Clinical and Demographic Characteristics of Upper Limb Dystonia

Scott A Norris¹, Hyder A Jinnah², Christine Klein³, Joseph Jankovic⁴, Brian D Berman⁵, Emmanuel Roze^{6

7}, Abhimanyu Mahajan⁸, Alberto J Espay⁸, Avinash V Murthy⁹, Victor S C Fung¹⁰, Mark S LeDoux^{11

12}, Florence C F Chang¹⁰, Marie Vidailhet^{13

14

15}, Claudia Testa¹⁶, Richard Barbano¹⁷, Irene A Malaty¹⁸, Tobias Bäumer³, Sebastian Loens³, Laura J Wright¹⁹, Joel S Perlmutter^{1

20}

Affiliations

¹ Departments of Neurology and Radiology, Washington University School of Medicine, St. Louis, Missouri, USA.
² Departments of Neurology and Human Genetics, Emory University, Atlanta, Georgia, USA.
³ Institute of Neurogenetics and Department of Neurology, University of Luebeck, Luebeck, Germany.
⁴ Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
⁵ Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
⁶ Sorbonne University, Inserm U 1127, National Centre for Scientific Research, Joint Research Units 7225, Institut du Cerveau et de la Moelle épinière and Assistance Public Hôpitaux de Paris, Paris, France.
⁷ Department of Neurology, Pitié-Salpêtrière Hospital, Paris, France.
⁸ James J. and Joan A. Gardner Family Center for Parkinson's Disease and Movement Disorders, Department of Neurology, University of Cincinnati, Cincinnati, Ohio, USA.
⁹ Washington University in St. Louis, St. Louis, Missouri, USA.
¹⁰ Movement Disorders Unit, Neurology Department, Westmead Hospital & Sydney Medical School, University of Sydney, Sydney, Australia.
¹¹ Department of Psychology and School of Health Studies, University of Memphis, Memphis, Tennessee, USA.
¹² Veracity Neurosciences LLC, Memphis, Tennessee, USA.
¹³ Department of Neurology, Salpetriere Hospital, AP-HP, Paris, France.
¹⁴ Department of Neurology, Sorbonne Université, Paris, France.
¹⁵ Brain & Spine Institute, Joint Research Units 1127, INSERM 1127, Center National De Recherche Scientific 7235, Paris, France.
¹⁶ Department of Neurology, Virginia Commonwealth University, Richmond, Virginia, USA.
¹⁷ Department of Neurology, University of Rochester, Rochester, New York, USA.
¹⁸ University of Florida Department of Neurology, Fixel Institute for Neurologic Diseases, Gainesville, Florida, USA.
¹⁹ Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
²⁰ Departments of Neuroscience, Physical Therapy, and Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri, USA.

PMID: 32845549
PMCID: PMC8350751
DOI: 10.1002/mds.28223

Multicenter Study

Clinical and Demographic Characteristics of Upper Limb Dystonia

Scott A Norris et al. Mov Disord. 2020 Nov.

. 2020 Nov;35(11):2086-2090.

doi: 10.1002/mds.28223. Epub 2020 Aug 26.

Authors

Affiliations

¹ Departments of Neurology and Radiology, Washington University School of Medicine, St. Louis, Missouri, USA.
² Departments of Neurology and Human Genetics, Emory University, Atlanta, Georgia, USA.
³ Institute of Neurogenetics and Department of Neurology, University of Luebeck, Luebeck, Germany.
⁴ Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
⁵ Department of Neurology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
⁶ Sorbonne University, Inserm U 1127, National Centre for Scientific Research, Joint Research Units 7225, Institut du Cerveau et de la Moelle épinière and Assistance Public Hôpitaux de Paris, Paris, France.
⁷ Department of Neurology, Pitié-Salpêtrière Hospital, Paris, France.
⁸ James J. and Joan A. Gardner Family Center for Parkinson's Disease and Movement Disorders, Department of Neurology, University of Cincinnati, Cincinnati, Ohio, USA.
⁹ Washington University in St. Louis, St. Louis, Missouri, USA.
¹⁰ Movement Disorders Unit, Neurology Department, Westmead Hospital & Sydney Medical School, University of Sydney, Sydney, Australia.
¹¹ Department of Psychology and School of Health Studies, University of Memphis, Memphis, Tennessee, USA.
¹² Veracity Neurosciences LLC, Memphis, Tennessee, USA.
¹³ Department of Neurology, Salpetriere Hospital, AP-HP, Paris, France.
¹⁴ Department of Neurology, Sorbonne Université, Paris, France.
¹⁵ Brain & Spine Institute, Joint Research Units 1127, INSERM 1127, Center National De Recherche Scientific 7235, Paris, France.
¹⁶ Department of Neurology, Virginia Commonwealth University, Richmond, Virginia, USA.
¹⁷ Department of Neurology, University of Rochester, Rochester, New York, USA.
¹⁸ University of Florida Department of Neurology, Fixel Institute for Neurologic Diseases, Gainesville, Florida, USA.
¹⁹ Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
²⁰ Departments of Neuroscience, Physical Therapy, and Occupational Therapy, Washington University School of Medicine, St. Louis, Missouri, USA.

PMID: 32845549
PMCID: PMC8350751
DOI: 10.1002/mds.28223

Abstract

Background: Knowledge of characteristics in upper limb dystonia remains limited, derived primarily from small, single-site studies.

Objective: The objective of this study was to characterize demographic and clinical characteristics of upper limb dystonia from the Dystonia Coalition data set, a large, international, multicenter resource.

Methods: We evaluated clinical and demographic characteristics of 367 participants with upper limb dystonia from onset, comparing across subcategories of focal (with and without dystonia spread) versus nonfocal onset.

Results: Focal onset occurred in 80%; 67% remained focal without spread. Task specificity was most frequent in this subgroup, most often writer's cramp and affecting the dominant limb (83%). Focal onset with spread was more frequent in young onset (<21 years). Focal onset occurred equally in women and men; nonfocal onset affected women disproportionately.

Conclusions: Upper limb dystonia distribution, focality, and task specificity relate to onset age and likelihood of regional spread. Observations may inform clinical counseling and design, execution, and interpretation of future studies. © 2020 International Parkinson and Movement Disorder Society.

Keywords: alleviating maneuver; musician's cramp; sensory trick; task-specific; writer's cramp.

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Conflict of interest statement

Relevant conflicts of interests/financial disclosures: Nothing to report.

References

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1. Sheehy MP, Marsden CD. Writers’ cramp-a focal dystonia. Brain 1982;105(Pt 3):461–480. - PubMed

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Clinical and Demographic Characteristics of Upper Limb Dystonia

Affiliations

Clinical and Demographic Characteristics of Upper Limb Dystonia

Authors

Affiliations

Abstract

Conflict of interest statement

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Medical