Toward a network model of dystonia

Ann N Y Acad Sci. 2012 Aug;1265:46-55. doi: 10.1111/j.1749-6632.2012.06692.x. Epub 2012 Jul 23.


Dystonia has generally been considered a basal ganglia (BG) disorder. Early models hypothesized that dystonia occurred as the result of reduced mean discharge rates in the internal segment of the globus pallidus (GPi). Increasing evidence suggests a more systemwide disruption of the basal ganglia thalamic circuit (BGTC) resulting in altered firing patterns, synchronized oscillations, and widened receptive fields. A model of dystonia incorporating these changes within the BGTC is presented in which we postulate that this pathophysiology arises from disruptions within the striatum. Alterations in the cerebellothalamocortical (CBTC) pathway to the development of dystonia may also play a role. However, the contribution of CBTC abnormalities to dystonia remains unclear and may vary with different etiologies of dystonia. Finally, the relevance of established and emerging theories related to the pathophysiology of dystonia is addressed within the context of improving conventional approaches for deep brain stimulation (DBS) treatment strategies.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Basal Ganglia / physiopathology
  • Brain Stem / physiopathology
  • Cerebellum / physiopathology
  • Deep Brain Stimulation / methods
  • Dystonia / physiopathology*
  • Dystonia / therapy
  • Globus Pallidus / physiopathology
  • Humans
  • Models, Neurological*
  • Nerve Net / physiology*
  • Neural Pathways / physiology*
  • Thalamus / physiopathology