Gueven N
Pharmacy, School of Medicine, University of Tasmania, Hobart, Australia.
Drugs Today (Barc). 2016 Mar;52(3):173-81. doi: 10.1358/dot.2016.52.3.2463564.
Idebenone is a rapidly absorbed, safe and well-tolerated drug and is currently the only clinically proven treatment option for Leber's hereditary optic neuropathy (LHON) patients. Idebenone (Raxone®) is approved by the European Medicines Agency for the treatment of LHON and has been available on the European market since 2015. Due to its molecular mode of action of bypassing the defective mitochondrial complex I, idebenone leads to improved energy supply and a functional recovery of retinal ganglion cells during the acute stage of the disease, thereby preventing further vision loss and promoting recovery of vision. Thus, commencing treatment shortly after the onset of symptoms is likely to have the best therapeutic effect, a hypothesis that is supported by the available clinical data.
艾地苯醌是一种吸收迅速、安全且耐受性良好的药物，目前是唯一经临床验证的用于治疗莱伯遗传性视神经病变（LHON）患者的治疗选择。艾地苯醌（商品名：雷卡奈®）已获欧洲药品管理局批准用于治疗LHON，自2015年起在欧洲市场上市。由于其通过绕过有缺陷的线粒体复合体I的分子作用模式，艾地苯醌在疾病急性期可改善能量供应并使视网膜神经节细胞功能恢复，从而防止视力进一步丧失并促进视力恢复。因此，在症状出现后不久开始治疗可能会产生最佳治疗效果，这一假设得到了现有临床数据的支持。
