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

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Majander , A , Robson , A G , Joao , C , Holder , G E , Chinnery , P F , Moore , A T , Votruba , M , Stockman , A & Yu-Wai-Man , P 2017 , ' The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy ' , Mitochondrion , vol. 36 , pp. 138-149 . https://doi.org/10.1016/j.mito.2017.07.006

Title: The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
Author: Majander, A.; Robson, A. G.; Joao, C.; Holder, G. E.; Chinnery, P. F.; Moore, A. T.; Votruba, M.; Stockman, A.; Yu-Wai-Man, P.
Contributor: University of Helsinki, Department of Ophthalmology and Otorhinolaryngology
Date: 2017-09
Language: eng
Number of pages: 12
Belongs to series: Mitochondrion
ISSN: 1567-7249
URI: http://hdl.handle.net/10138/228672
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.
Subject: Leber hereditary optic neuropathy (LHON)
The pattern electroretinogram (PERG)
The photopic negative responses (PhNR)
Critical flicker fusion
Spatial contrast sensitivity
Chromatic resolution
PHOTOPIC NEGATIVE RESPONSE
THERAPY CLINICAL-TRIAL
ASYMPTOMATIC CARRIERS
ELECTROPHYSIOLOGICAL EVIDENCE
EXPERIMENTAL GLAUCOMA
COHERENCE TOMOGRAPHY
MTDNA MUTATIONS
VISUAL-SYSTEM
ATROPHY
NERVE
3125 Otorhinolaryngology, ophthalmology
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