Narcolepsy and Hypothalamic Region Tumors: Presentation and Evolution

Publication date: Available online 14 February 2018
Source:Pediatric Neurology
Author(s): Alexander G. Weil, Katherine Muir, Juliette Hukin, Alex Desautels, Véronique Martel, Sébastien Perreault
ObjectiveSince most cases of brain tumor-associated narcolepsy have been published in the form of a case report or small series, the clinical presentation and evolution has not been well described. We sought to better define the epidemiology, etiology, and outcome of brain tumor-related narcolepsy.MethodsAn extensive review of the literature was conducted to extract 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.ResultsWe report an unusual case of 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 (13/25, 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 case, had a complete resolution of symptoms. All had biopsy and were treated with adjuvant therapy. For patients with persistent symptoms, most (60%) improved following medical management of narcolepsy.ConclusionTo our knowledge, this is the largest series of patients published with this association. Our study allows us to better understand the etiology and outcome of patients with narcolepsy-related to brain tumors.



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