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

TitleThe pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy.
Publication TypeJournal Article
Year of Publication2017
AuthorsMajander, A, Robson, AG, João, C, Holder, GE, Chinnery, PF, Moore, AT, Votruba, M, Stockman, A, Yu-Wai-Man, P
JournalMitochondrion
Date Published2017 Jul 18
ISSN1872-8278
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.

DOI10.1016/j.mito.2017.07.006
Alternate JournalMitochondrion
Citation Key10.1016/j.mito.2017.07.006
PubMed ID28729193