Abstract

Background: Neurofibromatosis type 1 (NF1) is a neurodevelopmental disorder associated with elevated risk of specific cognitive impairments and a high prevalence of psychological comorbidities. Children with NF1 have also been reported to display significant difficulties with peer relationships, although the exact nature of their social competence difficulties remains unclear. This study aimed to explore the nature of the day to day social competence difficulties observed in children with NF1 and to investigate how these difficulties might relate to cognitive dysfunction and symptoms of psychopathology. Methods and Findings: This study investigated parent ratings of day to day social competence in 23 children with neurofibromatosis type 1 (NF1) compared to 23 chronological age-matched typically developing controls using a brief, standardised questionnaire - the Social Competence with Peers Questionnaire. The relationships between social competence, psychopathology (parent ratings of Attention Deficit Hyperactivity Disorder or Autism Spectrum Disorder symptomatology), and cognitive ability (Full Scale IQ and parent ratings of functional executive behaviour) in children with NF1 were also explored using correlational analyses. Results indicated that children with NF1 displayed significantly poorer day to day social competence than controls. These social competence deficits were not related to Attention Deficit Hyperactivity Disorder symptomatology, Full Scale IQ or functional executive behaviour. However, difficulties with social competence were significantly related to Autism Spectrum Disorder symptomatology and socially anxious/avoidant behaviours in our NF1 cohort. Conclusions: These results indicate that children with NF1 are at significant risk of day to day social competence difficulties, especially those who display high levels of autistic symptomatology and socially anxious behaviour. Our findings suggest a need to incorporate screening for social competence problems and comorbid psychopathology into the more general clinical management of children with NF1.

Highlights

  • Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with an estimated prevalence of approximately 1 in 3,000

  • Mean raw scores on the Social Competence with Peers Questionnaire (SCPQ)-P and standardised T-scores on the Social responsiveness scale (SRS), Conners 3-PL, and Behavior rating inventory of executive function (BRIEF) subscales were compared between groups using the Mann-Whitney U test

  • This was considered to be appropriate, as a previous normative study revealed that there was no effect of age on SCPQ-P ratings in a typically developing sample aged between 8 and 17 years [44]

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Summary

Introduction

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with an estimated prevalence of approximately 1 in 3,000. Of children with NF1 [3], common complications of the condition include specific cognitive impairments [4, 5] and a high prevalence of psychological comorbidities [6, 7]. The aim of the current study was to investigate, in greater detail, the nature of day to day social competence difficulties in children with NF1. Neurofibromatosis type 1 (NF1) is a neurodevelopmental disorder associated with elevated risk of specific cognitive impairments and a high prevalence of psychological comorbidities. This study aimed to explore the nature of the day to day social competence difficulties observed in children with NF1 and to investigate how these difficulties might relate to cognitive dysfunction and symptoms of psychopathology

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