The authors report a patient with dystonia secondary to bilateral lesions of the 
basal ganglia, who improved dramatically with levodopa. The patient presented at 
the age of 4 years with progressive dystonia of the lower extremities and right 
upper extremity. Magnetic resonance imaging (MRI) of the brain showed bilateral 
hyperintensities of the globus pallidus that remained stable over the years. 
Despite extensive investigations, the etiology of her basal ganglia lesions 
remained nebulous. The patient's dystonia responded to Trihexyphenidyl and to 
tetrabenazine, but these medications needed to be stopped because of side 
effects. At the age of 12 years, small doses of levodopa-carbidopa were tried 
and resulted in dramatic improvement of her dystonia. The authors believe that 
in the pediatric population with secondary dystonias other than Segawa disease, 
even though this has been reported only rarely to be effective, a therapeutic 
trial with levodopa should be considered in some instances.