Leber hereditary optic neuropathy (LHON) produces a subacute and typically bilateral but sequential optic neuropathy. LHON is a mitochondrial disease and the most common mutations are at positions 11778, 14484 and 3460. LHON typically presents in young (age 20-40), healthy men but may occur in either sex and at any age. We report a case of LHON in a 72-year-old man and we emphasize the importance of testing for LHON in all cases of unexplained bilateral and sequential optic neuropathy with a ceco-central or central scotoma.
