Current Opinions and Areas of Consensus on the Role of the Cerebellum in Dystonia

Vikram G. Shakkottai, Amit Batla, Kailash Bhatia, William T. Dauer, Christian Dresel, Martin Niethammer, David Eidelberg, Robert S. Raike, Yoland Smith, H. A. Jinnah, Ellen J. Hess, Sabine Meunier, Mark Hallett, Rachel Fremont, Kamran Khodakhah, Mark S. LeDoux, Traian Popa, Cécile Gallea, Stéphane Lehericy, Andreea C. BostanPeter L. Strick

Research output: Contribution to journalReview articlepeer-review

158 Scopus citations


A role for the cerebellum in causing ataxia, a disorder characterized by uncoordinated movement, is widely accepted. Recent work has suggested that alterations in activity, connectivity, and structure of the cerebellum are also associated with dystonia, a neurological disorder characterized by abnormal and sustained muscle contractions often leading to abnormal maintained postures. In this manuscript, the authors discuss their views on how the cerebellum may play a role in dystonia. The following topics are discussed:The relationships between neuronal/network dysfunctions and motor abnormalities in rodent models of dystonia.Data about brain structure, cerebellar metabolism, cerebellar connections, and noninvasive cerebellar stimulation that support (or not) a role for the cerebellum in human dystonia.Connections between the cerebellum and motor cortical and sub-cortical structures that could support a role for the cerebellum in dystonia. Overall points of consensus include:Neuronal dysfunction originating in the cerebellum can drive dystonic movements in rodent model systems.Imaging and neurophysiological studies in humans suggest that the cerebellum plays a role in the pathophysiology of dystonia, but do not provide conclusive evidence that the cerebellum is the primary or sole neuroanatomical site of origin.

Original languageEnglish (US)
Pages (from-to)577-594
Number of pages18
Issue number2
StatePublished - Apr 1 2017
Externally publishedYes


  • Ataxia
  • Cerebellum
  • Circuits
  • DYT1
  • Dystonia
  • Networks

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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