The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy.
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Peer-reviewed
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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.
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Keywords
Chromatic resolution, Critical flicker fusion, Leber hereditary optic neuropathy (LHON), Spatial contrast sensitivity, The pattern electroretinogram (PERG), The photopic negative responses (PhNR), Adolescent, Adult, Child, Female, Humans, Male, Middle Aged, Optic Atrophy, Hereditary, Leber, Prospective Studies, Retinal Ganglion Cells, Visual Pathways, Young Adult
Journal Title
Mitochondrion
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Journal ISSN
1567-7249
1872-8278
1872-8278
Volume Title
36
Publisher
Elsevier BV
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Sponsorship
Wellcome Trust (101876/Z/13/Z)