Hamada Shigeaki, Shimakawa Seishi, Satomura Shigeko, Naito Etsuo, Hashimoto Toshiaki
Department of Pharmacy, Japanese Red Cross Tokushima Hinomine Rehabilitation Center for People with Disabilities, Komatsujima, Tokushima..
No To Hattatsu. 2014 Nov;46(6):439-42.
We report a 21-year-old male patient with dentatorubral-pallidoluysian atrophy (DRPLA) showing progressive myoclonus epilepsy (PME), who responded to levetiracetam (LEV) at an initial dose of 1,000 mg/day. The patient developed epilepsy at the age of 10 years, and also showed intellectual regression. Various antiepileptic drugs showed no effects on generalized tonic seizures, tonic-clonic seizures, and myoclonus. Addition of LEV (1,000 mg/day) led to the reduction of myoclonus and tonic-clonic seizures, and improved the EEG and sleep-wake rhythm. He had a better appetite and gain weight. It is suggested that LEV may improve quality of life in patients with DRPLA, in addition to reducing the frequency of epileptic seizures.
我们报告了一名21岁患有齿状核红核苍白球路易体萎缩症（DRPLA）且表现为进行性肌阵挛癫痫（PME）的男性患者，其对初始剂量为每日1000毫克的左乙拉西坦（LEV）有反应。该患者10岁时患癫痫，还出现了智力衰退。各种抗癫痫药物对全身性强直发作、强直阵挛发作和肌阵挛均无效果。加用左乙拉西坦（每日1000毫克）后，肌阵挛和强直阵挛发作减少，脑电图及睡眠 - 觉醒节律得到改善。他食欲变好且体重增加。提示左乙拉西坦除了降低癫痫发作频率外，还可能改善DRPLA患者的生活质量。
