title: Current Opinions and Areas of Consensus on the Role of the Cerebellum in Dystonia.
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.
authors.affiliation: Department of Neurology, University of Michigan, Room 4009, BSRB, 109 Zina Pitcher Place, Ann Arbor, MI, 48109-2200, USA. vikramsh@med.umich.edu.|Department of Molecular and Integrative Physiology, University of Michigan, Ann Arbor, MI, 48109-2200, USA. vikramsh@med.umich.edu.
authors.forename: Vikram G
authors.initials: VG
authors.lastname: Shakkottai
authors.affiliation: Sobell Department of Motor Neuroscience and Movement Disorders, University College London, London, UK.
authors.forename: Amit
authors.initials: A
authors.lastname: Batla
authors.affiliation: Sobell Department of Motor Neuroscience and Movement Disorders, University College London, London, UK.
authors.forename: Kailash
authors.initials: K
authors.lastname: Bhatia
authors.affiliation: Department of Neurology, University of Michigan, Room 4009, BSRB, 109 Zina Pitcher Place, Ann Arbor, MI, 48109-2200, USA.|Department of Cell and Developmental Biology, University of Michigan Medical School, Ann Arbor, MI, USA.
authors.forename: William T
authors.initials: WT
authors.lastname: Dauer
authors.affiliation: Center for Neurosciences, The Feinstein Institute for Medical Research, Manhasset, NY, USA.
authors.forename: Christian
authors.initials: C
authors.lastname: Dresel
authors.affiliation: Center for Neurosciences, The Feinstein Institute for Medical Research, Manhasset, NY, USA.
authors.forename: Martin
authors.initials: M
authors.lastname: Niethammer
authors.affiliation: Center for Neurosciences, The Feinstein Institute for Medical Research, Manhasset, NY, USA.
authors.forename: David
authors.initials: D
authors.lastname: Eidelberg
authors.affiliation: Global Research Organization, Medtronic Inc. Neuromodulation, Minneapolis, MN, USA.
authors.forename: Robert S
authors.initials: RS
authors.lastname: Raike
authors.affiliation: Yerkes National Primate Center and Department of Neurology, Emory University, Atlanta, GA, USA.
authors.forename: Yoland
authors.initials: Y
authors.lastname: Smith
authors.affiliation: Department of Neurology, Human Genetics and Pediatrics, Emory University, Atlanta, GA, USA.
authors.forename: H A
authors.initials: HA
authors.lastname: Jinnah
authors.affiliation: Departments of Pharmacology and Neurology, Emory University, Atlanta, GA, USA.
authors.forename: Ellen J
authors.initials: EJ
authors.lastname: Hess
authors.affiliation: Institut du Cerveau et de la Moelle épinière (ICM), Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06 UMR, S 1127, Paris, France.|Human Motor Control Section, Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA.
authors.forename: Sabine
authors.initials: S
authors.lastname: Meunier
authors.affiliation: Human Motor Control Section, Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA.
authors.forename: Mark
authors.initials: M
authors.lastname: Hallett
authors.affiliation: Dominick P. Purpura Department of Neuroscience, Albert Einstein College of Medicine, New York, NY, USA.
authors.forename: Rachel
authors.initials: R
authors.lastname: Fremont
authors.affiliation: Dominick P. Purpura Department of Neuroscience, Department of Psychiatry and Behavioral Sciences, and The Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, New York, NY, USA.
authors.forename: Kamran
authors.initials: K
authors.lastname: Khodakhah
authors.affiliation: Departments of Neurology, and Anatomy and Neurobiology, University of Tennessee Health Science Center, Memphis, TN, USA.
authors.forename: Mark S
authors.initials: MS
authors.lastname: LeDoux
authors.affiliation: Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, F-75013, Paris, France.
authors.forename: Traian
authors.initials: T
authors.lastname: Popa
authors.affiliation: Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, F-75013, Paris, France.|Centre de NeuroImagerie de Recherche - CENIR, ICM, F-75013, Paris, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Gallea
authors.affiliation: Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, F-75013, Paris, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Lehericy
authors.affiliation: Systems Neuroscience Institute and Center for the Neural Basis of Cognition, University of Pittsburgh, Pittsburgh, PA, USA.
authors.forename: Andreea C
authors.initials: AC
authors.lastname: Bostan
authors.affiliation: Systems Neuroscience Institute and Center for the Neural Basis of Cognition, University of Pittsburgh, Pittsburgh, PA, USA.|Department of Neurobiology, University of Pittsburgh Brain Institute, University of Pittsburgh, Pittsburgh, PA, USA.
authors.forename: Peter L
authors.initials: PL
authors.lastname: Strick
mesh_terms: D000818:Animals; D002531:Cerebellum / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D004421:Dystonia / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D009434:Neural Pathways / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology
keywords: Ataxia; Cerebellum; Circuits; DYT1; Dystonia; Networks