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

Lookup NU author(s): Professor Patrick Chinnery, Dr Patrick Yu Wai Man



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


© 2017. 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.

Publication metadata

Author(s): Majander A, Robson AG, Joao C, Holder GE, Chinnery PF, Moore AT, Votruba M, Stockman A, Yu-Wai-Man P

Publication type: Article

Publication status: Published

Journal: Mitochondrion

Year: 2017

Volume: 36

Pages: 138-149

Print publication date: 01/09/2017

Online publication date: 18/07/2017

Acceptance date: 14/07/2017

Date deposited: 16/10/2017

ISSN (print): 1567-7249

ISSN (electronic): 1872-8278

Publisher: Elsevier BV


DOI: 10.1016/j.mito.2017.07.006

PubMed id: 28729193


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