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