It affects men five times more commonly than women. Onset is usually between 18 and 30 years of age, but may occur at any age as acute or subacute visual loss affecting both eyes, simultaneously or sequentially.
Most patients maintain some peripheral vision, with only a few requiring guide dogs for mobility.
The ophthalmoscopic features may be nonspecific and missed by even the most skilled clinician. The diagnosis is sometimes confused with other causes of optic atrophy, or hysteria. A family history of similar visual loss is almost always present. Sometimes, mothers will conceal the diagnosis in distant relatives, so it is important to take a tactful but extensive pedigree. Few medical professionals can keep abreast of the rapidly changing knowledge about LHON and, on reviewing the literature, may notice some conflicting statements.