• B Barton and K North.
• Neurogenetics Research Unit, The Children's Hospital at Westmead, Westmead, NSW, Australia. belindab@chw.edu.au
• Child Care Health Dev. 2007 Jul 1; 33 (4): 401-8.

BackgroundNeurofibromatosis type 1 (NF1) is a genetic disorder associated with cognitive deficits, learning problems, medical complications and cosmetic disfigurement. Despite the wide-ranging impact of NF1, very few studies have examined the psychosocial adjustment of individuals with NF1, and in particular, self-concept. The aims of this study were threefold: (i) to examine the self-concept of children and adolescents with NF1; (ii) to compare the self-concept of children with NF1 (NF1 only), children with NF1 and learning difficulties [NF1 + LA (low achievement)], and children with NF1 and attention deficit hyperactivity disorder (ADHD) (NF1 + ADHD); and (iii) to examine the academic self-concept of these three groups relative to objective criteria - academic achievement and teacher ratings of academic competence.MethodsMeasures of self-concept, academic achievement and intelligence were administered to 49 children and 26 adolescents with NF1. Parents and teachers completed behavioural rating scales.ResultsThe majority of children and adolescents with NF1 reported positive global self-concept, with some exceptions on specific domains. Children and adolescents with NF1 reported significantly poorer self-concept for physical abilities. Adolescents also reported significantly poorer self-concept for mathematics and general self when compared with normative mean values. Clinical severity of NF1 was not a significant predictor of self-concept for physical appearance or physical abilities. Despite a significant difference between NF1 only, NF1 + LA and NF1 + ADHD groups on measures of academic achievement and teacher ratings of academic competence, there was no significant difference between the groups for academic domains of self-concept, or any other domain of self-concept. All three groups reported inflated academic self-perceptions relative to objective criteria.ConclusionChildren and adolescents with NF1 are most likely to develop negative self-concepts about their physical and sporting abilities. Inflated academic self-perceptions are discussed in light of the positive illusory bias, which may serve as an adaptive or protective function.

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