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Clinical and demographic characteristics related to onset site and spread of cervical dystonia
Author(s) -
Norris Scott A.,
Jinnah H. A.,
Espay Alberto J.,
Klein Christine,
Brüggemann Norbert,
Barbano Richard L.,
Malaty Irene Andonia C.,
Rodriguez Ramon L.,
Vidailhet Marie,
Roze Emmanuel,
Reich Stephen G.,
Berman Brian D.,
LeDoux Mark S.,
Richardson Sarah Pirio,
Agarwal Pinky,
Mari Zoltan,
Ondo William G.,
Shih Ludy C.,
Fox Susan H.,
Berardelli Alfredo,
Testa Claudia M.,
Cheng Florence ChingFen,
Truong Daniel,
Nahab Fatta B.,
Xie Tao,
Hallett Mark,
Rosen Ami R.,
Wright Laura J.,
Perlmutter Joel S.
Publication year - 2016
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.26817
Subject(s) - cervical dystonia , focal dystonia , dystonia , medicine , age of onset , cohort , demographics , pediatrics , disease , psychiatry , demography , sociology
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.