Leigh‐like encephalopathy complicating Leber's hereditary optic neuropathy

Abstract
Leber's hereditary optic neuropathy is a mitochondrial disease caused by point mutations in mitochondrial DNA. It usually presents as severe bilateral visual loss in young adults. We report on a neurological disorder resembling Leigh syndrome, which complicated Leber's hereditary optic neuropathy in three unrelated male patients harboring mitochondrial DNA mutations at nucleotide positions 3460, 14459, and 14484, respectively. This Leigh‐like encephalopathy appears to be associated with a much more severe outcome than isolated Leber's hereditary optic neuropathy.