J Korean Ophthalmol Soc.  1990 Feb;31(2):241-248.

Leber's Hereditary Optic Neuropathy in Two Brothers of a Family

• Hur J
• Sohn HY
• Won IG

Abstract

The Leber's hereditary optic neuropathy, which affects mainly males in the late teens or in the early twenties, is a rare inherited disorder characterized by bilateral rapid loss of central vision. Leber's disease undergoes like optic neuritis in acute stage, but in late stage it results in optic atrophy with severe impairment of visual acuity and absolute central scotoma. Recently the authors have experienced two cases of Leber's optic neuropathy in a family. We observed a patient whose visual acuity of right eye was 0.8 at first examination, but reduced to 0.04 by 2 months after onset in spite of medical treatment, So we described the characteristic clinical findings of Leber's disease with brief review of the literatures.

Keyword

Leber's optic neuropathy

MeSH Terms

Adolescent
Humans
Male
Optic Atrophy
Optic Atrophy, Hereditary, Leber*
Optic Neuritis
Scotoma
Siblings*
Visual Acuity