Journal
SLEEP
Volume 41, Issue 5, Pages -Publisher
OXFORD UNIV PRESS INC
DOI: 10.1093/sleep/zsy026
Keywords
cataplexy; pseudocataplexy; cataplectic facies; functional neurological symptom disorder; narcolepsy
Categories
Funding
- Swiss National Research Foundation - Clinical Research Priority Program Sleep and Health of the University of Zurich
- HSM Program of the Canton of Zurich
- UCB Pharma
- AbbVie
- NIHR RfPB
- MRC Welcome Strategic grant [WT089698]
- Horizon EC grant Propag-Aging
- Ipsen pharmaceutical company
- Merz pharmaceutical company
- Allergan pharmaceutical company
- Sun Pharma pharmaceutical company
- Teva Lundbeck pharmaceutical company
- Medical Research Council
- National Institutes of Health Research UK
- Merz Pharma
- Medtronic
- Netherlands Organization for Scientific Research [016.116.371]
- Jazz
- Bioprojet
- Theranexus
- Flamel
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Study Objectives: To describe the motor pattern of cataplexy and to determine its phenomenological differences from pseudocataplexy in the differential diagnosis of episodic falls. Methods: We selected 30 video-recorded cataplexy and 21 pseudocataplexy attacks in 17 and 10 patients evaluated for suspected narcolepsy and with final diagnosis of narcolepsy type 1 and conversion disorder, respectively, together with self-reported attacks features, and asked expert neurologists to blindly evaluate the motor features of the attacks. Video documented and self-reported attack features of cataplexy and pseudocataplexy were contrasted. Results: Video-recorded cataplexy can be positively differentiated from pseudocataplexy by the occurrence of facial hypotonia (ptosis, mouth opening, tongue protrusion) intermingled by jerks and grimaces abruptly interrupting laughter behavior (i.e. smile, facial expression) and postural control (head drops, trunk fall) under clear emotional trigger. Facial involvement is present in both partial and generalized cataplexy. Conversely, generalized pseudocataplexy is associated with persistence of deep tendon reflexes during the attack. Self-reported features confirmed the important role of positive emotions (laughter, telling a joke) in triggering the attacks, as well as the more frequent occurrence of partial body involvement in cataplexy compared with pseudocataplexy. Conclusions: Cataplexy is characterized by abrupt facial involvement during laughter behavior. Video recording of suspected cataplexy attacks allows the identification of positive clinical signs useful for diagnosis and, possibly in the future, for severity assessment.
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