Dopa responsive dystonia / 临床神经病学杂志

ABSTRACT

Objective To explore the clinical feature and treatment method of the dopa responsive dystonia (DRD).Methods To observe the clinical manifestation,auxiliary test and the response on the treatment of levodopa in 6 patients from 4 families.Results 1 patient for childhood onset and 2 patients for adolescent onset with DRD, first symptom showed leg dystonia and toe walking with difficulty, whereas 3 patients of adult onset showed tremor and rigidity. Babinski sign was presented in 3 cases. Diurnal fluctuation in symptom severity occurred in all cases. All patients had obvious response to small dose levodopa in 1～6 days. In 2 cases, the effect was maintained for 7 years, without increasing the intake of levodopa.Conclusion The clinical feature of DRD was more remarkable,the therapeutic effectiveness of levodopa was rapid,sustained and obvious.