Iivanainen M, Himberg J J
Arch Neurol. 1982 Apr;39(4):236-8. doi: 10.1001/archneur.1982.00510160042008.
Twenty-six adults with severe progressive myoclonus epilepsy (PME) were admitted to a long-term prospective clinical study; 19 were followed up for six years. The previous medication, phenytoin sodium and other antiepileptic drugs, was changed to valproate sodium (Deprakine [Finland]; Depakene Syrup, comparable US product), clonazepam (Clonopin), and phenobarbital, and the patients' conditions improved. After six years of follow-up, the favorable result continued. The mean plasma concentration in 26 patients after four months of follow-up was 27.8 +/- 6.9 mg/L for valproate, 0.053 +/- 0.025 mg/L for clonazepam, and 19.2 +/- 7.9 mg/L for phenobarbital. The clinical response was not in accordance with the plasma drug concentrations when the data of the whole population were statistically analyzed. The classification of disability of patients with PME into five degrees was useful. The longlasting favorable result suggests that the combination of valproate, clonazepam, and phenobarbital is the most effective therapy for severe PME.
26例严重进行性肌阵挛癫痫（PME）成人患者被纳入一项长期前瞻性临床研究；其中19例接受了为期6年的随访。先前使用的药物苯妥英钠和其他抗癫痫药物被更换为丙戊酸钠（德巴金[芬兰]；德巴金糖浆，美国同类产品）、氯硝西泮（氯硝安定）和苯巴比妥，患者病情有所改善。经过6年的随访，良好效果持续存在。随访4个月后，26例患者的丙戊酸平均血浆浓度为27.8±6.9mg/L，氯硝西泮为0.053±0.025mg/L，苯巴比妥为19.2±7.9mg/L。对全体人群的数据进行统计分析时，临床反应与血浆药物浓度并不一致。将PME患者的残疾程度分为五级是有用的。长期良好的结果表明，丙戊酸、氯硝西泮和苯巴比妥联合使用是治疗严重PME最有效的疗法。
