Fabio Pizza1,2, Elena Antelmi1,2, Stefano Vandi1,2, Stefano Meletti3, Roberto Erro4,5, Christian R Baumann6, Kailash P Bhatia4, Yves Dauvilliers7, Mark J Edwards4,8, Alex Iranzo9, Sebastiaan Overeem10,11, Michele Tinazzi12, Rocco Liguori1,2, Giuseppe Plazzi1,2. 1. Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy. 2. IRCCS Institute of the Neurological Sciences, Ospedale Bellaria, AUSL di Bologna, Bologna, Italy. 3. Department of Biomedical, Metabolic, and Neural Sciences, University of Modena and Reggio Emilia, NOCSAE Hospital, Modena, Italy. 4. Sobell Department of Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, United Kingdom. 5. Department of Medicine, Center for Neurodegenerative Diseases (CEMAND), Neuroscience Section, University of Salerno, Italy. 6. Department of Neurology, University Hospital Zurich, Zurich, Switzerland. 7. Department of Neurology, Reference National Center for Narcolepsy, Sleep Unit, Gui-de-Chauliac Hospital, University of Montpellier 1, INSERM U1061, Montpellier, France. 8. St George's University of London, Cranmer Terrace, London, United Kingdom. 9. Hospital Clinic de Barcelona, Neurology Service, Multidisciplinary Sleep Unit, IDIBAPS, CIBERNED, Barcelona, Spain. 10. Sleep Medicine Center Kempenhaeghe, Heeze, The Netherlands. 11. Eindhoven University of Technology, Eindhoven, The Netherlands. 12. Department of Neuroscience, Biomedicine and Movement Science, University of Verona, Verona, Italy.
Abstract
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.
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.