Volume 28, Issue 7 p. 863-873
Review

Phenomenology and classification of dystonia: A consensus update

Alberto Albanese MD

Corresponding Author

Alberto Albanese MD

Department of Neurology, Catholic University, Milan, Italy

Department of Neurology, Carlo Besta National Neurological Institute, Milan, Italy

Correspondence to: Dr. Alberto Albanese, Fondazione Istituto Neurologico Carlo Besta, Via G. Celoria, 11, 20133 Milano, Italy; [email protected]Search for more papers by this author
Kailash Bhatia MD, FRCP

Kailash Bhatia MD, FRCP

Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London (UCL), London, United Kingdom

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Susan B. Bressman MD

Susan B. Bressman MD

Departments of Neurology, Beth Israel Medical Center and Albert Einstein College of Medicine, New York, New York and Bronx, New York, USA

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Mahlon R. DeLong MD

Mahlon R. DeLong MD

Department of Neurology, Emory University, Atlanta, Georgia, USA

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Stanley Fahn MD

Stanley Fahn MD

Department of Neurology, Columbia University, New York, New York, USA

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Victor S.C. Fung PhD, FRACP

Victor S.C. Fung PhD, FRACP

Movement Disorders Unit, Department of Neurology, Westmead Hospital and Sydney Medical School, University of Sydney, Sydney, Australia

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Mark Hallett MD

Mark Hallett MD

Human Motor Control Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA

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Joseph Jankovic MD

Joseph Jankovic MD

Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA

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Hyder A. Jinnah PhD

Hyder A. Jinnah PhD

Departments of Neurology, Human Genetics and Pediatrics, Emory University, Atlanta, Georgia, USA

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Christine Klein MD

Christine Klein MD

Section of Clinical and Molecular Neurogenetics at the Department of Neurology, University of Lübeck, Lübeck, Germany

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Anthony E. Lang MD

Anthony E. Lang MD

Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson's Disease, Toronto Western Hospital and the University of Toronto, Toronto, Canada

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Jonathan W. Mink MD, PhD

Jonathan W. Mink MD, PhD

Departments of Neurology, Neurobiology, and Anatomy, Brain and Cognitive Sciences, and Pediatrics, University of Rochester, Rochester, New York, USA

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Jan K. Teller PhD

Jan K. Teller PhD

Dystonia Medical Research Foundation, Chicago, Illinois, USA

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First published: 06 May 2013
Citations: 1,472

Relevant conflicts of interest/financial disclosures: Full financial disclosures and author roles may be found in the Acknowledgments section online.

ABSTRACT

This report describes the consensus outcome of an international panel consisting of investigators with years of experience in this field that reviewed the definition and classification of dystonia. Agreement was obtained based on a consensus development methodology during 3 in-person meetings and manuscript review by mail. Dystonia is defined as a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Dystonic movements are typically patterned and twisting, and may be tremulous. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation. Dystonia is classified along 2 axes: clinical characteristics, including age at onset, body distribution, temporal pattern and associated features (additional movement disorders or neurological features); and etiology, which includes nervous system pathology and inheritance. The clinical characteristics fall into several specific dystonia syndromes that help to guide diagnosis and treatment. We provide here a new general definition of dystonia and propose a new classification. We encourage clinicians and researchers to use these innovative definition and classification and test them in the clinical setting on a variety of patients with dystonia. © 2013 Movement Disorder Society