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Case Reports
. 2018 Nov 3;31(1):102-105.
doi: 10.1016/j.joco.2018.10.003. eCollection 2019 Mar.

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

Michele Fantini  1   2 , Samuel Asanad  2   3 , Rustum Karanjia  2   3   4   5 , Alfredo Sadun  2   3
Affiliations
Case Reports

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

Michele Fantini et al. J Curr Ophthalmol. .

Abstract

Purpose: To 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.

Methods: We 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.

Results: The 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.

Conclusion: Idebenone treatment combined with HRT may have a synergistic effect in enhancing cellular bioenergetics and may explain the patient's accelerated visual improvement.

Keywords: Accelerated visual improvement; Hormone replacement therapy; Leber's hereditary optic neuropathy; Mitochondrial disease.

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Figures

Fig. 1
Fig. 1
(A, B) Humphrey visual field (HVF) 24-2 maps of the visual field examination on initial presentation, revealing significant cecocentral scotomas in the left and right eye, respectively. (C, D) Depict total deviation of the visual field examination on initial presentation in the left and right eye, respectively. Mean deviation: –15.66 dB OS, –18.25 dB OD.
Fig. 2
Fig. 2
(A, B) Humphrey visual field (HVF) 30-2 maps of the visual field examination at two months, revealing mild decrease in cecocentral scotomas in the left and right eye, respectively. (C, D) depict total deviation of the visual field examination on initial presentation in the left and right eye, respectively. Mean deviation: −8.11 dB OD, −13.14 dB OS.
Fig. 3
Fig. 3
(A, B) Humphrey visual field (HVF) 30-2 maps of the visual field examination at eight months, revealing further decrease of the scotomas in the left and right eye, respectively. (C, D) depict less total deviation of the visual field examination on second visit in the left and right eye, respectively. Mean deviation: −4.56 dB OD, −6.61 dB OS.
See this image and copyright information in PMC

References

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