The Anatomical Basis for Dystonia: The Motor Network Model.

• Type of Material: Article
• Authors: Hyder Jinnah, Emory University; Vladimir Neychev, Medical University of Sofia; Ellen Hess, Emory University
• Language: English
• Date: 2017
• Publisher: Columbia University, Library/Information Service, Center for Digital Research and Scholarship: Creative Commons Attribution Non-Commercial No Derivatives
• Publication Version: Final Published Version
• Copyright Statement: © 2017 Jinnah et al.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.7916/D8V69X3S
• Title of Journal or Parent Work: Tremor and Other Hyperkinetic Movements
• ISSN: 2160-8288
• Volume: 7
• Start Page: 506
• End Page: 506
• Grant/Funding Information: It was also supported in part by grant R01 NS088528.; This work was supported in part by a grant to the Dystonia Coalition, a consortium of the Rare Diseases Clinical Research Network (RDCRN) that is supported by the Office of Rare Diseases Research (ORDR) at the National Center for Advancing Clinical and Translational Studies (NCATS; U54 TR001456) in collaboration with the National Institute for Neurological Diseases and Stroke (NINDS; U54 NS065701).
• Keywords: torticollis; neuroimaging; neuroanatomy; blepharospasm; spasmodic dysphonia; focal hand dystonia; Dystonia; laryngeal dystonia; cervical dystonia
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, Pharmacology

Abstract

Background: The dystonias include a clinically and etiologically very diverse group of disorders. There are both degenerative and non-degenerative subtypes resulting from genetic or acquired causes. Traditionally, all dystonias have been viewed as disorders of the basal ganglia. However, there has been increasing appreciation for involvement of other brain regions including the cerebellum, thalamus, midbrain, and cortex. Much of the early evidence for these other brain regions has come from studies of animals, but multiple recent studies have been done with humans, in an effort to confirm or refute involvement of these other regions. The purpose of this article is to review the new evidence from animals and humans regarding the motor network model, and to address the issues important to translational neuroscience. Methods: The English literature was reviewed for articles relating to the neuroanatomical basis for various types of dystonia in both animals and humans. Results: There is evidence from both animals and humans that multiple brain regions play an important role in various types of dystonia. The most direct evidence for specific brain regions comes from animal studies using pharmacological, lesion, or genetic methods. In these studies, experimental manipulations of specific brain regions provide direct evidence for involvement of the basal ganglia, cerebellum, thalamus and other regions. Additional evidence also comes from human studies using neuropathological, neuroimaging, non-invasive brain stimulation, and surgical interventions. In these studies, the evidence is less conclusive, because discriminating the regions that cause dystonia from those that reflect secondary responses to abnormal movements is more challenging. Discussion: Overall, the evidence from both animals and humans suggests that different regions may play important roles in different subtypes of dystonia. The evidence so far provides strong support for the motor network model. There are obvious challenges, but also advantages, of attempting to translate knowledge gained from animals into a more complete understanding of human dystonia and novel therapeutic strategies.