Christos Ganos1, Lucie Maugest2, Emmanuelle Apartis3, Carmen Gasca-Salas4, María T Cáceres-Redondo5, Roberto Erro6, Irene Navalpotro-Gómez7, Amit Batla6, Elena Antelmi6, Bertrand Degos8, Emmanuel Roze9, Marie-Laure Welter9, Tiago Mestre10, Francisco J Palomar5, Reina Isayama11, Robert Chen12, Carla Cordivari13, Pablo Mir14, Anthony E Lang4, Susan H Fox4, Kailash P Bhatia6, Marie Vidailhet9. 1. Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, UK Department of Neurology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany. 2. Department of Neurology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France CRICM UPMC/INSERM UMR 1127 CNRS UMR7225 Brain and Spine Institute, Paris, France. 3. CRICM UPMC/INSERM UMR 1127 CNRS UMR7225 Brain and Spine Institute, Paris, France Neurophysiology Unit, AP-HP, Saint-Antoine Hospital, Paris, France Pierre Marie Curie Paris-6 University, Paris, France. 4. Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson's Disease, University Health Network, Toronto, Canada. 5. Unidad de Trastornos del Movimiento, Servicio de Neurología y Neurofisiología Clínica, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla, Seville, Spain. 6. Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, UK. 7. Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, UK Department of Neurology, Hospital del Mar, Barcelona, Spain. 8. Department of Neurology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France Pierre Marie Curie Paris-6 University, Paris, France. 9. Department of Neurology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France CRICM UPMC/INSERM UMR 1127 CNRS UMR7225 Brain and Spine Institute, Paris, France Pierre Marie Curie Paris-6 University, Paris, France. 10. Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson's Disease, University Health Network, Toronto, Canada Parkinson's Disease and Movement Disorder Centre, division of Neurology, department of Medicine, University of Ottawa, The Ottawa Hospital, Ottawa, Canada. 11. Department of Medicine, University of Toronto, Toronto, Canada Division of Brain, Imaging and Behavior-Systems Neuroscience, Toronto Western Research Institute, Toronto, Canada. 12. Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson's Disease, University Health Network, Toronto, Canada Department of Medicine, University of Toronto, Toronto, Canada Division of Brain, Imaging and Behavior-Systems Neuroscience, Toronto Western Research Institute, Toronto, Canada. 13. Department of Clinical Neurophysiology, National Hospital for Neurology and Neurosurgery, London, UK. 14. Unidad de Trastornos del Movimiento, Servicio de Neurología y Neurofisiología Clínica, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla, Seville, Spain Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Spain.
Abstract
OBJECTIVES: Orthostatic tremor is a rare condition characterised by high-frequency tremor that appears on standing. Although the essential clinical features of orthostatic tremor are well established, little is known about the natural progression of the disorder. We report the long-term outcome based on the largest multicentre cohort of patients with orthostatic tremor. METHODS: Clinical information of 68 patients with clinical and electrophysiological diagnosis of orthostatic tremor and a minimum follow-up of 5 years is presented. RESULTS: There was a clear female preponderance (76.5%) with a mean age of onset at 54 years. Median follow-up was 6 years (range 5-25). On diagnosis, 86.8% of patients presented with isolated orthostatic tremor and 13.2% had additional neurological features. At follow-up, seven patients who initially had isolated orthostatic tremor later developed further neurological signs. A total 79.4% of patients reported worsening of orthostatic tremor symptoms. These patients had significantly longer symptom duration than those without reported worsening (median 15.5 vs 10.5 years, respectively; p=0.005). There was no change in orthostatic tremor frequency over time. Structural imaging was largely unremarkable and dopaminergic neuroimaging (DaTSCAN) was normal in 18/19 cases. Pharmacological treatments were disappointing. Two patients were treated surgically and showed improvement. CONCLUSIONS: Orthostatic tremor is a progressive disorder with increased disability although tremor frequency is unchanged over time. In most cases, orthostatic tremor represents an isolated syndrome. Drug treatments are unsatisfactory but surgery may hold promise. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
OBJECTIVES: Orthostatic tremor is a rare condition characterised by high-frequency tremor that appears on standing. Although the essential clinical features of orthostatic tremor are well established, little is known about the natural progression of the disorder. We report the long-term outcome based on the largest multicentre cohort of patients with orthostatic tremor. METHODS: Clinical information of 68 patients with clinical and electrophysiological diagnosis of orthostatic tremor and a minimum follow-up of 5 years is presented. RESULTS: There was a clear female preponderance (76.5%) with a mean age of onset at 54 years. Median follow-up was 6 years (range 5-25). On diagnosis, 86.8% of patients presented with isolated orthostatic tremor and 13.2% had additional neurological features. At follow-up, seven patients who initially had isolated orthostatic tremor later developed further neurological signs. A total 79.4% of patients reported worsening of orthostatic tremor symptoms. These patients had significantly longer symptom duration than those without reported worsening (median 15.5 vs 10.5 years, respectively; p=0.005). There was no change in orthostatic tremor frequency over time. Structural imaging was largely unremarkable and dopaminergic neuroimaging (DaTSCAN) was normal in 18/19 cases. Pharmacological treatments were disappointing. Two patients were treated surgically and showed improvement. CONCLUSIONS: Orthostatic tremor is a progressive disorder with increased disability although tremor frequency is unchanged over time. In most cases, orthostatic tremor represents an isolated syndrome. Drug treatments are unsatisfactory but surgery may hold promise. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
Authors: Christian Blahak; Tamara Sauer; Hansjoerg Baezner; Marc E Wolf; Assel Saryyeva; Christoph Schrader; Hans-Holger Capelle; Michael G Hennerici; Joachim K Krauss Journal: J Neurol Date: 2016-08-13 Impact factor: 4.849