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The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy

Majinder, A., Robson, Anthony G., Jo, Catarina, Holder, Graham E., Chinnery, Patrick F., Moore, Anthony T., Votruba, Marcela ORCID:, Stockman, Andrew and Yu-Wai-Man, Patrick 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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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.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Optometry and Vision Sciences
Uncontrolled Keywords: Leber hereditary optic neuropathy (LHON); The pattern electroretinogram (PERG); The photopic negative responses (PhNR); Critical flicker fusion; Spatial contrast sensitivity; Chromatic resolution
Publisher: Elsevier
ISSN: 1567-7249
Funders: NIHR, BBSRC
Date of First Compliant Deposit: 20 July 2017
Date of Acceptance: 14 July 2017
Last Modified: 17 Nov 2023 13:04

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