Hormone replacement therapy in Leber's hereditary optic neuropathy: Accelerated visual recovery in vivo

PURPOSETo report an accelerated course of visual recovery in a case of Leber's hereditary optic neuropathy (LHON) following treatment with idebenone and hormone replacement therapy (HRT). We hereby demonstrate the clinical utility of estrogen's protective role in LHON in vivo. METHODSWe present a case of LHON in a menopausal woman carrying the 10197 mitochondrial DNA (mtDNA) mutation, who experienced loss of vision shortly after discontinuing her estrogen replacement regimen. Functional visual outcomes are reported following treatment with idebenone and HRT. RESULTSThe patient exhibited an accelerated course of visual recovery, experiencing improvement in vision as early as one month and complete reversal of vision loss by eight months post-therapy. CONCLUSIONIdebenone treatment combined with HRT may have a synergistic effect in enhancing cellular bioenergetics and may explain the patient's accelerated visual improvement.