Abstract
Over the last 30 years, the concept of dystonia has dramatically changed, from being considered a motor neurosis, to a pure basal ganglia disorder, to finally reach the definition of a network disorder involving the basal ganglia, cerebellum, thalamus and sensorimotor cortex. This progress has been possible due to the collaboration between clinicians and scientists, and the development of increasingly sophisticated electrophysiological techniques able to non-invasively investigate pathophysiological mechanisms in humans. This review is a chronological excursus of the electrophysiological studies that laid the foundation for the understanding of the pathophysiology of dystonia and delineated its electrophysiological signatures. Evidence for neurophysiological abnormalities is grouped according to the neural system involved, and a unifying theory, bringing together all the hypothesis and evidence provided to date, is proposed at the end.
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Funding
KPB has received grant support from Horizon 2020 EU grant 634821 and honoraria/financial support to speak/attend meetings from GSK, Boehringer-Ingelheim, Ipsen, Merz, Sun Pharma, Allergan, Teva, Lundbeck and Orion pharmaceutical companies. KPB receives royalties from Oxford University press and a stipend for MDCP editorship. This research study was supported by the National Institute for Health Research University College London Hospitals Biomedical Research Centre and the Edmond J. Safra Philanthropic Foundation. All the other authors have no disclosures.
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Latorre, A., Rocchi, L. & Bhatia, K.P. Delineating the electrophysiological signature of dystonia. Exp Brain Res 238, 1685–1692 (2020). https://doi.org/10.1007/s00221-020-05863-2
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DOI: https://doi.org/10.1007/s00221-020-05863-2