Intermediate hypocretin-1 cerebrospinal fluid levels and typical cataplexy: their significance in the diagnosis of narcolepsy type 1.

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  • Additional Information
    • Source:
      Publisher: Oxford University Press Country of Publication: United States NLM ID: 7809084 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1550-9109 (Electronic) Linking ISSN: 01618105 NLM ISO Abbreviation: Sleep Subsets: MEDLINE
    • Publication Information:
      Publication: 2017- : New York : Oxford University Press
      Original Publication: New York, Raven Press.
    • Subject Terms:
      Cataplexy*/cerebrospinal fluid ; Cataplexy*/diagnosis ; Narcolepsy*/cerebrospinal fluid ; Narcolepsy*/diagnosis ; Neuropeptides*; Cross-Sectional Studies ; Humans ; Intracellular Signaling Peptides and Proteins ; Orexins/cerebrospinal fluid ; Retrospective Studies
    • Abstract:
      Study Objectives: The diagnosis of narcolepsy type 1 (NT1) is based upon the presence of cataplexy and/or a cerebrospinal fluid (CSF) hypocretin-1/orexin-A level ≤ 110 pg/mL. We determined the clinical and diagnostic characteristics of patients with intermediate hypocretin-1 levels (111-200 pg/mL) and the diagnostic value of cataplexy characteristics in individuals with central disorders of hypersomnolence.
      Methods: Retrospective cross-sectional study of 355 people with known CSF hypocretin-1 levels who visited specialized Sleep-Wake Centers in the Netherlands. For n = 271, we had full data on cataplexy type ("typical" or "atypical" cataplexy).
      Results: Compared to those with normal hypocretin-1 levels (>200 pg/mL), a higher percentage of individuals with intermediate hypocretin-1 levels had typical cataplexy (75% or 12/16 vs 9% or 8/88, p < .05), and/or met the diagnostic polysomnographic (PSG) and Multiple Sleep Latency Test (MSLT) criteria for narcolepsy (50 vs 6%, p < .001). Of those with typical cataplexy, 88% had low, 7% intermediate, and 5% normal hypocretin-1 levels (p < .001). Atypical cataplexy was also associated with hypocretin deficiency but to a lesser extent. A hypocretin-1 cutoff of 150 pg/mL best predicted the presence of typical cataplexy and/or positive PSG and MSLT findings.
      Conclusion: Individuals with intermediate hypocretin-1 levels or typical cataplexy more often have outcomes fitting the PSG and MSLT criteria for narcolepsy than those with normal levels or atypical cataplexy. In addition, typical cataplexy has a much stronger association with hypocretin-1 deficiency than atypical cataplexy. We suggest increasing the NT1 diagnostic hypocretin-1 cutoff and adding the presence of clearly defined typical cataplexy to the diagnostic criteria of NT1. Clinical trial information: This study is not registered in a clinical trial register, as it has a retrospective database design.
      (© The Author(s) 2022. Published by Oxford University Press on behalf of Sleep Research Society. All rights reserved. For permissions, please e-mail: [email protected].)
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    • Contributed Indexing:
      Keywords: cataplexy; central disorders of hypersomnolence; orexin-A; sleep disorder
    • Accession Number:
      0 (Intracellular Signaling Peptides and Proteins)
      0 (Neuropeptides)
      0 (Orexins)
    • Publication Date:
      Date Created: 20220513 Date Completed: 20220517 Latest Revision: 20230920
    • Publication Date:
      20230920
    • Accession Number:
      PMC9113791
    • Accession Number:
      10.1093/sleep/zsac052
    • Accession Number:
      35554594