Evaluating everyday executive functions and psychosocial behavior in children with neurofibromatosis type I

Brian S. Potter (2006)

This study examines the executive functioning and social/emotional adjustment of children and adolescents with Neurofibromatosis type 1 (NF1). Archival data of respondent report instruments were used to assess the above factors. Thus far, studies exploring the executive functions, social, and emotional outcomes in children with NF1 have yielded mixed results. The current study investigated profiles of everyday executive functioning via parent ratings on the Behavior Rating Inventory of Executive Function (BRIEF) for 60 children with NF1 relative to children with Attention-Deficit/Hyperactivity Disorder-Combined type (ADHD-C), Attention-Deficit Hyperactivity Disorder-Inattentive type (ADHD-I), and a non-clinical control group. The groups were matched for age, gender, and mother's level of education. A profile analysis was conducted as a mixed repeated measures ANOVA, with scores on each of the BRIEF scales as within-subjects measures and participant group (NF1, ADHD-C, ADHD-I, Nonclinical) as the between subjects variable. In addition, this study examines the social and emotional impact on children with NF1 as well as examines the differences between genders. Findings indicated that the NF1 group had significantly different executive function profiles when compared with the non-clinical group and the ADHD-C group. BRIEF profiles for the NF1 group were not significantly different from the ADHD-I group, suggesting that the profile of executive function deficits in NF1 is similar to that seen in children diagnosed with ADHD-I. Children with NF I exhibited greater difficulty with metacognitive aspects of executive functioning than with behavioral regulation. Parent ratings of children with NF1 on the Behavioral Assessment System for Children (BASC) were compared to normative expectations. Children with NF1 were rated as having more internalizing and social difficulties. This study also examines gender differences in children with NF1. Males with NF1 were rated as having greater degree of executive deficits as well as more externalizing problems than females. No gender differences were noted between genders for internalizing problems.