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Contemporary Reviews in Sleep Medicine |

Central Disorders of HypersomnolenceCentral Disorders of Hypersomnolence: Focus on the Narcolepsies and Idiopathic Hypersomnia

Zeeshan Khan, DO; Lynn Marie Trotti, MD
Author and Funding Information

From the Emory Sleep Center, Emory University School of Medicine, Atlanta, GA.

CORRESPONDENCE TO: Lynn Marie Trotti, MD, 12 Executive Park Dr NE, Atlanta, GA 30329; e-mail: Lbecke2@emory.edu


FUNDING/SUPPORT: This study was supported in part by the National Institutes of Neurological Disorders and Stroke [Grant K23 NS083748 to Dr Trotti].

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;148(1):262-273. doi:10.1378/chest.14-1304
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The central disorders of hypersomnolence are characterized by severe daytime sleepiness, which is present despite normal quality and timing of nocturnal sleep. Recent reclassification distinguishes three main subtypes: narcolepsy type 1, narcolepsy type 2, and idiopathic hypersomnia (IH), which are the focus of this review. Narcolepsy type 1 results from loss of hypothalamic hypocretin neurons, while the pathophysiology underlying narcolepsy type 2 and IH remains to be fully elucidated. Treatment of all three disorders focuses on the management of sleepiness, with additional treatment of cataplexy in those patients with narcolepsy type 1. Sleepiness can be treated with modafinil/armodafinil or sympathomimetic CNS stimulants, which have been shown to be beneficial in randomized controlled trials of narcolepsy and, quite recently, IH. In those patients with narcolepsy type 1, sodium oxybate is effective for the treatment of both sleepiness and cataplexy. Despite these treatments, there remains a subset of hypersomnolent patients with persistent sleepiness, in whom alternate therapies are needed. Emerging treatments for sleepiness include histamine H3 antagonists (eg, pitolisant) and possibly negative allosteric modulators of the gamma-aminobutyric acid-A receptor (eg, clarithromycin and flumazenil).


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