{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T21:10:47Z","timestamp":1784149847924,"version":"3.55.0"},"reference-count":25,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2019,1,18]],"date-time":"2019-01-18T00:00:00Z","timestamp":1547769600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To characterize the adaptive behavior profile of children with neurofibromatosis type 1 (<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>1) and determine its relationship to neuropsychological functioning and non\u2010neoplastic T2\u2010weighted hyperintense brain lesions on brain magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>In this cross\u2010sectional study, we retrospectively reviewed neuropsychological reports from 104 children with <jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>1 (56 males, 48 females; mean age 10y 4mo; standard deviation [<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content>] 3y 4mo; range 3y 5mo\u201317y 6mo), and extracted data from a range of cognitive and behavioral measures, including the Adaptive Behavior Assessment System (<jats:styled-content style=\"fixed-case\">ABAS<\/jats:styled-content>). Brain <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> was retrospectively reviewed in 42 individuals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Adaptive Behavior Assessment System scores were continuously distributed and pathologically shifted by 0.79 to 1.26<jats:styled-content style=\"fixed-case\">SD<\/jats:styled-content> 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 (<jats:italic>r<\/jats:italic>=\u20139.543, <jats:italic>p<\/jats:italic>&lt;0.001), externalizing problems (<jats:italic>r<\/jats:italic>=\u22120.366, <jats:italic>p<\/jats:italic>&lt;0.001), and attention (<jats:italic>r<\/jats:italic>=\u22129.467, <jats:italic>p<\/jats:italic>=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\u2010deficit\/hyperactivity disorder symptomatology. There was no relationship between <jats:styled-content style=\"fixed-case\">ABAS<\/jats:styled-content> scores and the number or location of unidentified bright objects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Adaptive functioning deficits are common among children with <jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>1 and are associated with impairment in other cognitive\/behavioral domains, independent of general cognitive ability.<\/jats:p><\/jats:sec><jats:sec><jats:title>What this paper adds<\/jats:title><jats:p>\n<jats:list list-type=\"bullet\">\n\n<jats:list-item><jats:p>Deficits in adaptive behavior are common in children with neurofibromatosis type 1 (NF1).<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>Poor adaptive functioning is associated with impairments in executive function, externalizing behaviors, and attention, regardless of cognitive ability.<\/jats:p><\/jats:list-item>\n\n<jats:list-item><jats:p>The presence or location of unidentified bright objects do not predict adaptive behavior skills in children with NF1.<\/jats:p><\/jats:list-item>\n<\/jats:list>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.14144","type":"journal-article","created":{"date-parts":[[2019,1,19]],"date-time":"2019-01-19T19:27:02Z","timestamp":1547926022000},"page":"972-978","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging"],"prefix":"10.1111","volume":"61","author":[{"given":"Noah S","family":"Eby","sequence":"first","affiliation":[{"name":"Department of Neurology Washington University School of Medicine St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer L","family":"Griffith","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David H","family":"Gutmann","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0461-1098","authenticated-orcid":false,"given":"Stephanie M","family":"Morris","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,1,18]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2017.4"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073811423974"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000179303.72345.ce"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.13526"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2012.04399.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2788.2005.00711.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamapsychiatry.2014.2463"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0954579402001037"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.13631"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00004703-199608000-00004"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0012162204000921"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/690432"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.44.5.878"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1080\/87565640903265137"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738040190110201"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2006.108134"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.6.1660"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2011.04139.x"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/CPJ.0000000000000494"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003881"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12043"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2012.04377.x"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.37.12.933"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamapsychiatry.2016.2600"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12734"}],"container-title":["Developmental Medicine &amp; 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