4.6 Article

The distinguishing motor features of cataplexy: a study from video-recorded attacks

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

Funding

  1. Swiss National Research Foundation - Clinical Research Priority Program Sleep and Health of the University of Zurich
  2. HSM Program of the Canton of Zurich
  3. UCB Pharma
  4. AbbVie
  5. NIHR RfPB
  6. MRC Welcome Strategic grant [WT089698]
  7. Horizon EC grant Propag-Aging
  8. Ipsen pharmaceutical company
  9. Merz pharmaceutical company
  10. Allergan pharmaceutical company
  11. Sun Pharma pharmaceutical company
  12. Teva Lundbeck pharmaceutical company
  13. Medical Research Council
  14. National Institutes of Health Research UK
  15. Merz Pharma
  16. Medtronic
  17. Netherlands Organization for Scientific Research [016.116.371]
  18. Jazz
  19. Bioprojet
  20. Theranexus
  21. 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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