Mechanisms of Social Deficits in Youth with Neurofibromatosis Type 1
Abstract
Rationale: Youth with neurofibromatosis type 1 (NF1) often have difficulties with social functioning (e.g., getting along with peers) that are similar to those seen in youth with autism spectrum disorder (ASD). Children with NF1 have fewer friends and experience more teasing and rejection from peers compared to their unaffected siblings or classmates. Such difficulties in youth are associated with higher rates of psychological distress, risky behavior, and suicidality later in young adulthood. However, research evaluating the underlying mechanisms that contribute to these difficulties remains limited, hindering the development of appropriate and effective interventions to improve social adjustment outcomes. Prior research suggests that the social difficulties go beyond the neurocognitive impairments often seen in NF1 and have been related to socio-emotional processes that are typically impaired in ASD. An emerging model of social skills development grounded in social cognitive neuroscience emphasizes the role of cognitive and affective functions (CAF), such as executive function, facial processing, social attention, and theory of mind (i.e., ability to understand others’ perspectives), and highlights brain areas essential to CAF (e.g., facial processing) that are often disrupted in atypical brain development. Such a model underscores potential mechanisms for poor social adjustment difficulties that can be targeted for screening and intervention efforts. Given that these domains remain understudied in NF1, there is a critical need to evaluate the underlying mechanisms contributing to impaired social adjustment. Objective: The long-term goals of this program of research are to apply knowledge of the CAF contributors to poor social adjustment outcomes in youth with NF1 to develop screening and intervention approaches and, ultimately, enhance the peer acceptance of youth with NF1. The objective of this study is to compare the CAF abilities of youth with NF1 to typically developing (TD) youth and youth with ASD and to establish associations between CAF abilities and social adjustment outcomes in youth with NF1. A secondary objective is to describe the neurobiological mechanisms underlying the CAF abilities of youth with NF1. Study procedures will include measuring CAF abilities, including executive function, facial processing, social attention, and perspective taking, as well as evaluating metrics of functional brain connectivity both at rest and during a social processing task using fMRI. Data on youth with ASD and TD youth will be leveraged from an existing data set, and this project will involve collecting new data on identical measures on youth with NF1. We anticipate that (1) youth with NF1 will show CAF deficits compared to TD youth that are similar to youth with ASD; (2) poorer CAF abilities will be related to worse social adjustment outcomes; and (3) youth with NF1 will exhibit reduced connectivity on fMRI metrics compared to TD youth that are similar to youth with ASD. Applicability and Impact: The proposed project has applicability to all youth diagnosed with NF1 and is highly relevant in allocating the delivery of scarce psychosocial assessment and intervention resources. Given the high prevalence of social difficulties in youth with NF1 and the consequential negative impact of poor social acceptance in childhood on later adult psychological functioning, it is important to determine the mechanisms for poor social adjustment in children with NF1 in order to develop effective screening and intervention approaches. Ascertaining CAF abilities that are related to social dysfunction in NF1 will direct screening and intervention services appropriately so that efforts are not spent on evaluating or intervening on factors that are not conceptually or empirically related to social adjustment outcomes. Findings from the proposed study could pinpoint areas that are essential to evaluate in psychosocial screen
Document Details
- Document Type
- DoD Grant Award
- Publication Date
- Mar 10, 2021
- Source ID
- W81XWH2010311
Entities
People
- Matthew Hocking
Organizations
- United States Army