Leber hereditary optic atrophy

Le·ber he·red·i·tar·y op·tic at·ro·phy

(lā'bĕr), [MIM*535000]

degeneration of the optic nerve and papillomacular bundle with resulting loss of central vision and blindness, progressive for several weeks, then usually becoming stationary with permanent central scotoma; the age of onset is variable, most often in the third decade; more males than females are affected. Mitochondrial or cytoplasmic inheritance through the maternal lineage, caused by mutation in the mitochondrial gene(s) acting autonomously or in association with each other.

Synonym(s): Leber optic neuropathy

Le·ber he·red·i·tar·y op·tic at·ro·phy

(lā'bĕr hĕr-ed'i-tar-ē op'tik at'rŏ-fē)

Degeneration of the optic nerve and papillomacular bundle with resulting loss of central vision and blindness, which is progressive for several weeks, then usually becomes stationary with permanent central scotoma; the age of onset is variable, most often in the third decade; more males than females are affected.

Leber,

Theodor, German ophthalmologist, 1840-1917.

amaurosis congenita of Leber - an autosomal recessive cone-rod abiotrophy causing blindness or severely reduced vision at birth.

Leber hereditary optic atrophy - hereditary degeneration of the optic nerve and papillomacular bundle, resulting in rapid loss of central vision.

Leber idiopathic stellate neuroretinitis - a unilateral neuroretinitis with perifoveal exudates in Henle nerve fiber layer producing a macular star and spontaneous regression in a few months. Synonym(s): stellate neuroretinitis

Leber plexus - a small venous plexus in the eye between the venous sinuses of the sclera (of Schlemm) and the spaces of the iridocorneal angle (of Fontana).