title: Clinical and demographic characteristics related to onset site and spread of cervical dystonia.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Scott A
authors.initials: SA
authors.lastname: Norris
authors.affiliation: Departments of Neurology and Human Genetics, Emory University, Atlanta, Georgia, USA.
authors.forename: H A
authors.initials: HA
authors.lastname: Jinnah
authors.affiliation: James J. and Joan A. Gardner Family Center for Parkinson's Disease and Movement Disorders, Department of Neurology, University of Cincinnati, Cincinnati, Ohio, USA.
authors.forename: Alberto J
authors.initials: AJ
authors.lastname: Espay
authors.affiliation: Institute of Neurogenetics and Department of Neurology, University of Luebeck, Luebeck, Germany.
authors.forename: Christine
authors.initials: C
authors.lastname: Klein
authors.affiliation: Institute of Neurogenetics and Department of Neurology, University of Luebeck, Luebeck, Germany.
authors.forename: Norbert
authors.initials: N
authors.lastname: Brüggemann
authors.affiliation: Department of Neurology, University of Rochester, Rochester, New York, USA.
authors.forename: Richard L
authors.initials: RL
authors.lastname: Barbano
authors.affiliation: Department of Neurology, University of Florida, Gainesville, Florida, USA.
authors.forename: Irene Andonia C
authors.initials: IA
authors.lastname: Malaty
authors.affiliation: Department of Neurology, University of Florida, Gainesville, Florida, USA.
authors.forename: Ramon L
authors.initials: RL
authors.lastname: Rodriguez
authors.affiliation: Hôpital Universitaire Pitié-Salpêtrière, Sorbonne Universités, Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Vidailhet
authors.affiliation: Hôpital Universitaire Pitié-Salpêtrière, Sorbonne Universités, Paris, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Roze
authors.affiliation: Department of Neurology, University of Maryland, Baltimore, Maryland, USA.
authors.forename: Stephen G
authors.initials: SG
authors.lastname: Reich
authors.affiliation: Department of Neurology, University of Colorado Denver School of Medicine, Aurora, Colorado, USA.
authors.forename: Brian D
authors.initials: BD
authors.lastname: Berman
authors.affiliation: Departments of Neurology and Anatomy and Neurobiology, University of Tennessee Health Science Center, Memphis, Tennessee, USA.
authors.forename: Mark S
authors.initials: MS
authors.lastname: LeDoux
authors.affiliation: Department of Neurology, University of New Mexico, Albuquerque, New Mexico, USA.
authors.forename: Sarah Pirio
authors.initials: SP
authors.lastname: Richardson
authors.affiliation: Booth Gardner Parkinson's Care Center, Kirkland, Washington, USA.
authors.forename: Pinky
authors.initials: P
authors.lastname: Agarwal
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
authors.forename: Zoltan
authors.initials: Z
authors.lastname: Mari
authors.affiliation: Department of Neurology, Houston Methodist, Houston Texas, USA.
authors.forename: William G
authors.initials: WG
authors.lastname: Ondo
authors.affiliation: Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
authors.forename: Ludy C
authors.initials: LC
authors.lastname: Shih
authors.affiliation: Division of Neurology, Department of Medicine, University of Toronto, University Health Network, Toronto, Ontario, Canada.
authors.forename: Susan H
authors.initials: SH
authors.lastname: Fox
authors.affiliation: Department of Neurology and Psychiatry, Sapienza University of Rome and IRCCS Neuromed, Italy.
authors.forename: Alfredo
authors.initials: A
authors.lastname: Berardelli
authors.affiliation: Department of Neurology, Virginia Commonwealth University, Richmond, Virginia, USA.
authors.forename: Claudia M
authors.initials: CM
authors.lastname: Testa
authors.affiliation: Neurology Department, West-mead Hospital, Westmead, Australia.
authors.forename: Florence Ching-Fen
authors.initials: FC
authors.lastname: Cheng
authors.affiliation: The Parkinson and Movement Disorder Institute, Fountain Valley, California, USA.
authors.forename: Daniel
authors.initials: D
authors.lastname: Truong
authors.affiliation: Department of Neurosciences, University of California, San Diego, California, USA.
authors.forename: Fatta B
authors.initials: FB
authors.lastname: Nahab
authors.affiliation: Department of Neurology, University of Chicago Medical Center, Chicago, Illinois, USA.
authors.forename: Tao
authors.initials: T
authors.lastname: Xie
authors.affiliation: Human Motor Control Section, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: Mark
authors.initials: M
authors.lastname: Hallett
authors.affiliation: Department of Neurology, Emory University, Atlanta, Georgia, USA.
authors.forename: Ami R
authors.initials: AR
authors.lastname: Rosen
authors.affiliation: Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Laura J
authors.initials: LJ
authors.lastname: Wright
authors.affiliation: Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.|Departments of Psychiatry, Radiology, Neurobiology, Physical Therapy, and Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Joel S
authors.initials: JS
authors.lastname: Perlmutter
mesh_terms: D000328:Adult; D000368:Aged; D016208:Databases, Factual; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D014103:Torticollis / Q000145:classification / Q000453:epidemiology* / Q000503:physiopathology*
keywords: focal; geste antagoniste; neck; segmental; spasmodic torticollis