14 Cases of late-onset Leber's hereditary optic neuropathy

Abstract

Leber's hereditary optic neuropathy (LHON) typically manifests in young men. Herein, we describe the clinical features of cases of late-onset LHON. Among 92 patients with LHON, 14 patients were over 50 years old when the disease first became symptomatic. We investigated age, sex, mtDNA mutations, and the clinical features of these patients. All 14 patients had m.11778G> A and 11 patients were men. Eight patients had family histories of LHON. They all had a central scotoma, and their visual acuity was 0.01 in their worst stage and 0.02 in their recovery stage. Their past medical histories included alcohol addiction, retinal vein occlusion, and total gastrectomy. Late-onset LHON is not rare. Some, but not all, of these patients had potentially relevant medical histories and we should discuss possible initiating events of this condition.