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Neuroimaging in Leber Hereditary Optic Neuropathy: State-of-the-art and future prospects

Chow-Wing-Bom, HT; Callaghan, MF; Wang, J; Wei, S; Dick, F; Yu-Wai-Man, P; Dekker, TM; (2022) Neuroimaging in Leber Hereditary Optic Neuropathy: State-of-the-art and future prospects. NeuroImage: Clinical , 36 , Article 103240. 10.1016/j.nicl.2022.103240. Green open access

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Abstract

Leber Hereditary Optic Neuropathy (LHON) is an inherited mitochondrial retinal disease that causes the degeneration of retinal ganglion cells and leads to drastic loss of visual function. In the last decades, there has been a growing interest in using Magnetic Resonance Imaging (MRI) to better understand mechanisms of LHON beyond the retina. This is partially due to the emergence of gene-therapies for retinal diseases, and the accompanying expanded need for reliably quantifying and monitoring visual processing and treatment efficiency in patient populations. This paper aims to draw a current picture of key findings in this field so far, the challenges of using neuroimaging methods in patients with LHON, and important open questions that MRI can help address about LHON disease mechanisms and prognoses, including how downstream visual brain regions are affected by the disease and treatment and why, and how scope for neural plasticity in these pathways may limit or facilitate recovery.

Type: Article
Title: Neuroimaging in Leber Hereditary Optic Neuropathy: State-of-the-art and future prospects
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.nicl.2022.103240
Publisher version: https://doi.org/10.1016/j.nicl.2022.103240
Language: English
Additional information: © 2022 The Author(s). Published by Elsevier Inc. under a Creative Commons license (https://creativecommons.org/licenses/by/4.0/).
Keywords: Optic neuropathies, LHON, MRI, DTI, Optic pathways
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Div of Psychology and Lang Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Div of Psychology and Lang Sciences > Experimental Psychology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Imaging Neuroscience
URI: https://discovery.ucl.ac.uk/id/eprint/10160172
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