The functional neuroanatomy of dystonia

• Type of Material: Article
• Authors: Vladimir K. Neychev, Danbury Hospital; Robert Gross, Emory University; Stephane Lehericy, Université Pierre et Marie Curie; Ellen Hess, Emory University; Hyder A Jinnah, Emory University
• Language: English
• Date: 2011-05-01
• Publisher: Elsevier: 12 months
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2011 Elsevier Inc. All rights reserved.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.nbd.2011.01.026
• Title of Journal or Parent Work: Neurobiology of Disease
• ISSN: 0969-9961
• Volume: 42
• Issue: 2
• Start Page: 185
• End Page: 201
• Grant/Funding Information: Supported in part by grants from the NIH Office of Rare Diseases Research and the National Institutes of Neurological Disorders & Stroke (NS033592, NS040470, NS967501).
• Keywords: DEPENDENT CALCIUM-CHANNEL; Thalamus; History; PRIMARY GENERALIZED DYSTONIA; Neurosciences; Animal model; PRIMARY TORSION DYSTONIA; CEREBRAL GLUCOSE-METABOLISM; WHITE-MATTER CHANGES; IDIOPATHIC CERVICAL DYSTONIA; Neurosciences & Neurology; FOCAL HAND DYSTONIA; DEEP BRAIN-STIMULATION; SPINOCEREBELLAR ATAXIA TYPE-6; Life Sciences & Biomedicine; Deep brain stimulation; Science & Technology; PRIMARY SOMATOSENSORY CORTEX; Basal ganglia; Cerebellum
• Research Categories: Health Sciences, Medicine and Surgery; Biology, Neuroscience; Health Sciences, Pharmacology

Abstract

Dystonia is a neurological disorder characterized by involuntary twisting movements and postures. There are many different clinical manifestations, and many different causes. The neuroanatomical substrates for dystonia are only partly understood. Although the traditional view localizes dystonia to basal ganglia circuits, there is increasing recognition that this view is inadequate for accommodating a substantial portion of available clinical and experimental evidence. A model in which several brain regions play a role in a network better accommodates the evidence. This network model accommodates neuropathological and neuroimaging evidence that dystonia may be associated with abnormalities in multiple different brain regions. It also accommodates animal studies showing that dystonic movements arise with manipulations of different brain regions. It is consistent with neurophysiological evidence suggesting defects in neural inhibitory processes, sensorimotor integration, and maladaptive plasticity. Finally, it may explain neurosurgical experience showing that targeting the basal ganglia is effective only for certain subpopulations of dystonia. Most importantly, the network model provides many new and testable hypotheses with direct relevance for new treatment strategies that go beyond the basal ganglia. This article is part of a Special Issue entitled "Advances in dystonia".