Social Competence in Youth with Neurofibromatosis Type 1
Authors:   , McCurdy Mark David , Daly Brian P. 
Published: July 2021

Background Children and adolescents with neurofibromatosis type 1 (NF1) experience significant difficulties with social adjustment (SA), adversely impacting quality of life across the lifespan. SA refers to the quality of the child's social relationships and their attainment of developmentally appropriate goals. Youth with NF1 also experience clinically significant impairments across a variety of neuropsychological domains including global intelligence, attention, and executive function. Despite evidence that neurocognitive weaknesses and family-systems factors contribute to worse SA in other pediatric neurological populations, few studies have examined these associations in children with NF1. A greater understanding of processes underlying psychosocial deficits in this population will allow providers to identify those youth at greatest risk for social difficulties, and inform novel, targeted intervention strategies aimed at improving functional and quality of life outcomes. Aims The current study applied a developmental model of social competence in childhood brain disorder to identify aspects of social information processing (SIP) underlying social interaction (SI) and SA difficulties in youth with NF1. SIP encompasses domains such as executive functions and social-affective processing, including theory of mind (ToM). The specific aims of the current study were to 1) compare youth with NF1 and healthy controls (HC) across domains of social competence; 2) establish associations among SIP, SI, and SA constructs in youth with NF1, and 3) determine the potential mediating role of family functioning in youth's social behaviors and adjustment. Methods Participants diagnosed with NF1, and typically-developing HC youth, between the ages of 10 and 15 years were recruited prospectively from the Children's Hospital of Philadelphia. Participants completed a brief neuropsychological battery assessing global intellectual functioning (WASI-II), cognitive processing speed and working memory (WISC-V), performance- (TEA-Ch) and caregiver-reported (BRIEF-2) executive functioning, ToM (Jack & Jill Task), relationship quality (NRI-RQV), and perceived peer acceptance (PROMIS). Caregiver perceptions of peer acceptance (PROMIS) and family functioning (FAD) were also collected, and ratings of social interaction styles were assessed via teacher-report (BASC-3). The final sample consisted of 20 youth diagnosed with NF1 (M age = 12.55 years; SD = 1.67) and 14 HC (M age = 12.66 years; SD = 1.75) participants. MANOVAs were conducted to examine group differences across SIP, SI, and SA constructs. Serial parallel mediation analyses were used to evaluate relationships among social competence constructs and moderation analyses evaluated the conditional effects of family functioning on SA within the NF1 group. Results One-way MANOVA revealed significant group differences across all SIP variables. Compared to the HC group, youth with NF1 exhibited significantly worse TEA-Ch EF Composite performance, F(1, 32) = 6.29, p = .017, [eta]p2 = .164, higher caregiver-rated executive dysfunction on the BRIEF-2 GEC, F(1, 32) = 11.25, p = .002, [eta]p2 = .260, and lower accuracy on key ToM trials of the J&J Task, F(1, 32) = 5.97, p = .020, [eta]p2 = .157. Groups also differed on caregiver-reported PROMIS ratings, F(1, 32) = 9.79, p = .004, [eta]p2 = .234, but not on other SA variables of interest (ps > .05) . No significant differences were observed across teacher-reported SI variables (ps > .05). No significant indirect effects of SIP or SI were observed on the relationship between NF1 status and caregiver-reported peer acceptance (PROMIS). Within the NF1 group, family functioning (FAD) significantly moderated the relationship between TEA-Ch EF Composite performance and teacher-reported affiliative SI style (BASC-3 Social Skills; p = .049), and caregiver-reported PROMIS ratings (p = .040). FAD ratings also moderated the relationship between BRIEF-2 GEC scores and teacher-reported social withdrawal (BASC-3 Withdrawal; p = .020), and caregiver PROMIS ratings (p = .036), as well as the relationship between ToM performance (J&J) and teacher-reported aggression (BASC-3 Aggression; p = .039). Discussion Results of the current study suggest that youth with NF1 experience deficits in underlying SIP skills, including executive functioning and ToM, as well as lower caregiver-reported peer acceptance relative to their typically-developing peers. Although no significant indirect effects of underlying SIP skills and/or SI tendencies on the relationship between NF1 and SA were observed, these factors may play an important role in determining social competence outcomes in this population. Importantly, findings suggest that family functioning may play a significant role in moderating the relationships among these aspects of social competence. Results of the present study can inform the development of evidence-based interventions designed to improve SA in this population.