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

Cerebellum. 2017 Apr;16(2):577-594. doi: 10.1007/s12311-016-0825-6.

Abstract

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.

Keywords: Ataxia; Cerebellum; Circuits; DYT1; Dystonia; Networks.

Publication types

  • Consensus Development Conference
  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Intramural

MeSH terms

  • Animals
  • Cerebellum / diagnostic imaging
  • Cerebellum / pathology
  • Cerebellum / physiopathology*
  • Dystonia / diagnostic imaging
  • Dystonia / pathology
  • Dystonia / physiopathology*
  • Humans
  • Neural Pathways / diagnostic imaging
  • Neural Pathways / pathology
  • Neural Pathways / physiopathology