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

Fabio Pizza, Elena Antelmi, Stefano Vandi, Stefano Meletti, Roberto Erro, Christian R. Baumann, Kailash P. Bhatia, Yves Dauvilliers, Mark J. Edwards, Alex Iranzo, Sebastiaan Overeem, Michele Tinazzi, Rocco Liguori, Giuseppe Plazzi

Onderzoeksoutput: Bijdrage aan tijdschriftTijdschriftartikelAcademicpeer review

33 Citaten (Scopus)

Samenvatting

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.

Originele taal-2Engels
Artikelnummerzsy026
Aantal pagina's9
TijdschriftSleep
Volume41
Nummer van het tijdschrift5
Vroegere onlinedatum7 feb. 2018
DOI's
StatusGepubliceerd - 1 mei 2018

Financiering

Conflicts of interest statement. F.P., E.A., S.V., S.M., A.I., M.T., and R.L. report no disclosures. R.E. received consultancies from Zambon and honoraria from TEVA. C.R.B. received a grant from the Swiss National Research Foundation, is supported by the Clinical Research Priority Program Sleep and Health of the University of Zurich, received a research grant from the HSM Program of the Canton of Zurich, and received unrestricted research grants from UCB Pharma and from AbbVie. None of these grants are related to the present work. K.P.B. receives royalties from Oxford University Press and a stipend for MDCP editorship, holds grants from NIHR RfPB, MRC Welcome Strategic grant (WT089698), Horizon 2020 EC grant Propag-Aging, and has received honoraria/financial support to speak/attend meetings or serve on advisory boards from Ipsen, Merz, Allergan, Sun Pharma, and Teva Lundbeck pharmaceutical companies. Y.D. has received funds for speaking, board engagements, and travel to conferences with UCB Pharma, Jazz, Theranexus, Flamel, and Bioprojet. M.J.E. receives grant support from the Medical Research Council and the National Institutes of Health Research UK and has received grants and honoraria for education activities from Merz Pharma, UCB Pharma, and Medtronic. S.O. is supported by a VIDI research grant from the Netherlands Organization for Scientific Research (grant no.  016.116.371), received an unrestricted research grant from UCB Pharma not related to the present work, and received conference travel support from UCB Pharma, Novartis, and Boehringer Ingelheim. G.P. has received funds for board engagements from UCB Pharma, Jazz, and Bioprojet.

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