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

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

Author information

1
Department of Neurology, University of Michigan, Room 4009, BSRB, 109 Zina Pitcher Place, Ann Arbor, MI, 48109-2200, USA. vikramsh@med.umich.edu.
2
Department of Molecular and Integrative Physiology, University of Michigan, Ann Arbor, MI, 48109-2200, USA. vikramsh@med.umich.edu.
3
Sobell Department of Motor Neuroscience and Movement Disorders, University College London, London, UK.
4
Department of Neurology, University of Michigan, Room 4009, BSRB, 109 Zina Pitcher Place, Ann Arbor, MI, 48109-2200, USA.
5
Department of Cell and Developmental Biology, University of Michigan Medical School, Ann Arbor, MI, USA.
6
Center for Neurosciences, The Feinstein Institute for Medical Research, Manhasset, NY, USA.
7
Global Research Organization, Medtronic Inc. Neuromodulation, Minneapolis, MN, USA.
8
Yerkes National Primate Center and Department of Neurology, Emory University, Atlanta, GA, USA.
9
Department of Neurology, Human Genetics and Pediatrics, Emory University, Atlanta, GA, USA.
10
Departments of Pharmacology and Neurology, Emory University, Atlanta, GA, USA.
11
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.
12
Human Motor Control Section, Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA.
13
Dominick P. Purpura Department of Neuroscience, Albert Einstein College of Medicine, New York, NY, USA.
14
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.
15
Departments of Neurology, and Anatomy and Neurobiology, University of Tennessee Health Science Center, Memphis, TN, USA.
16
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.
17
Centre de NeuroImagerie de Recherche - CENIR, ICM, F-75013, Paris, France.
18
Systems Neuroscience Institute and Center for the Neural Basis of Cognition, University of Pittsburgh, Pittsburgh, PA, USA.
19
Department of Neurobiology, University of Pittsburgh Brain Institute, University of Pittsburgh, Pittsburgh, PA, USA.

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

PMID:
27734238
PMCID:
PMC5336511
DOI:
10.1007/s12311-016-0825-6
[Indexed for MEDLINE]
Free PMC Article

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