Idiopathic Hypersomnia and Other Hypersomnia Syndromes
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Idiopathic Hypersomnia and Other Hypersomnia Syndromes Lynn Marie Trotti 1
&
Isabelle Arnulf 2
# The American Society for Experimental NeuroTherapeutics, Inc. 2020
Abstract There are numerous disorders of known or presumed neurologic origin that result in excessive daytime sleepiness, collectively known as the central disorders of hypersomnolence. These include narcolepsy types 1 and 2, idiopathic hypersomnia, Kleine– Levin syndrome, and hypersomnia due to or associated with medical disease, neurologic disease, psychiatric disease, medications or substances, and insufficient sleep durations. This chapter focuses on the treatment of nonnarcoleptic hypersomnia syndromes, from those that are commonly encountered in neurologic practice, such as hypersomnia due to Parkinson’s disease, to those that are exceedingly rare but present with dramatic manifestations, such as Kleine–Levin syndrome. The level of evidence for the treatment of sleepiness in these disorders is generally lower than in the well-characterized syndrome of narcolepsy, but available clinical and randomized, controlled trial data can provide guidance for the management of each of these disorders. Treatments vary by diagnosis but may include modafinil/armodafinil, traditional psychostimulants, solriamfetol, pitolisant, clarithromycin, flumazenil, sodium oxybate, melatonin, methylprednisolone, and lithium. Keywords Idiopathic hypersomnia . Kleine–Levin syndrome . modafinil . psychostimulants . lithium . methylprednisolone
Introduction The central disorders of hypersomnolence are a group of disorders manifesting primarily as excessive daytime sleepiness, either persistent or episodic. These include narcolepsy types 1 and 2, idiopathic hypersomnia, Kleine–Levin syndrome, hypersomnia due to a medical or neurologic disorder, hypersomnia due to medication or substance, hypersomnia associated with psychiatric disorders, and insufficient sleep syndrome. Numerous controlled trials have evaluated treatment strategies for narcolepsy, particularly narcolepsy type 1 but also narcolepsy type 2. In contrast, very few controlled trials are available to guide treatment decisions for the other central disorders of hypersomnolence, and reliance on published clinical series, understanding of drug mechanisms,
* Lynn Marie Trotti [email protected] 1
Department of Neurology, Emory University School of Medicine, and Emory Sleep Center, Emory Healthcare, 12 Executive Park Dr NE, Atlanta, GA 30329, USA
2
Service des Pathologies du Sommeil, Centre National de Reference des Hypersomnies Rares, Hôpitaux Universitaires Pitié-Salpêtrière, Sorbonne Université, Paris 75013, France
and off-label use of medications approved for narcolepsy is often necessary for their management. This chapter focuses on the management of this latter group of hypersomnia disorders.
Idiopathic Hypersomnia Idiopathic hypersomnia (IH) is a central disorder of hypersomnolence that results in daily excessive daytime sleepiness, in the absence of another identified cause. Although sleepiness may
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