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Leber's Hereditary Optic Neuropathy

Background

Leber's Hereditary Optic Neuropathy: Leber's Optic Atrophy: LHON

This 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 they 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.

What are the symptoms? View What are the symptoms?

Medical text written October 2004 by Miss Isabelle Russell-Eggitt. Last updated December 2008 by Miss Isabelle Russell-Eggitt FRCS FRCOphth, Consultant Ophthalmic Surgeon, Great Ormond Street Hospital for Children, London, UK with the kind help of Professor David A Mackey MD FRANZCO, Ophthalmologist, Royal Victorian Eye and Ear Hospital, Melbourne, Australia.

 

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