title: Secondary narcolepsy in children with brain tumors.
abstract: We report two cases of children with disabling daytime sleepiness associated with suprasellar tumors and hypothalamic obesity. Multiple sleep latency testing demonstrated features consistent with severe narcolepsy, with sleep latencies of 0.25 and 0.75 minutes, and REM latencies of 2.1 and 1.5 minutes, respectively. An additional patient with hypothalamic damage secondary to a brain tumor, who was thought to be in a vegetative state, had features of narcolepsy on polysomnography. All children responded well to treatment with stimulants. We speculate that secondary narcolepsy associated with hypothalamic tumors is due to damage or loss of hypothalamic hypocretin-containing neurons. In view of the good response to treatment, we recommend that all children with excessive daytime sleepiness and hypothalamic damage be evaluated for narcolepsy.
authors.affiliation: The Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University, Baltimore, MD, USA. cmarcus@welch.jhu.edu
authors.forename: Carole L
authors.initials: CL
authors.lastname: Marcus
authors.forename: William H
authors.initials: WH
authors.lastname: Trescher
authors.forename: Ann C
authors.initials: AC
authors.lastname: Halbower
authors.forename: Janita
authors.initials: J
authors.lastname: Lutz
mesh_terms: D000293:Adolescent; D001559:Benzhydryl Compounds / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000150:complications* / Q000473:pathology; D000697:Central Nervous System Stimulants / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D000077408:Modafinil; D009290:Narcolepsy / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D017286:Polysomnography
chemical_list: D001559:Benzhydryl Compounds; D000697:Central Nervous System Stimulants; D000077408:Modafinil