Guo S T, Jiang L B
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Key Laboratory of Ophthalmology ＆ Visual Sciences, Beijing 100730, China.
Zhonghua Yan Ke Za Zhi. 2021 May 11;57(5):386-390. doi: 10.3760/cma.j.cn112142-20200721-00494.
Mitochondrial optic neuropathy (MON) describes a group of optic neuropathies that exhibit mitochondrial dysfunction in retinal ganglion cells. Pathogenesis of MON includes genetic factors, such as Leber hereditary optic neuropathy and dominant optic atrophy, or acquired factors, such as drug intoxication and nutritional deficiencies, or the combination of both genetic factors and acquired factors. Regardless of different causes, MON shares similar features including bilateral central visual acuity loss, equally normal or slightly sluggish reaction of pupils to light and so on. Many novel therapies, such as pharmacological strategies, genetic therapy and stem cell therapy, are being widely studied in order to limit or reverse the damage of retinal ganglion cells. This article review the pathogenesis, clinical manifestations, ancillary testing, differential diagnosis and treatment progress of MON. .
线粒体性视神经病变（MON）是指一组在视网膜神经节细胞中表现出线粒体功能障碍的视神经病变。MON的发病机制包括遗传因素，如Leber遗传性视神经病变和显性视神经萎缩，或后天因素，如药物中毒和营养缺乏，或遗传因素与后天因素的结合。无论病因如何，MON都具有相似的特征，包括双侧中心视力丧失、瞳孔对光反应同等正常或略迟钝等。为了限制或逆转视网膜神经节细胞的损伤，许多新的治疗方法，如药物策略、基因治疗和干细胞治疗，正在被广泛研究。本文综述了MON的发病机制、临床表现、辅助检查、鉴别诊断及治疗进展。
