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We report observations on the treatment of 66 patients with presumed dopa- responsive dystonia(DRD).Forty-seven of these patients had hereditary disease;19 had disease of sporadic occurrence.Initial diagnostic confusion with"cerebral palsy"or"spastic diplegia"existed in 16 patients.Several patients benefited from anticholinergic medications and a few from carbamazepine.Levodopa was the most effective treatment in all cases.In the majority,there was an excellent response,with continued long-term clinical stability on levodopa therapy for as long as 10 to 22 years.Four men with sporadic disease and 1 woman with a sister affected with adolescent-onset parkinsonism had similar initial treatment response,but developed"wearing-off"and a less satisfactory response to levodopa within the first few years of treatment.This indicates that some patients with clinical syndromes suggestive of DRD may not have an excellent prognosis on long-term levodopa treatment and may represent misclassified cases of childhood-onset parkinsonism. |
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