Neuroimaging in Narcolepsy and Idiopathic Hypersomnia: from Neural Correlates to Clinical Practice

Jari K. Gool, Nathan Cross, Rolf Fronczek, Gert Jan Lammers, Ysbrand D. van der Werf, Thien Thanh Dang-Vu

Research output: Contribution to journalReview articleAcademicpeer-review

3 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)251-266
Number of pages16
JournalCurrent Sleep Medicine Reports
Issue number4
Publication statusPublished - Dec 2020


  • Hypocretin
  • Hypothalamus
  • Idiopathic hypersomnia
  • Narcolepsy
  • Neuroimaging

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