−0.366, p<0.001), and attention (r=−9.467, p=0.001). Cluster analysis revealed three distinct phenotypic subgroups, one of which exhibited normal cognitive ability, but impaired adaptive functioning, with persistent deficits in executive function, behavioral problems, and attention-deficit/hyperactivity disorder symptomatology. There was no relationship between ABAS scores and the number or location of unidentified bright objects. Interpretation: Adaptive functioning deficits are common among children with NF1 and are associated with impairment in other cognitive/behavioral domains, independent of general cognitive ability. What this paper adds: Deficits in adaptive behavior are common in children with neurofibromatosis type 1 (NF1). Poor adaptive functioning is associated with impairments in executive function, externalizing behaviors, and attention, regardless of cognitive ability. The presence or location of unidentified bright objects do not predict adaptive behavior skills in children with NF1.
AB - 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<0.001), externalizing problems (r=−0.366, p<0.001), and attention (r=−9.467, p=0.001). Cluster analysis revealed three distinct phenotypic subgroups, one of which exhibited normal cognitive ability, but impaired adaptive functioning, with persistent deficits in executive function, behavioral problems, and attention-deficit/hyperactivity disorder symptomatology. There was no relationship between ABAS scores and the number or location of unidentified bright objects. Interpretation: Adaptive functioning deficits are common among children with NF1 and are associated with impairment in other cognitive/behavioral domains, independent of general cognitive ability. What this paper adds: Deficits in adaptive behavior are common in children with neurofibromatosis type 1 (NF1). Poor adaptive functioning is associated with impairments in executive function, externalizing behaviors, and attention, regardless of cognitive ability. The presence or location of unidentified bright objects do not predict adaptive behavior skills in children with NF1.
U2 - 10.1111/dmcn.14144
DO - 10.1111/dmcn.14144
JO - Developmental Medicine and Child Neurology
JF - Developmental Medicine and Child Neurology
Eby NS, Griffith JL, Gutmann DH, Morris SM. Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging. Developmental Medicine and Child Neurology. 2019 Aug;61(8):972-978. doi: 10.1111/dmcn.14144