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Author Notes:

Corresponding Author: Scott A Norris, MD. Washington University School of Medicine, Department of Neurology, Campus Box 8111, 660 S. Euclid Avenue, St. Louis, MO. 63110, USA (norriss@npg.wustl.edu). P: 314-362-7782; F: 314-362-0168.

Scott Norris performed all statistical analyses, wrote the first draft and revised the final version critically for important intellectual content.

Alberto Espay contributed to generation of figures.

Joel Perlmutter and H. A. Jinnah conceived the Dystonia Coalition and managed activities and supervised clinical data collection for this study.

All authors contributed to data collection, data analysis, drafting, and/or revising the manuscript critically for important intellectual content, and approved the final version of the manuscript.

All collaborators in the Dystonia Coalition contributed to data collection and site investigators assisted with data analysis.

See publication for full list of members of the Dystonia Coalition.

See publication for full list of disclosures.

Subjects:

Research Funding:

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).

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).

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Clinical Neurology
  • Neurosciences & Neurology
  • spasmodic torticollis
  • focal
  • segmental
  • geste antagoniste
  • neck
  • IDIOPATHIC TORSION DYSTONIA
  • MOVEMENT-DISORDERS
  • FOCAL DYSTONIA
  • TREMOR
  • BLEPHAROSPASM
  • RISK
  • TORTICOLLIS
  • MULTICENTER
  • SYMPTOMS
  • FEATURES

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

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Journal Title:

Movement Disorders

Volume:

Volume 31, Number 12

Publisher:

, Pages 1874-1882

Type of Work:

Article | Post-print: After Peer Review

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.

Copyright information:

© 2016 International Parkinson and Movement Disorder Society

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