AIM This systematic review aimed to pull together the findings from research into behavioural systems and attention in children with neurofibromatosis type 1 (NF1) and to identify areas that need further study.METHOD Relevant papers were identified through searches of electronic databases (MEDLINE, PsycINFO, EMBASE) and manual searches through reference lists. In total, 5746 articles were identified and 57 met the inclusion criteria. The data were synthesized using the narrative approach, as the studies varied considerably in terms of participants and measures. RESULTSThe results of the review showed that intelligence, academic skills, visuospatial skills, social competence, and attention are impaired in children with NF1. Evidence of deficits in memory, motor functioning, language, and executive functions was less clear. INTERPRETATIONResearch has made marked progress in outlining the behavioural phenotype of NF1. However, although the general areas of impairment are becoming better known, the exact nature of the impairment is still not understood in many areas of behaviour. Care needs to be taken with the way in which behavioural constructs are defined and measured, and the variability of problems in NF1 is a particular challenge. Nevertheless, research is steadily moving towards comprehensive understanding of behaviour in children with NF1.Neurofibromatosis type 1 (NF1) is one of the most common genetic disorders with a prevalence in the UK of 1 per 4560 live births. 1 The main disease feature in childhood is the emergence of multiple café-au-lait spots (flat coffee-coloured skin lesions) and skinfold freckling. The most common complications in childhood are different deficits of cognition and behaviour, which affect the majority of children with NF1. Children with NF1 are also at a higher risk of a number of much rarer physical complications that can affect virtually every system of the body 2 and include disfiguring plexiform neurofibromas, scoliosis, pseudarthrosis, and optic nerve gliomas.The cognitive and behavioural problems associated with NF1 are a major impairment to children's functioning at school and are issues that repeatedly come up in clinical practice as causing great distress to parents. The IQ of children with NF1 is usually within the normal range although somewhat lower than that of their unaffected siblings or community comparisons. More than 70% of children with NF1 perform more poorly at school than would be expected given their intellectual abilities.3 Furthermore, only a small proportion of children with NF1 are diagnosed with attention-deficit-hyperactivity disorder (ADHD), 4 although experimental studies using clinical diagnostic criteria for ADHD suggest that the incidence of this disorder among children with NF1 is up to 40 to 50%. 3 Issues concerning learning and various behaviour problems (such as difficulties with attention, peer relationships, and challenging behaviours) are among the most common complaints by parents of children with NF1 that emerge in our clinic work. Questi...

Awake craniotomy with language mapping enables maximum resection of tumours in eloquent areas while preserving function. This study aims to understand the lived experiences of those undergoing an awake craniotomy. Six participants who underwent awake craniotomy were interviewed, and the data were analysed using Interpretative Phenomenological Analysis. Themes were identified as 'Unspeakable Fear', 'Dissociation' and 'Control and Responsibility'. Participants discussed how surgery was a threat to the sense of self. Dissociation during surgery operated as a protective mechanism, while the surgical team all had roles in maintaining this bubble of dissociation, such as being a support to the patient's emotional needs.