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Tremor-Dominant Cervical Dystonia: a Cerebellar Syndrome

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Abstract

The objective of this study is to examine the role of the cerebellum in the tremor-dominant subtype of cervical dystonia (CD). CD patients with head tremor at onset (Tr-CD) were age- and sex-matched to CD patients without head tremor at onset (nTr-CD). All patients were evaluated for cerebellar disability using the Scale for the Assessment and Rating of Ataxia (SARA), gait variability using ProtoKinetics Zeno Walkway, and cerebellar volume analysis extracted from brain magnetic resonance imaging (MRI) using a semiquantitative scale. Compared to nTr-CD (n = 10, median age, 70.5 years), Tr-CD patients (n = 10, 71.5 years) exhibited higher median SARA scores (9 vs 7.5, p = 0.03) and greater median gait variability index (131 vs 124, p = 0.03). SARA scores inversely correlated with cerebellar volume in all patients (− 0.4, p = 0.04). Tr-CD patients exhibited greater superior vermian atrophy than nTr-CD patients (p = 0.01). Head tremor at onset heralds a CD subtype with prominent axial cerebellar disability and atrophy of the superior vermis of the cerebellum.

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Funding

The study was funded by the Dystonia Medical Research Foundation Clinical Fellowship award.

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Authors and Affiliations

  1. Section of Movement Disorders, Rush University Medical Center, Chicago, IL, USA

    Abhimanyu Mahajan

  2. Department of Neurology, James J. and Joan A. Gardner Center for Parkinson’s Disease and Movement Disorders, University of Cincinnati, 260 Stetson Street, Suite 2300, Cincinnati, OH, 45219, USA

    Abhimanyu Mahajan, Lyndsey Schroder & Alberto J. Espay

  3. Department of Radiology, University of Cincinnati, Cincinnati, OH, USA

    Aleksander Rekhtman & Lily L. Wang

  4. Department of Molecular and Translational Medicine, Division of Biostatistics and Epidemiology, Texas Tech University Health Sciences Center, El Paso, TX, USA

    Alok K Dwivedi

Authors
  1. Abhimanyu Mahajan
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  2. Lyndsey Schroder
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  3. Aleksander Rekhtman
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  4. Alok K Dwivedi
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  5. Lily L. Wang
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  6. Alberto J. Espay
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Contributions

AM: Research project: conception, organization, execution; statistical analysis: design, review, and critique; manuscript preparation: writing of the first draft, review, and critique

LS: Research project: organization, execution; manuscript preparation: review and critique

AR: Research project: organization, execution; manuscript preparation: review and critique

AD: Research project: conception, statistical analysis: design, execution; manuscript preparation: review and critique

LW: Research project: conception, organization, execution; manuscript preparation: review and critique

AJE: Research project: conception, organization, execution; statistical analysis: review and critique; manuscript preparation: review and critique

Corresponding author

Correspondence to Alberto J. Espay.

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Financial Disclosure Related to Research Covered in this Article

Dr. Mahajan received grant support from the Dystonia Medical Research Foundation for the conduct of this study. He reports no conflict of interest.

Ms. Schroder reports no relevant financial disclosures or conflicts of interest.

Dr. Dwivedi reports no relevant financial disclosures or conflicts of interest.

Dr. Wang reports no relevant financial disclosures or conflicts of interest.

Dr. Espay reports no relevant financial disclosures or conflicts of interest.

Full Financial Disclosure for the Previous 12 Months

Dr. Mahajan has received grant support from the Dystonia Medical Research Foundation, Sunflower Parkinson’s disease Foundation and the Parkinson’s Foundation.

Ms. Schroder has nothing to disclose.

Dr. Rekhtman has nothing to disclose.

Dr. Dwivedi is currently supported as a co-investigator by the NIH (1R21HL143030-01) and (R21 AI133207) grants. He is also currently serving as a statistician in CPRIT-funded studies (PP200006, PP190058, PP180003, and PP170068). Dr. Dwivedi is also an Adjunct Associate Professor in the Department of Neurology and Rehabilitation Medicine, University of Cincinnati, Cincinnati, Ohio.

Dr. Wang is receiving grant support from the NIH as a central imaging core lab reader (NS103824-01) and UC Gardner Neuroscience Institute.

Dr. Espay has received grant support from the NIH and the Michael J Fox Foundation; personal compensation as a consultant/scientific advisory board member for AbbVie, Neuroderm, Neurocrine, Amneal, Adamas, Acadia, Acorda, InTrance, Sunovion, Lundbeck, and US WorldMeds; publishing royalties from Lippincott Williams & Wilkins, Cambridge University Press, and Springer; and honoraria from US WorldMeds, Acadia, and Sunovion.

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Mahajan, A., Schroder, L., Rekhtman, A. et al. Tremor-Dominant Cervical Dystonia: a Cerebellar Syndrome. Cerebellum 20, 300–305 (2021). https://doi.org/10.1007/s12311-020-01211-w

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  • DOI: https://doi.org/10.1007/s12311-020-01211-w

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