Adaptive Behavior and Executive Functioning in Children with Neurofibromatosis Type 1 Using a Mixed Design

Abstract

Objective: Children with neurofibromatosis type 1 (NF1) demonstrate poorer adaptive functioning compared with same-aged peers; however, there is limited research about the longitudinal pattern of adaptive behavior. The aim of this investigation was to examine parent-reported adaptive behavior of children with NF1 longitudinally beginning in early childhood and to examine relations with executive functioning. Method: Children with NF1 were assessed during early childhood (n = 59; aged 3–7; mean = 4.8, SD = 1.42) or school age (n = 39; aged 9–13; mean = 10.85, SD = 1.58), and a subset was seen at both time points (n = 26). The Scales of Independent Behavior—Revised was used to assess adaptive functioning, and the Behavior Rating Inventory of Executive Function—Preschool Version/Behavior Rating Inventory of Executive Function was used to evaluate everyday executive functioning. Results: Adaptive behavior in early childhood was significantly correlated with adaptive behavior at school age (with the exception of social interaction and communication skills) and was significantly poorer at school age. The frequency of difficulties increased over time for overall adaptive behavior and motor skills. Executive functioning was related to adaptive behavior cross-sectionally within early childhood and at school age and showed longitudinal predictive value over time. Conclusion: This research contributes to the limited NF1 adaptive behavior literature by characterizing the longitudinal pattern of adaptive behavior and relations with executive abilities.

Demographic and Disease-Related Predictors of Socioemotional Development in Children with Neurofibromatosis Type 1 and Plexiform Neurofibromas: An Exploratory Study

“…Impairments in cognitive abilities frequently found in the NF1 population [ 2 , 3 ] may have also contributed to the continuing socioemotional difficulties [ 6 , 50 ]. Indeed, improvements in development over time are rarely found among children with NF1 [ 29 , 30 ], and some previous research has found worsening problems, including increasing internalizing problems and poor adaptive skills over time [ 31 , 32 ]. The current study also found persistently poorer adaptive skills in the sample than in the normative sample over time.…”

“…Similarly, there was no significant change in risk for atypical behaviors in 39 young children with NF1 from 21 to 40 months old [ 30 ]. In contrast, the adaptive skills of 26 children with NF1 generally decreased over time from early childhood to school age [ 31 ]. Another study demonstrated that internalizing (but not externalizing) problems significantly increased across three years in a group of young children from 2 to 6 years old ( n = 23) [ 32 ].…”

Adolescents and young adults with neurofibromatosis type 1: A descriptive study of adaptive functioning

“…These deficits have even been documented in the context of average cognitive abilities (Eby, Griffith, Gutmann, & Morris, 2019). Glad et al (2020) found executive functioning in early childhood to be predictive of adaptive functioning in the school‐age period.…”

“…These deficits have even been documented in the context of average cognitive abilities (Eby, Griffith, Gutmann, & Morris, 2019). Glad et al (2020) found executive functioning in early childhood to be predictive of adaptive functioning in the school-age period.NF1 is a genetic disorder that occurs in approximately 1 out of every 2500-3500 births and is typically diagnosed in early childhood (Evans et al, 2010;Tonsgard, 2006). NF1 causes a wide variety of physical, psychosocial, and cognitive impairments, all of which may contribute to challenges in adaptive functioning, particularly during the years of transition from adolescence to young adulthood.…”

“…Areas of adaptive functioning include communication, daily living skills, and socialization (Sparrow, Cicchetti, & Balla, 2005). For individuals diagnosed with NF1, these deficits continue (Cohen et al, 2020;Payne et al, 2021) and in one study worsen (Glad et al, 2020) throughout childhood and adolescence. These deficits have even been documented in the context of average cognitive abilities (Eby, Griffith, Gutmann, & Morris, 2019).…”