Narcolepsy and Hypothalamic Region Tumors: Presentation and Evolution.

OBJECTIVE: Because most cases of brain tumor-associated narcolepsy have been published in the form of case reports or small series, the clinical presentation and evolution have not been well described. We sought to better define the epidemiology, etiology, and outcome of brain tumor-related narcolepsy. METHODS: We conducted an extensive review of the literature to identify cases of narcolepsy associated with brain tumors. Only cases of brain tumors involving the hypothalamic region including the suprasellar, sellar, and thalamus were included in this study. RESULTS: We report a child with possible narcolepsy in a child with a brain tumor. Through our literature review, we identified 25 additional cases of narcolepsy associated with brain tumors affecting the hypothalamic area. Most symptomatic narcolepsy cases were reported in children (70%). Half of the patients (13 of 25, 52%) developed narcolepsy after surgery, whereas 11 patients (44%) were symptomatic at the time of the tumor diagnosis. Ten patients had narcolepsy without cataplexy. Most cases were associated with craniopharyngioma (38%), adenoma (24%), and glioma (14%). Three, including our patient, experienced a complete resolution of symptoms. All patients underwent biopsy and were treated with adjuvant therapy. For patients with persistent symptoms, most (60%) improved following medical management of narcolepsy. CONCLUSION: This study represents the largest compilation of patients with this association. Our study allows us to better understand the etiology and outcome of patients with narcolepsy-related brain tumors.

Psychosocial Features of Neurofibromatosis Type 1 in Children and Adolescents.

Neurofibromatosis type 1 (NF1) is a common neurologic condition associated with a wide variety of developmental deficits that have an important impact on children and adolescents. OBJECTIVE: This article aims to document the psychosocial features of NF1 and to report the interventions described to address the needs of pediatric patients with NF1. METHODS: A literature review was conducted concerning the social life, mental health, and quality of life (QOL) of children and adolescents with NF1 as well as the psychosocial interventions addressed to this population. RESULTS: Compared to unaffected children and adolescents of the general population, pediatric patients with NF1 have an increased risk of having social difficulties, mental health disorders, behavioral and emotional problems, as well as diminished QOL. Only 3 articles describe interventions within the NF1 population to address these difficulties. CONCLUSION: There is a need to develop and assess psychosocial interventions for patients with NF1.