In most cases of Leber's hereditary optic neuropathy (LHON) the only clinic
al manifestation is visual loss. A multiple sclerosis-like illness has been
infrequently reported in association with LHON. Most patients are women ha
rboring the mtDNA 11778 mutation. We present a young man with clinical and
paraclinical evidence of a demyelinating process with profound bilateral vi
sual loss who harbored the mtDNA 14484 mutation associated with LHON.