Leber Hereditary Optic Neuropathy

LHON, a mitochondrial cause of sudden central vision loss

Overview

Leber hereditary optic neuropathy is a mitochondrial genetic disease that causes painless, subacute loss of central vision, usually in one eye followed by the other within weeks to months. It is passed down through the maternal line because mitochondria are inherited from the mother, and most cases are due to one of three common mitochondrial DNA variants. It most often affects young men, although not all carriers develop vision loss, and smoking and heavy alcohol use are recognized triggers. It was described by the German ophthalmologist Theodor Leber in 1871.