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Idiopathic Hypersomnia: Neurobiology, Diagnosis, and Management

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Abstract

Idiopathic hypersomnia is a chronic neurologic sleep disorder that manifests as excessive daytime sleepiness despite normal or prolonged sleep times for age. Frequently, idiopathic hypersomnia is clinically characterized by marked sleep inertia, long and unrefreshing naps, and a high sleep efficiency. Since the initial description, there has been an ongoing evolution of its nomenclature, approach to diagnosis, characterization of symptoms, and determination of the burden of disease. In addition, an increased attention to and study of its epidemiology, neurobiology, and potential therapeutic strategies has begun to contribute to a better approach to identifying and treating it. At present, idiopathic hypersomnia is considered an orphan disease with unknown frequency and the cause is unknown; however, there is evidence to suggest circadian and sleep structure differences, structural brain changes, and neurochemical changes may contribute to the development and expression of this disease. The approach to treatment can be challenging owing to a limited number of approved treatments (calcium, magnesium, potassium, and sodium oxybates) in idiopathic hypersomnia. However, consideration of therapies shown to improve excessive daytime sleepiness in other disorders is frequently employed. Future directions require a clear consensus on the defining characteristics of idiopathic hypersomnia to enhance the opportunity for improved recognition, diagnosis, and treatment strategies to be established. This article provides a historical review of the evolving diagnostic classification of idiopathic hypersomnia, potential insights to the underlying pathophysiology, and a summary of proposed approaches for diagnosis and therapeutic intervention.

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Authors and Affiliations

  1. Department of Child Neurology and Pediatric Sleep Medicine, Geisinger Commonwealth School of Medicine, Geisinger Medical Center, Janet Weis Children’s Hospital, 100 N. Academy Ave, MC 14-05, Danville, PA, 17820, USA

    Anne Marie Morse

  2. Department of Sleep Medicine, Pulmonary and Critical Care, Geisinger Wyoming Valley Medical Center, Wilkes-Barre, PA, USA

    Sreelatha Naik

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Correspondence to Anne Marie Morse.

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AMM has served as a consultant for Jazz Pharmaceuticals, Harmony Biosciences, and Avadel. She has served as a speaker for Jazz Pharmaceutical and Avadel. She serves on the scientific advisory board for the Sleep Consortium and the Medical Advisory Board for the Hypersomnia Foundation. She is a committee member of the American Academy of Sleep Medicine Public Advocacy Committee. SN has no conflicts of interest to disclose.

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AMM conceptualized, drafted, and revised this manuscript. SN conceptualized, drafted, and revised this manuscript. All authors have read and approved the final version of the manuscript, and agree to be accountable for the work.

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Morse, A.M., Naik, S. Idiopathic Hypersomnia: Neurobiology, Diagnosis, and Management. CNS Drugs 37, 305–322 (2023). https://doi.org/10.1007/s40263-023-00998-6

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