TY - JOUR AU - Priglinger, Claudia AU - Klopstock, Thomas AU - Rudolph, Günter AU - Priglinger, Siegfried Georg TI - [Leber's Hereditary Optic Neuropathy]. JO - Klinische Monatsblätter für Augenheilkunde VL - 236 IS - 11 SN - 0023-2165 CY - Stuttgart PB - Thieme78968 M1 - DZNE-2020-07919 SP - 1271-1282 PY - 2019 AB - Leber's hereditary optic neuropathy (LHON) typically affects young adults with a higher prevalence in men, but can ultimately occur at any age and also in women. LHON is caused by point mutations in the mitochondrial DNA, which lead to a defect in complex I of the mitochondrial respiratory chain. This in turn causes dysfunction and later degeneration of retinal ganglion cells, followed by ascending optic atrophy. Classically, LHON presents as a subacute unilateral loss of visual acuity, dyschromatopsia in the red-green axis and a central or centrocecal scotoma. The partner eye usually develops similar symptoms within 3 - 6 months of onset of the disease. In 25 KW - DNA, Mitochondrial KW - Female KW - Genetic Therapy KW - Humans KW - Male KW - Optic Atrophy, Hereditary, Leber: genetics KW - Optic Atrophy, Hereditary, Leber: therapy KW - Retinal Ganglion Cells KW - Visual Acuity KW - Young Adult KW - DNA, Mitochondrial (NLM Chemicals) LB - PUB:(DE-HGF)16 C6 - pmid:31639883 DO - DOI:10.1055/a-0972-1552 UR - https://pub.dzne.de/record/141595 ER -