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Introduction :  In movement disordersoccurring in developmental brain particular symptoms appear along withmaturation of the involved neuronal systems.  Among two striatal pathways of the basalganglia, the direct pathway matures earlier than the indirect pathway.  As for the output pathway of the basalganglia the descending pathway attains maturational levels by 5 years while theascending pathway around 15 years.  Methods :  The pathophysiologiesof dopa-responsive dystonia (DRD) and Tourette syndrome (TS) were evaluated basedon the developmental course the basal ganglia and the nigrostriatal dopamine (NS-DA).Results :  Among DRD,postural type of Segawa disease (SD) caused by deficiency of tyrosinehydroxylase in the terminals of the NS-DA neuron develops postural dystonia inchildhood through the direct pathway and the descending output. In action typeof SD dysfunction of the DA neuron innervating to the subthalamic nucleus inthe indirect pathway causes dystonic movement by disfacilitation of thedescending output in late childhood and by disfacilitation of the ascendingpathways focal or segmental dystonia and parkinsonism in late childhood toadulthood. TS is caused by deficiency of DA in the substantia nigra. Developmentof DA receptor upward regulation induces simple tics in early childhood throughthe direct pathway and produces complex tics and obsessive compulsive disordersin late childhood through the ascending pathways.Conclusion:  Thus, to evaluatethe symptoms of the movement disorders of children with correlation of the agesof the developmental courses of the basal ganglia makes it possible to clarify pathophysiologiesof these disorders.

Basal Ganglia; Dopamine Neuron; Development; Dopa-responsive Dystonia; Tourette syndrome