Abstract
Background
Although head tremor (HT) and pain are prevalent in cervical dystonia (CD), their joint relationship to phenotypic features of focal dystonia remains unclear.
Objectives
We examined how severity of HT and pain are associated with age of CD onset and duration, and whether HT subtypes (“jerky” or “regular”) exhibit distinct relationships between severity of HT and pain.
Methods
The severity of HT and pain were assessed with the Toronto Western Spasmodic Torticollis Rating Scale in retrospective review of 188 CD patients recruited through the Dystonia Coalition.
Results
HT severity was associated with longer CD duration (p < 0.0005), whereas pain severity was associated with younger age at onset (p = 0.043). HT severity and pain severity were not correlated for jerky HT (p = 0.996), but positively correlated for regular HT (p = 0.01).
Conclusions
The distinct associations of HT and pain with age at onset, disease duration, and HT subtype further characterize the heterogeneity of CD’s clinical presentation and suggest similarly heterogeneous underlying mechanisms.


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Data availability
Original data available from the Dystonia Coalition upon reasonable request.
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Acknowledgements
We gratefully acknowledge Laura Wright and Matt Hicks (WUSM) for assistance with providing data access.
Funding
This research was conducted via the Dystonia Coalition, which is part of the Rare Diseases Clinical Research Network, an initiative funded by the Office of Rare Diseases Research at the National Center for Advancing Translational Sciences (U54 TR001456) in collaboration with the National Institute of Neurological Disorders and Stroke (U54 NS065701 and U54 NS116025) at the National Institute of Health (NIH). This work was also supported by the Office of the Assistant Secretary of Defense for Health Affairs, through the Peer-Reviewed Medical Research Program under Award No. W81XWH-17-1-0393. Opinions, interpretations, conclusions and recommendations are those of the author and are not necessarily endorsed by the Department of Defense.
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Research project: organization and execution; statistical analysis: review and critique; manuscript preparation: writing of the first draft, review and critique—JPV. Statistical analysis: execution; manuscript preparation: writing of the first draft, review and critique—HYL. Research project: organization and execution; statistical analysis design and execution; manuscript preparation: review and critique—QC. Research project: execution; manuscript preparation: review and critique—EC. Research project: execution; manuscript preparation: review and critique—RLB. Research project: execution; manuscript preparation: review and critique—CGG. Research project: execution; manuscript preparation: review and critique—JJ. Research project: execution; manuscript preparation: review and critique—HAJ. Research project: execution; manuscript preparation: review and critique—JSP. Manuscript preparation: review and critique—BDB. Statistical analysis: design, execution, review and critique; manuscript preparation: review and critique—MIA. Research project: conception, execution; statistical analysis: design, execution, review and critique; manuscript preparation: review and critique—GTS. Research project: conception; statistical analysis: review and critique; manuscript preparation: review and critique—CLC. Research project: conception, organization, execution; statistical analysis: design, execution, review and critique; manuscript preparation: writing of the first draft, review and critique—DAP.
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The Helsinki Declaration is subsumed by "The protocols for this study were approved by the Human Research Protection Offices at the Washington University School of Medicine (WUSM), Rush University Medical Center (RUMC), and the University of California, San Diego (UCSD; protocol 111255X).
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All patients provided informed consent prior to participation.
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Vu, J.P., Lee, H.Y., Chen, Q. et al. Head tremor and pain in cervical dystonia. J Neurol 268, 1945–1950 (2021). https://doi.org/10.1007/s00415-020-10378-5
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DOI: https://doi.org/10.1007/s00415-020-10378-5