Adult onset dystonia: A disorder of the collicular–pulvinar–amygdala network

Abstract

Models attempting to explain the pathogenesis of adult onset idiopathic focal dystonia often fail to accommodate the entire spectrum of this disorder: the diverse motor and non-motor symptoms, psychiatric and cognitive dysfunction, as well as the sub-clinical, physiological and anatomical, abnormalities.We propose, and present the accumulating evidence, that the adult onset dystonia syndrome is due to disruption in the covert-attentional network, the unconscious sub-cortical mechanism for the detection of potentially environmentally threatening (salient) stimuli, involving the collicular–pulvinar–amygdala network.A critical consideration of this network indicates a number of hypothesis-generated research questions aimed at elucidating the pathogenesis of adult onset dystonia.Given the rarity of adult onset dystonia, international, multidisciplinary, multicentre studies are required to elucidate the prevalence of non-motor symptoms in unaffected relatives, in particular, using temporal discrimination. Research focussing on the non-motor symptoms and the collicular–pulvinar–amygdala pathway may be the key to understanding adult-onset idiopathic focal dystonias (AOIFD) pathophysiology.