K Feil1, N Böttcher2, F Guri3, S Krafczyk4, F Schöberl5, A Zwergal6, M Strupp7. 1. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany; German Center for Vertigo and Balance Disorders, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: katharina.feil@med.uni-muenchen.de. 2. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany; German Center for Vertigo and Balance Disorders, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: Nicolina.boettcher@med.uni-muenchen.de. 3. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: Flavia.guri@med.uni-muenchen.de. 4. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany; German Center for Vertigo and Balance Disorders, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: Siegbert.krafczyk@med.uni-muenchen.de. 5. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany; German Center for Vertigo and Balance Disorders, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: Florian.schoeberl@med.uni-muenchen.de. 6. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany; German Center for Vertigo and Balance Disorders, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: Andreas.zwergal@med.uni-muenchen.de. 7. Department of Neurology, University Hospital Munich, Campus Grosshadern, Munich, Germany; German Center for Vertigo and Balance Disorders, University Hospital Munich, Campus Grosshadern, Munich, Germany. Electronic address: Michael.strupp@med.uni-muenchen.de.
Abstract
OBJECTIVE: Primary orthostatic tremor (OT) is a rare neurological disease of unknown pathophysiology characterized by a high-frequency tremor mainly of the legs when standing. The aim of this study was to examine its long-term course by subjective estimation and objective recording by serial posturography and to obtain further standardized epidemiological and clinical data on patients with OT. METHODS: A clinical cohort of 37 patients with the diagnosis of primary OT was screened for this longitudinal follow-up study. Eighteen patients consented to participate. During study visit all patients underwent a standardized neurological examination and completed subjective scales and scores. Posturographic recordings at follow-up were compared to prior clinical posturographic measurements in 15 cases. RESULTS: In our cohort the mean duration of symptoms was 14.1 ± 6.8 years. Subjectively, 78% of patients reported progression of the disease. Posturographic data (5.4 ± 4.0 years) revealed a significant increase of the total sway path (standing on firm ground with eyes open) from 2.4 ± 1.3 to 3.4 ± 1.4 m/min (p = 0.022) and of the total root mean square values from 9.8 ± 4.3 to 12.4 ± 4.8 mm (p = 0.028). None of these observations are explained by aging of the patients. Mean frequency of the tremor did not change over time (14.7 ± 1.9 Hz vs. 14.9 ± 2.0 Hz at follow-up). Clinically, most patients had signs of cerebellar dysfunction and a substantial portion also showed proprioceptive deficits in the long-term course. CONCLUSIONS: This long-term follow-up study indicates, that primary OT is a progressive disorder. Furthermore, the clinical observation of cerebellar dysfunction in most OT patients in the long-term course might indicate an important role of the cerebellum in its pathophysiology.
OBJECTIVE:Primary orthostatic tremor (OT) is a rare neurological disease of unknown pathophysiology characterized by a high-frequency tremor mainly of the legs when standing. The aim of this study was to examine its long-term course by subjective estimation and objective recording by serial posturography and to obtain further standardized epidemiological and clinical data on patients with OT. METHODS: A clinical cohort of 37 patients with the diagnosis of primary OT was screened for this longitudinal follow-up study. Eighteen patients consented to participate. During study visit all patients underwent a standardized neurological examination and completed subjective scales and scores. Posturographic recordings at follow-up were compared to prior clinical posturographic measurements in 15 cases. RESULTS: In our cohort the mean duration of symptoms was 14.1 ± 6.8 years. Subjectively, 78% of patients reported progression of the disease. Posturographic data (5.4 ± 4.0 years) revealed a significant increase of the total sway path (standing on firm ground with eyes open) from 2.4 ± 1.3 to 3.4 ± 1.4 m/min (p = 0.022) and of the total root mean square values from 9.8 ± 4.3 to 12.4 ± 4.8 mm (p = 0.028). None of these observations are explained by aging of the patients. Mean frequency of the tremor did not change over time (14.7 ± 1.9 Hz vs. 14.9 ± 2.0 Hz at follow-up). Clinically, most patients had signs of cerebellar dysfunction and a substantial portion also showed proprioceptive deficits in the long-term course. CONCLUSIONS: This long-term follow-up study indicates, that primary OT is a progressive disorder. Furthermore, the clinical observation of cerebellar dysfunction in most OT patients in the long-term course might indicate an important role of the cerebellum in its pathophysiology.
Authors: Pierre Cabaraux; Sunil K Agrawal; Huaying Cai; Rocco Salvatore Calabro; Carlo Casali; Loic Damm; Sarah Doss; Christophe Habas; Anja K E Horn; Winfried Ilg; Elan D Louis; Hiroshi Mitoma; Vito Monaco; Maria Petracca; Alberto Ranavolo; Ashwini K Rao; Serena Ruggieri; Tommaso Schirinzi; Mariano Serrao; Susanna Summa; Michael Strupp; Olivia Surgent; Matthis Synofzik; Shuai Tao; Hiroo Terasi; Diego Torres-Russotto; Brittany Travers; Jaimie A Roper; Mario Manto Journal: Cerebellum Date: 2022-04-12 Impact factor: 3.847
Authors: Julián Benito-León; Elan D Louis; Virginia Mato-Abad; Ulrike Dydak; Juan Álvarez-Linera; Juan Antonio Hernández-Tamames; José Antonio Molina-Arjona; Norberto Malpica; Michele Matarazzo; Juan Pablo Romero; Álvaro Sánchez-Ferro Journal: Medicine (Baltimore) Date: 2016-09 Impact factor: 1.889