The clinical features of cataplexy: a questionnaire study in narcolepsy patients with and without hypocretin-1 deficiency
Abstract
Background Narcolepsy is often not recognized or accurately diagnosed. This may be due to the fact that cataplexy, a core symptom which is virtually 100% specific, can-in practice-only be diagnosed based on the patient's history. However, the current definition of cataplexy is not very precise and the common distinction between "typical" and "atypical" cataplexy is not well codified.Methods We aimed to provide a detailed description of the phenotypic variability of cataplexy. We included 109 patients with a definite history of cataplexy and a proven hypocretin-1 deficiency. The questionnaire contained 37 items to broadly cover the clinical aspects of cataplexy, including triggers, pattern and duration of muscle weakness, associated aspects such as sensory phenomena, and limitations in daily life due to cataplexy.Results "Laughing" only listed in place 11th of most frequent triggers. "Laughing excitedly" was much more potent, showing that a certain intensity of the emotion is important for a "cataplectogenic" effect. Anger was the highest ranking "non-humorous" trigger, followed by "unexpectedly meeting someone well known." About 60% of patients also had spontaneous cataplectic attacks. Forty-five percent of patients experienced both partial and complete attacks and 30% only partial cataplexy. Fifteen percent of complete attacks were reported to last longer than 2 min. An abrupt return of muscle function was an important feature. The jaw and the face were most often involved in partial attacks, even more than the knee or the leg.Conclusions Cataplexy presents with a large phenotypical diversity, so the current "typical" versus "atypical" distinction may be difficult to hold. We propose that grading cataplexy with different levels of diagnostic confidence may be more useful.
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Cataplexy concerns paresis or paralysis triggered by emotions, usually laughter, but also by a range of other triggers.
“Cataplexy concerns paresis or paralysis triggered by emotions, usually laughter, but also by a range of other triggers.”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [427]
Cataplexy is a key feature of narcolepsy; other cardinal symptoms are excessive daytime sleepiness, sleep-onset paralysis, and hypnagogic hallucinations.
“Cataplexy is a key feature of narcolepsy; other cardinal symptoms are excessive daytime sleepiness, sleep-onset paralysis, and hypnagogic hallucinations.”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [427]
In cataplexy, patients are conscious even when considered unconscious by eyewitnesses, and there is no amnesia.
“Patients are conscious even when considered unconscious by eyewitnesses, and there is no amnesia.”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [427]
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- Authors
- Overeem, S., van Nues, S. J., van der Zande, W. L., Donjacour, C. E., van Mierlo, P., & Lammers, G. J.
- Affiliations
- Radboud University Nijmegen (NL)
- Leiden University Medical Center (NL)
- Corresponding author
- Overeem, Sebastiaan
- Venue
- Sleep Medicine
- Publisher
- Elsevier BV
- Published
- 2011-01
- Volume
- 12
- Issue
- 1
- Pages
- 12-18
- ISSN
- 1389-9457 (ISSN-L)
- OpenAlex
- W2070482396
- Cited by
- 144
- References
- 20
- Citations by year
- 2026: 5 · 2025: 4 · 2024: 10 · 2023: 10 · 2022: 12 · 2021: 7 · 2020: 12 · 2019: 12 · 2018: 12 · 2017: 11 · 2016: 12 · 2015: 10 · 2014: 6 · 2013: 11 · 2012: 6
- FWCI
- 3.87
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Sleep Medicine (publishedVersion)
- PubMed (publishedVersion)
- TU/e Research Portal (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Leiden Repository (Leiden University) (submittedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Funders
- VENI grant from the Dutch Organization for Scientific Research (916.56.103)
- Topics
- Life Sciences › Neuroscience › Cognitive Neuroscience › Sleep and Wakefulness Research
- Social Sciences › Psychology › Experimental and Cognitive Psychology › Sleep and related disorders
- Health Sciences › Medicine › Epidemiology › Restless Legs Syndrome Research
- Keywords
- cataplexy, narcolepsy, muscle weakness, diagnostic confidence, phenotypic variability
- MeSH terms
- Orexins, Activities of Daily Living, Adolescent, Adult, Aged, Anger, Cataplexy, Female, Humans, Laughter, Male, Middle Aged, Narcolepsy, Neuropeptides, Phenotype, Surveys and Questionnaires, Intracellular Signaling Peptides and Proteins, Young Adult
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