Hirose Mieko, Yokoyama Hiroyuki, Noguchi Rie, Haginoya Kazuhiro, Aoki Masashi, Iinuma Kazuie
Tohoku University, School of Medicine, Sendai, Miyagi.
No To Hattatsu. 2004 Jan;36(1):75-9.
We report a 13-year-old girl with dentatorubal-pallidoluysian atrophy (DRPLA), presenting clinically as progressive myoclonic epilepsy. The action myoclonus, which severely impaired her daily life, was markedly improved by administration of piracetam, a drug reportedly useful for myoclonus of cortical origin. In our case, piracetam effectively suppressed severe subcortical myoclonus of DRPLA, suggesting that the drug may be useful in the treatment of both cortical, and subcortical myoclonus.
我们报告了一名患有齿状核红核苍白球路易体萎缩症（DRPLA）的13岁女孩，临床表现为进行性肌阵挛癫痫。动作性肌阵挛严重影响了她的日常生活，通过服用吡拉西坦（一种据报道对皮质源性肌阵挛有效的药物），症状得到了明显改善。在我们的病例中，吡拉西坦有效抑制了DRPLA严重的皮质下肌阵挛，这表明该药物可能对治疗皮质性和皮质下肌阵挛均有用。
