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International Parkinson and Movement Disorder Society

        VOLUME 29, ISSUE 3 • SEPTEMBER 2025. 

The definition and classification of the dystonias: A new consensus report  

 

Since its introduction more than 100 years ago,1 the concept of dystonia has evolved considerably. Over time, there was evolution in the descriptions of the defining features of dystonia, along with evolution in the many types of disorders where features of dystonia were reported.2-5 The first international consensus report regarding the definition and classification of dystonia appeared in 2013, more than 10 years ago.6 In this report, the many different clinical manifestations and causes were separated into two axes, one addressing clinical features, and the other addressing biological causes. This report rapidly became the gold standard, and it was widely implemented. The novel two-axis system was later adopted for classifying other movement disorders, including tremor7 and myoclonus.8   

The 2013 consensus report acknowledged the potential need for future changes as more was learned about the many different types of dystonia. Ten years after it was introduced, a new committee was established to review implementation of the proposal. Indeed, several studies had reported successful clinical applicability of the plan for different populations, with a few shortcomings.9-11 In addition, more has been learned about dystonia, particularly regarding its underlying biological heterogeneity. After two years of review, an updated consensus report was released in 2025 to address what has been learned following the original consensus report.12   

The new report retains most of the concepts of the original report, with most of the changes reflecting clarifications of the intent of the original report.  The definition of dystonia has been largely retained, except that references to “muscle contractions” have been eliminated to prevent confusion with neuromuscular disorders.   

There were some clarifications to aid in a more consistent application of Axis I, and some additional clinical features relevant to specific subtypes of dystonia were added. The main additions to Axis I recognize some relevant clinical features of dystonia, such as relationship to voluntary movement and a sometimes tremulous or jerky quality.13 Overall, Axis I provides a more comprehensive description of a variety of clinical features relevant for appreciating specific subgroups of dystonia.   

Some more substantial changes were made to Axis II, because much of what has been learned in the past decade has related to the etiology and pathogenesis of dystonia.14 The terminology used in Axis II and its structure have been revised. In addition to some clarifications of terminology, Axis II now more explicitly acknowledges the extensive underlying biological heterogeneity across many subtypes of dystonia. Although many cases remain in the “idiopathic” category, there has been enormous growth in appreciation of the heterogeneity of etiologies and biological mechanisms that may be developmental, degenerative, metabolic, immunological, or others.

We anticipate the new consensus report will facilitate diagnosis, treatment, and further understanding of etiologies and shared mechanisms across all types of dystonia. Undoubtedly, there will be a need for further updates in the future, as we continue to learn more about dystonia at a rapid rate. 

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Citations 

  1. Klein C, Fahn S. Translation of Oppenheim's 1911 paper on dystonia. Mov Disord 2013;28:851-862. 
  2. Jinnah HA, Albanese A. The new classification for the dystonias: Why was it needed and how was it accomplished? Mov Disord Clin Pract 2014;1:280-284. 
  3. Camargo CH, Teive HA. Evolution of the concept of dystonia. Arq Neuropsiquiatr 2014;72:559-561. 
  4. Newby RE, Thorpe DE, Kempster PA, Alty JE. A history of dystonia: Ancient to modern. Mov Disord Clin Pract 2017;4:478-485. 
  5. Jinnah HA, Balint B, Fung VSC. The Dystonias. New York: Oxford University Press, 2025. 
  6. Albanese A, Bhatia K, Bressman SB, et al. Phenomenology and classification of dystonia:  A consensus update. Mov Disord 2013;28:863-873. 
  7. Bhatia KP, Bain P, Bajaj N, et al. Consensus statement on the classification of tremors. from the task force on tremor of the International Parkinson and Movement Disorder Society. Mov Disord 2018;33:75-87. 
  8. Lattore A, Van der Veen S, A. P, et al. IAPRD new consensus classification of myoclonus. Park Rel Disord 2025;in press. 
  9. van Egmond ME, Contarino MF, Lugtenberg CHA, et al. Variable Interpretation of the dystonia consensus classification items compromises its solidity. Mov Disord 2019;34:317-320. 
  10. Lumsden DE, Gimeno H, Lin JP. Classification of dystonia in childhood. Parkinsonism Relat Disord 2016;33:138-141. 
  11. Sasikumar S, Albanese A, Krauss JK, Fasano A. Implementation of the current dystonia classification from 2013 to 2018. Mov Disord Clin Pract 2019;6:250-253. 
  12. Albanese A, Bhatia K, Fung V, et al. Definition and classification of dystonia. Movement Disorderes 2025;40:1248-1259. 
  13. Shaikh AG, Fasano A, Pandey S, et al. Challenges in describing tremor and dystonia. Neurology 2025;104:e210209. 
  14. Albanese A, Bhatia K, Jinnah HA. Dystonia. Cambridge MA: Elsevier, 2023. 

 

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