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

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 electroretinograp...

Descripción completa

Detalles Bibliográficos
Autores principales: Majander, A., Robson, A.G., João, C., Holder, G.E., Chinnery, P.F., Moore, A.T., Votruba, M., Stockman, A., Yu-Wai-Man, P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier Science 2017
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5644721/
https://www.ncbi.nlm.nih.gov/pubmed/28729193
http://dx.doi.org/10.1016/j.mito.2017.07.006
_version_ 1783271780719263744
author Majander, A.
Robson, A.G.
João, C.
Holder, G.E.
Chinnery, P.F.
Moore, A.T.
Votruba, M.
Stockman, A.
Yu-Wai-Man, P.
author_facet Majander, A.
Robson, A.G.
João, C.
Holder, G.E.
Chinnery, P.F.
Moore, A.T.
Votruba, M.
Stockman, A.
Yu-Wai-Man, P.
author_sort Majander, A.
collection PubMed
description 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.
format Online
Article
Text
id pubmed-5644721
institution National Center for Biotechnology Information
language English
publishDate 2017
publisher Elsevier Science
record_format MEDLINE/PubMed
spelling pubmed-56447212017-10-19 The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy Majander, A. Robson, A.G. João, C. Holder, G.E. Chinnery, P.F. Moore, A.T. Votruba, M. Stockman, A. Yu-Wai-Man, P. Mitochondrion Article 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. Elsevier Science 2017-09 /pmc/articles/PMC5644721/ /pubmed/28729193 http://dx.doi.org/10.1016/j.mito.2017.07.006 Text en © 2017 The Authors http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Majander, A.
Robson, A.G.
João, C.
Holder, G.E.
Chinnery, P.F.
Moore, A.T.
Votruba, M.
Stockman, A.
Yu-Wai-Man, P.
The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
title The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
title_full The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
title_fullStr The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
title_full_unstemmed The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
title_short The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
title_sort pattern of retinal ganglion cell dysfunction in leber hereditary optic neuropathy
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5644721/
https://www.ncbi.nlm.nih.gov/pubmed/28729193
http://dx.doi.org/10.1016/j.mito.2017.07.006
work_keys_str_mv AT majandera thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT robsonag thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT joaoc thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT holderge thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT chinnerypf thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT mooreat thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT votrubam thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT stockmana thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT yuwaimanp thepatternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT majandera patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT robsonag patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT joaoc patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT holderge patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT chinnerypf patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT mooreat patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT votrubam patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT stockmana patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy
AT yuwaimanp patternofretinalganglioncelldysfunctioninleberhereditaryopticneuropathy

Ejemplares similares