We report the long-term response to levodopa in 20 patients with dopa-responsive dystonia (DRD). We found an inverse correlation between the daily dose of levodopa and duration of treatment (r=-0.59, P<0.01). Mild dyskinesias were present in 20% of our patients. Dyskinetic patients were on a higher dose of levodopa than non-dyskinetics. Dyskinesias responded to a reduction in levodopa, with no deterioration in motor function. We propose that the dopamine turnover might decrease with time, which would lead to a decrease in the requirement for levodopa and the occurrence of dyskinesias late in the course of DRD.