Leber's Optic Neuropathy

Leber's Congenital Amaurosis


SORRY

Leber's Hereditary Optic Neuropathy is not the same condition as Leber's Congenital Amaurosis. The LHON site does not have information relating to Leber's Congenital Amaurosis.

Leber's Congenital Amaurosis is a rare recessive disorder in which children are born with nystagmus (jiggling eyes) and poor vision - usually very poor and they are essentially blind from birth - although there have been some cases where the vision is not so bad. They can be difficult to diagnose because the eyes look normal in the early stages but generally will show some pigmentary retinopathy after a while. Electrical tests show a flat ERG. It is a retinal dystrophy vs LHON which affects the optic nerve ( and maybe the retina too - who knows!!) Leber also desribed some other retinal disorders (e.g. Leber's miliary anuerysms) so we have to be careful to use the correct terminology.

John Harrison FRACO
Brisbane
Australia.
This page gives some links to sites which do provide some inforrmation on Leber's Congenital Amaurosis



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This page last updated 28 October 2000