Idiopathic Hypersomnia and Other Hypersomnia Syndromes. 2021

Lynn Marie Trotti, and Isabelle Arnulf
Department of Neurology, Emory University School of Medicine, and Emory Sleep Center, Emory Healthcare, 12 Executive Park Dr NE, Atlanta, GA, 30329, USA. Lbecke2@emory.edu.

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.

UI MeSH Term Description Entries
D006970 Disorders of Excessive Somnolence Disorders characterized by hypersomnolence during normal waking hours that may impair cognitive functioning. Subtypes include primary hypersomnia disorders (e.g., IDIOPATHIC HYPERSOMNOLENCE; NARCOLEPSY; and KLEINE-LEVIN SYNDROME) and secondary hypersomnia disorders where excessive somnolence can be attributed to a known cause (e.g., drug affect, MENTAL DISORDERS, and SLEEP APNEA SYNDROME). (From J Neurol Sci 1998 Jan 8;153(2):192-202; Thorpy, Principles and Practice of Sleep Medicine, 2nd ed, p320) Daytime Sleepiness,Daytime Somnolence,Excessive Daytime Sleepiness,Hypersomnia,Hypersomnolence,Primary Hypersomnia Disorders,Secondary Hypersomnia Disorders,DOES (Disorders of Excessive Somnolence),Excessive Somnolence Disorders,Hypersomnia, Recurrent,Hypersomnolence Disorders,Hypersomnolence Disorders, Primary,Hypersomnolence Disorders, Secondary,Primary Hypersomnolence Disorders,Secondary Hypersomnolence Disorders,DOESs (Disorders of Excessive Somnolence),Daytime Sleepiness, Excessive,Daytime Sleepinesses,Daytime Somnolences,Excessive Daytime Sleepinesses,Excessive Somnolence Disorder,Hypersomnia Disorder, Primary,Hypersomnia Disorder, Secondary,Hypersomnias,Hypersomnolence Disorder,Hypersomnolence Disorder, Primary,Hypersomnolence Disorder, Secondary,Primary Hypersomnia Disorder,Primary Hypersomnolence Disorder,Recurrent Hypersomnia,Recurrent Hypersomnias,Secondary Hypersomnia Disorder,Secondary Hypersomnolence Disorder,Sleepiness, Daytime,Sleepiness, Excessive Daytime,Somnolence Disorder, Excessive,Somnolence, Daytime
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000697 Central Nervous System Stimulants A loosely defined group of drugs that tend to increase behavioral alertness, agitation, or excitation. They work by a variety of mechanisms, but usually not by direct excitation of neurons. The many drugs that have such actions as side effects to their main therapeutic use are not included here. Analeptic,Analeptic Agent,Analeptic Drug,Analeptics,CNS Stimulant,CNS Stimulants,Central Nervous System Stimulant,Central Stimulant,Analeptic Agents,Analeptic Drugs,Central Stimulants,Agent, Analeptic,Agents, Analeptic,Drug, Analeptic,Drugs, Analeptic,Stimulant, CNS,Stimulant, Central,Stimulants, CNS,Stimulants, Central
D020177 Idiopathic Hypersomnia A sleep disorder of central nervous system origin characterized by prolonged nocturnal sleep and periods of daytime drowsiness. Affected individuals experience difficulty with awakening in the morning and may have associated sleep drunkenness, automatic behaviors, and memory disturbances. This condition differs from narcolepsy in that daytime sleep periods are longer, there is no association with CATAPLEXY, and the multiple sleep latency onset test does not record sleep-onset rapid eye movement sleep. (From Chokroverty, Sleep Disorders Medicine, 1994, pp319-20; Psychiatry Clin Neurosci 1998 Apr:52(2):125-129) Hypersomnolence, CNS, Idiopathic,Hypersomnolence, Idiopathic,Idiopathic CNS Hypersomnolence,Idiopathic Central Nervous System Hypersomnolence,CNS Hypersomnolence, Idiopathic,CNS Hypersomnolences, Idiopathic,Hypersomnia, Idiopathic,Hypersomnias, Idiopathic,Hypersomnolence, Idiopathic CNS,Hypersomnolences, Idiopathic,Hypersomnolences, Idiopathic CNS,Idiopathic CNS Hypersomnolences,Idiopathic Hypersomnias,Idiopathic Hypersomnolence,Idiopathic Hypersomnolences
D064690 Wakefulness-Promoting Agents A specific category of drugs that prevent sleepiness by specifically targeting sleep-mechanisms in the brain. They are used to treat DISORDERS OF EXCESSIVE SOMNOLENCE such as NARCOLEPSY. Note that this drug category does not include broadly-acting central nervous system stimulants such as AMPHETAMINES. Wake-Promoting Agents,Wake-Promoting Drug,Eugeroic Drugs,Wake-Promoting Drugs,Wake-Promoting Substances,Agents, Wake-Promoting,Agents, Wakefulness-Promoting,Drug, Wake-Promoting,Drugs, Eugeroic,Drugs, Wake-Promoting,Substances, Wake-Promoting,Wake Promoting Agents,Wake Promoting Drug,Wake Promoting Drugs,Wake Promoting Substances,Wakefulness Promoting Agents

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