Abstract

目的 加强对多巴反应性肌张力失常的认识和重视.方法回顾近2年我们诊治的3例成年人DRD患者之临床表现、辅助检查与治疗.结果3例均为女性,无家族史.发病年龄14～29岁,平均20±7.94岁,平均误诊时间29.33±15.95年.表现为缓慢起病,四肢发僵,活动困难或伴有肢体震颤,足趾屈曲、内翻畸形;症状呈晨轻暮重.查体发现四肢肌张力强直性或齿轮样增高,双下肢腱反射活跃至亢进,1例病理征可疑阳性和脊柱前屈.辅助检查:血清学检查、CSF、头颅CT或MRI和神经电生理检查均正常.用小剂量复方左旋多巴有显著改善,平均剂量为98.21±49.17 mg/d,使用最长者已达14年,无需增加剂量.结论本病为少见的运动障碍疾病,在诊断中应与帕金森病鉴别.小剂量复方左旋多巴有显著、持久的疗效,早期应用安坦、金刚烷胺也有效。

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