Clinical and Demographic Characteristics Related to Onset Site and Spread of Cervical Dystonia

• Type of Material: Article
• Authors: Scott A. Norris, Washington University; Hyder Jinnah, Emory University; Alberto J. Espay, University of Cincinnati; Christine Klein, University of Luebeck; Norbert Brueggemann, University of Luebeck; Richard L. Barbano, University of Rochester; Irene Malaty, University of Florida; Ramon L. Rodriguez, University of Florida; Marie Vidailhet, Sorbonne Universités; Emmanuel Roze, Sorbonne Universités; Stephen G. Reich, University of Maryland; Brian D. Berman, University of Colorado; Mark S. LeDoux, University of Tennessee; Sarah Pirio Richardson, University of New Mexico; Pinky Agarwal, Booth Gardner Parkinson's Care Center; Zoftan Mari, Johns Hopkins University; William Ondo, Houston Methodist; Ludy C. Shih, Beth Israel Deaconess Medical Center; Susan Fox, University of Toronto; Alfredo Berardelli, Sapienza University of Rome; Claudia M. Testa, Virginia Commonwealth University; Florence C.F. Cheng, West-mead Hospital; Daniel Truong, The Parkinson and Movement Disorder Institute; Fatta Nahab, University of California, San Diego; Tao Xie, University of Chicago; Mark Hallett, National Institutes of Health; Ami Rosen, Emory University; Laura J. Wright, Washington University; J.S. Perlmutter, Washington University
• Language: English
• Date: 2016-12-01
• Publisher: Wiley
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2016 International Parkinson and Movement Disorder Society
• Final Published Version (URL): http://dx.doi.org/10.1002/mds.26817
• Title of Journal or Parent Work: Movement Disorders
• ISSN: 0885-3185
• Volume: 31
• Issue: 12
• Start Page: 1874
• End Page: 1882
• Grant/Funding Information: The study was additionally supported by grants from the NIH NINDS (NS065701, NS058714, NS41509, NS075321), Murphy Fund, American Parkinson Disease Association (APDA) Center for Advanced PD Research at Washington University; Greater St. Louis Chapter of the APDA, McDonnell Center for Higher Brain Function; and the Barnes-Jewish Hospital Foundation (Elliot Stein Family Fund and the Parkinson Disease Research Fund).; Dystonia Coalition (U54NS065701 & U54TR001456), a part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), NCATS. The Dystonia Coalition is funded through collaboration between NCATS and the National Institute of Neurological Diseases and Stroke (NINDS).
• Keywords: focal; SYMPTOMS; IDIOPATHIC TORSION DYSTONIA; spasmodic torticollis; Life Sciences & Biomedicine; Clinical Neurology; FEATURES; BLEPHAROSPASM; FOCAL DYSTONIA; MULTICENTER; Neurosciences & Neurology; TORTICOLLIS; MOVEMENT-DISORDERS; RISK; neck; TREMOR; geste antagoniste; segmental; Science & Technology
• Research Categories: Biology, Genetics; Biology, Neuroscience

Abstract

Background: Clinical characteristics of isolated idiopathic cervical dystonia such as onset site and spread to and from additional body regions have been addressed in single-site studies with limited data and incomplete or variable dissociation of focal and segmental subtypes. The objectives of this study were to characterize the clinical characteristics and demographics of isolated idiopathic cervical dystonia in the largest standardized multicenter cohort. Methods: The Dystonia Coalition, through a consortium of 37 recruiting sites in North America, Europe, and Australia, recruited 1477 participants with focal (60.7%) or segmental (39.3%) cervical dystonia on examination. Clinical and demographic characteristics were evaluated in terms of the body region of dystonia onset and spread. Results: Site of dystonia onset was: (1) focal neck only (78.5%), (2) focal onset elsewhere with later segmental spread to neck (13.3%), and (3) segmental onset with initial neck involvement (8.2%). Frequency of spread from focal cervical to segmental dystonia (22.8%) was consistent with prior reports, but frequency of segmental onset with initial neck involvement was substantially higher than the 3% previously reported. Cervical dystonia with focal neck onset, more than other subtypes, was associated with spread and tremor of any type. Sensory tricks were less frequent in cervical dystonia with segmental components, and segmental cervical onset occurred at an older age. Conclusions: Subgroups had modest but significant differences in the clinical characteristics that may represent different clinical entities or pathophysiologic subtypes. These findings are critical for design and implementation of studies to describe, treat, or modify disease progression in idiopathic isolated cervical dystonia. © 2016 International Parkinson and Movement Disorder Society.