Abstract
We observed a 32-year-old man with repeated bilateral optic papillitis followed by permanent visual loss. The patient had mitochondrial point mutation at nucleotide 11778. Fluorescein angiogram showed dye leakage from each disc at the onset. Following the fourth visual loss, no dye leakage at the disc characterized by circumpapillary microangiopathy could be detected. The patient and the family members examined had the 11778 mutation. Optic neuritis, an ocular disorder, may possibly be an extramito-chondrial risk factor for the onset of LHON.