CAJ | 학술논문

目的　探讨多巴反应性肌张力障碍的临床特点及治疗。方法　观察6例来自4个家庭患者的临床表现、辅助检查及对左旋多巴治疗的反应。结果　1例儿童期、2例少年期起病者，首发症状均为下肢肌张力异常、足跟着地困难；3例青年起病者表现为震颤、肢体僵硬；3例出现病理征阳性；6例症状均呈晨轻暮重。服用多巴制剂1～6天内均有明显疗效，用药最长者(2例)达7年，未增加剂量。结论　该病临床特点较明显，多巴制剂对其有快速、显著、持续的疗效。
목적　탐토다파반응성기장력장애적림상특점급치료。방법　관찰6례래자4개가정환자적림상표현、보조검사급대좌선다파치료적반응。결과　1례인동기、2례소년기기병자，수발증상균위하지기장력이상、족근착지곤난；3례청년기병자표현위진전、지체강경；3례출현병리정양성；6례증상균정신경모중。복용다파제제1～6천내균유명현료효，용약최장자(2례)체7년，미증가제량。결론　해병림상특점교명현，다파제제대기유쾌속、현저、지속적료효。
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.