Neurology of Touretteʹs syndrome (TS) TS as a developmental dopamine disorder: a hypothesis

The favorable effect of dopamine (DA) depletors or DA receptor blockers suggested the state of increased transmission of DA system as the pathophysiology of Touretteʹs syndrome (TS). We have analysed the neurological signs of TS and evaluated the role of levodopa on the symptoms of TS. The data were compared with age-matched patients with Hereditary Progressive Dystonia (HPD) with marked diurnal fluctuation. Neurological examination of 81 drug naive TS patients revealed the clumsiness of rapid alternating pronation-supination movements of the arms and induced rigidity in the contralateral arm, which responded to the oral levodopa, and suggested hypofunction of the nigrostriatal (NS)-DA system. Postural asymmetry or scoliosis and abnormal tilting response suggested the asymmetric involvement of DA. The rotation to the side of less affected DA neuron on stepping with closed eyes suggested DA receptor supersensitivity. The favourable effects of a small dose of levodopa on these signs suggest the existence of DA receptor supersensitivity, because a small dose of levodopa is considered to alleviate the supersensitized DA receptors.