Louise Bicart-Sée1, Jean-Luc Thibault1, Aurélia Poujois2, France Woimant3, Floriane Bouquet-Castiglione1, Pierre Lozeron1,4, Nathalie Kubis5,6. 1. Service de Physiologie Clinique-Explorations Fonctionnelles, APHP, Hôpital Lariboisière, 75010, Paris, France. 2. Centre de référence de la Maladie de Wilson et autres maladies rares liees au cuivre, Service de Neurologie, Hôpital Fondation Adolphe de Rothschild, 75019, Paris, France. 3. Centre de référence de la Maladie de Wilson et autres maladies rares liees au cuivre, AP-HP, Hôpital Lariboisière, Service de Neurologie, 75010, Paris, France. 4. Laboratory for Vascular Translational Science, Université de Paris, INSERM U1148, 75018, Paris, France. 5. Service de Physiologie Clinique-Explorations Fonctionnelles, APHP, Hôpital Lariboisière, 75010, Paris, France. nathalie.kubis@aphp.fr. 6. Laboratory for Vascular Translational Science, Université de Paris, INSERM U1148, 75018, Paris, France. nathalie.kubis@aphp.fr.
Abstract
BACKGROUND: Orthostatic tremor (OT) is characterized by tremor in orthostatism. Primary OT is characterized by a high-frequency tremor at surface EMG recording and assumed to be idiopathic, whereas slow-frequency OT is classically associated with neurological pathologies. We report here a retrospective monocentric cohort study of primary (fast OT) and pseudo-OT (slow OT) patients to describe associated neurological and non-neurological co-morbidities. METHODS: Between November 2014 and October 2019, 27 patients with OT were selected from the EMG database of the Department of Clinical Physiology in Lariboisière' s hospital. Patients were classified in primary OT if tremor frequency was ≥ 13 Hz and in pseudo-OT if tremor frequency was < 13 Hz. RESULTS: Leg tremor on standing represented 10.2% of all tremor recordings. Ten patients were included in the primary and 17 in the pseudo-OT group. Females were predominant (62.9%) (p = 0.04). Mean age at diagnosis was 64.8 ± 1.1 years. At the first visit, a movement disorder was associated with 30% of primary OT, among them one CADASIL patient, whereas extrapyramidal or cerebellar disorders were reported in 100% of pseudo-OT, among them three Wilson's disease patients. These pathologies all preceded primary OT and occurred concomitantly with pseudo-OT. Frequency remained unchanged during evolution, except pseudo-OT in two patients that completely resolved following the introduction of antiParkinsonian drugs. Treatment of primary OT was partially effective in 28% and in 50% of pseudo-OT patients. CONCLUSION: In this monocentric study, movement disorders were present in 30% of primary OT patients. This result questions the term "idiopathic" or "primary" OT, but the small number of patients does not allow answering this issue.
BACKGROUND:Orthostatic tremor (OT) is characterized by tremor in orthostatism. Primary OT is characterized by a high-frequency tremor at surface EMG recording and assumed to be idiopathic, whereas slow-frequency OT is classically associated with neurological pathologies. We report here a retrospective monocentric cohort study of primary (fast OT) and pseudo-OT (slow OT) patients to describe associated neurological and non-neurological co-morbidities. METHODS: Between November 2014 and October 2019, 27 patients with OT were selected from the EMG database of the Department of Clinical Physiology in Lariboisière' s hospital. Patients were classified in primary OT if tremor frequency was ≥ 13 Hz and in pseudo-OT if tremor frequency was < 13 Hz. RESULTS:Leg tremor on standing represented 10.2% of all tremor recordings. Ten patients were included in the primary and 17 in the pseudo-OT group. Females were predominant (62.9%) (p = 0.04). Mean age at diagnosis was 64.8 ± 1.1 years. At the first visit, a movement disorder was associated with 30% of primary OT, among them one CADASIL patient, whereas extrapyramidal or cerebellar disorders were reported in 100% of pseudo-OT, among them three Wilson's diseasepatients. These pathologies all preceded primary OT and occurred concomitantly with pseudo-OT. Frequency remained unchanged during evolution, except pseudo-OT in two patients that completely resolved following the introduction of antiParkinsonian drugs. Treatment of primary OT was partially effective in 28% and in 50% of pseudo-OT patients. CONCLUSION: In this monocentric study, movement disorders were present in 30% of primary OT patients. This result questions the term "idiopathic" or "primary" OT, but the small number of patients does not allow answering this issue.
Authors: Christos Ganos; Lucie Maugest; Emmanuelle Apartis; Carmen Gasca-Salas; María T Cáceres-Redondo; Roberto Erro; Irene Navalpotro-Gómez; Amit Batla; Elena Antelmi; Bertrand Degos; Emmanuel Roze; Marie-Laure Welter; Tiago Mestre; Francisco J Palomar; Reina Isayama; Robert Chen; Carla Cordivari; Pablo Mir; Anthony E Lang; Susan H Fox; Kailash P Bhatia; Marie Vidailhet Journal: J Neurol Neurosurg Psychiatry Date: 2015-03-13 Impact factor: 10.154