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Leber's Hereditary Optic Neuropathy
Background
Leber's Hereditary Optic Neuropathy: Leber's Optic Neuropathy; Leber's Optic Atrophy; LHON
Leber's Hereditary Optic Neuropathy (LHON) is a rare inherited condition which involves the optic nerves with either complete or partial loss of central vision. This disorder is unrelated to Leber's congenital amaurosis – the only connection being that both conditions were described by the same doctor.
The optic nerve is the 'information cable' joining the eye, the 'camera', to the brain. If damage occurs to the retina or the optic nerve then some of the 'wires' in the optic nerve will die. The 'finest wires' that allow us to see fine detail are lost in LHON. The nerve's health can be assessed by looking into the eye. A healthy nerve looks pink and one that has been damaged pale and is called 'atrophic'. However, early in LHON the nerve can look abnormally pink and slightly swollen with tortuous blood vessels around the nerve. This appearance can be seen in family members who may never lose vision.
