Majander, A;
Robson, AG;
João, C;
Holder, GE;
Chinnery, PF;
Moore, AT;
Votruba, M;
... Yu-Wai-Man, P; + view all
(2017)
The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy.
Mitochondrion
, 36
pp. 138-149.
10.1016/j.mito.2017.07.006.
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Abstract
Leber inherited optic neuropathy (LHON) is characterized by subacute bilateral loss of central vision due to dysfunction and loss of retinal ganglion cells (RGCs). Comprehensive visual electrophysiological investigations (including pattern reversal visual evoked potentials, pattern electroretinography and the photopic negative response) performed on 13 patients with acute and chronic LHON indicate early impairment of RGC cell body function and severe axonal dysfunction. Temporal, spatial and chromatic psychophysical tests performed on 7 patients with acute LHON and 4 patients with chronic LHON suggest severe involvement or loss of the midget, parasol and bistratified RGCs associated with all three principal visual pathways.
| Type: | Article |
|---|---|
| Title: | The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy |
| Location: | Netherlands |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.mito.2017.07.006 |
| Publisher version: | http://doi.org/10.1016/j.mito.2017.07.006 |
| Language: | English |
| Additional information: | Copyright © 2017 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY license (http://creativecommons.org/licenses/BY/4.0/). |
| Keywords: | Leber hereditary optic neuropathy (LHON); The pattern electroretinogram (PERG); The photopic negative responses (PhNR); Critical flicker fusion; Spatial contrast sensitivity; Chromatic resolution |
| 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 > Institute of Ophthalmology |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1570153 |
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