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Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin?

Kaji, R; Bhatia, K; Graybiel, AM; (2018) Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin? Journal of Neurology, Neurosurgery and Psychiatry , 89 (5) pp. 488-492. 10.1136/jnnp-2017-316250. Green open access

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Abstract

Dystonia is a disorder of motor programmes controlling semiautomatic movements or postures, with clinical features such as sensory trick, which suggests sensorimotor mismatch as the basis. Dystonia was originally classified as a basal ganglia disease. It is now regarded as a 'network' disorder including the cerebellum, but the exact pathogenesis being unknown. Rare autopsy studies have found pathology both in the striatum and the cerebellum, and functional disorganisation was reported in the somatosensory cortex in patients. Recent animal studies showed physiologically tight disynaptic connections between the cerebellum and the striatum. We review clinical evidence in light of this new functional interaction between the cerebellum and basal ganglia, and put forward a hypothesis that dystonia is a basal ganglia disorder that can be induced by aberrant afferent inputs from the cerebellum.

Type: Article
Title: Pathogenesis of dystonia: is it of cerebellar or basal ganglia origin?
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/jnnp-2017-316250
Publisher version: http://doi.org/10.1136/jnnp-2017-316250
Language: English
Additional information: © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/ licenses/by-nc/4.0/
Keywords: Cerebellar disease, dystonia, functional imaging, movement disorders, neurophysiology
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
URI: https://discovery.ucl.ac.uk/id/eprint/10043957
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