1. The pattern of retinal ganglion cell dysfunction in Leber hereditary optic neuropathy
- Author
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Majander, A, Robson, AG, João, C, Holder, GE, Chinnery, PF, Moore, AT, Votruba, M, Stockman, A, and Yu-Wai-Man, P
- Subjects
Biochemistry and Cell Biology ,Genetics ,Biological Sciences ,Eye Disease and Disorders of Vision ,Clinical Research ,Neurosciences ,Neurological ,Eye ,Adolescent ,Adult ,Child ,Female ,Humans ,Male ,Middle Aged ,Optic Atrophy ,Hereditary ,Leber ,Prospective Studies ,Retinal Ganglion Cells ,Visual Pathways ,Young Adult ,Chromatic resolution ,Critical flicker fusion ,Leber hereditary optic neuropathy ,Spatial contrast sensitivity ,The pattern electroretinogram ,The photopic negative responses ,Biochemistry & Molecular Biology ,Biochemistry and cell biology - 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.
- Published
- 2017