Leber’s hereditary optic neuropathy: from the clinical to the neurobiochemical and molecular findings for understanding the pathogenesis of the disorder

Leber’s hereditary optic neuropathy (LHON) is a well-defined disorder clinically characterized by acute or subacute bilateral visual loss, usually in young, otherwise healthy men. Women can also be affected. The disease is clearly maternally transmitted and, in contrast to X-inheritance, the descendants of men are never affected. ThSis particular type of genetic transmission directed the attention to the mitochondrial pathogenesis of the disease, which has recently been demonstrated (Wallace et al., 1988).