The Conundrum of Dystonia in Essential Tremor Patients: How does One Classify these Cases?

Tremor and Other Hyperkinetic Movements

article

Wenqin Du¹  Peter G. Bain²  Giovanni Defazio³  Joseph Jankovic⁴  Christine Y. Kim⁵  E. K. Tan⁶  Marie Vidailhet⁷  Elan D. Louis¹ 

[1] Department of Neurology, University of Texas Southwestern Medical Center;Department of Neurosciences, Division of Brain Sciences, Imperial College London;Department of Medical Sciences and Public Health, University of Cagliari;Parkinson’s Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine;Department of Neurology, Columbia University Medical Center;Department of Neurology, National Neuroscience Institute, Singapore General Hospital;Sorbonne Université, Institut du Cerveau – Paris Brain Institute – ICM, Inserm, CNRS, AP-HP, Hôpital Salpetriere, DMU Neuroscience 6

关键词: essential tremor;    dystonia;    diagnosis;    classification;   

DOI  :  10.5334/tohm.690

学科分类：社会科学、人文和艺术（综合）

来源: Ubiquity Press

【 摘 要 】

Background: The relationship between essential tremor (ET) and dystonia has been long debated and the boundaries between these disorders remain unclear. Here, we highlight the diagnostic uncertainty that can arise when observing dystonic postures in patients who have received ET diagnoses.Methods: An international panel of seven movement disorders neurologists from five countries reviewed the clinical history and videotaped neurological examinations of five individuals diagnosed with ET who also had various features of dystonia on neurological examination. Experts were instructed to assign diagnoses and provide their rationale for diagnostic assignments.Results: The five cases each exhibited a variety of abnormal postures. These were observed by all experts, and interpreted as dystonic postures by six experts. According to six of seven experts, all five cases had ET. One expert classified all cases as dystonic tremor rather than ET. One case had cervical dystonia, and five of seven experts assigned dual diagnoses of ET and dystonia in that case. The assignment of dystonia diagnoses was variable among the other four cases, with two to three experts assigning this diagnosis in each case, underscoring differences in diagnostic interpretation of dystonic postures on examination.Conclusions: This study draws attention to some of the differences between experts in assigning diagnoses of ET or dystonia to individuals with ET and abnormal postures. The goal here was not necessarily to build consensus, but to raise issues, highlight areas of uncertainty, and identify areas of common vs. differentiated thought. Several questions for additional research were also raised.

【 授权许可】

CC BY