Original Article Clinical Features of Leber's Hereditary Optic Neuropathy with

Leber's hereditary optic neuropathy (LHON) is amaternally inherited mitochondrial disease that results in acute or subacute bilateral visual loss, par-ticularly in young men. Several mtDNA mutations including those at nucleotide positions 11778, 3460, and 14484 have been proposed as primary mutations in the pathogenesis of LHON.(1) They are the most deleterious and are not found in normal controls. Among them, the 11778 mutation is most important and accounts for 50 % to 70 % of LHON patients in several studies.(2-5) Visual prognoses in LHON patients are usually poor, especially in patients with the 11778 mtDNA mutation. In addition, a recent Chinese study demonstrated that a homoplasmic 11778 mutation was exclusively present in 92 % of LHON patients.(6) However, the clinical manifesta