Neuroimaging in Narcolepsy and Idiopathic Hypersomnia: from Neural Correlates to Clinical Practice
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HYPERSOMNIA DISORDERS (DT PLANTE, SECTION EDITOR)
Neuroimaging in Narcolepsy and Idiopathic Hypersomnia: from Neural Correlates to Clinical Practice Jari K. Gool 1,2,3 & Nathan Cross 4,5 Thien Thanh Dang-Vu 4,5
&
Rolf Fronczek 2,3 & Gert Jan Lammers 2,3 & Ysbrand D. van der Werf 1 &
Accepted: 15 October 2020 / Published online: 10 November 2020 # Springer Nature Switzerland AG 2020
Abstract Purpose of Review Clinical presentation of central hypersomnolence disorders, including narcolepsy type 1 and 2 and idiopathic hypersomnia, is often similar, and determining the correct diagnosis remains challenging. Neuroimaging techniques have provided valuable insights into the pathophysiology of narcolepsy and idiopathic hypersomnia. Here, we review current structural and functional brain imaging findings in central hypersomnolence disorders and discuss the future perspectives of neuroimaging in these sleep disorders. Recent Findings Most studies have focused on narcolepsy type 1 (or narcolepsy with cataplexy), showing inconsistent but extensive structural differences in the hypothalamus and its normally widespread projections. Functional studies have mainly focused on resting-state or emotion regulation in narcolepsy type 1 and have revealed disturbed activity in limbic and mesolimbic structures in relation to cataplexy. Finally, recent studies suggest a disruption of the default-mode network in patients with idiopathic hypersomnia. Summary Most neuroimaging studies to date have been conducted in small samples, while narcolepsy type 2 (or narcolepsy without cataplexy) and idiopathic hypersomnia remain relatively understudied. Larger studies with consistent clinical phenotyping should be the focus of future investigations. In addition, multi-modal imaging methods will be crucial to resolve previous inconsistencies and identify reliable objective biomarkers that could aid in understanding the pathophysiology and potentially support the diagnostic process. Keywords Narcolepsy . Idiopathic hypersomnia . Neuroimaging . Hypocretin . Hypothalamus Jari K. Gool, Nathan Cross, Ysbrand D. van der Werf and Thien Thanh Dang-Vu contributed equally to this work. This article is part of the Topical Collection on Hypersomnia Disorders Research agenda - Multi-center studies with larger samples of people with NT1, NT2, and IH are required to directly compare the types of central hypersomnolence and reassess the current diagnostic classification - More detailed clinical phenotyping and neuropsychological assessment of patient groups is needed in neuroimaging studies - Multi-modal imaging modalities can provide comprehensive knowledge of the multiple pathophysiological processes underlying central disorders of hypersomnolence - Investigation of brain function during resting state and sleep stages is essential to understand the neural mechanisms of NT1, NT2, or IH and requires an objective monitoring of vigilance - Tailored task-based fMRI studies on daytime symptoms (e.g., vigilance, memory encoding, and reward processing) are important to c
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