Delineating the electrophysiological signature of dystonia

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.