1. Optical Coherence Tomography of the Retinal Ganglion Cell Complex in Leber's Hereditary Optic Neuropathy and Dominant Optic Atrophy
- Author
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Anne Stewart Gority Irvine, Anna Ter-Zakarian, Kaitlin Kogachi, Rustum Karanjia, Samuel Asanad, Alfredo A. Sadun, Piero Barboni, Khalil Ghasemi Falavarjani, Starleen E. Frousiakis, Jerry P Jiang, Jack J. Tian, Darvizeh Fatemeh, and Christian M. Felix
- Subjects
Adult ,Male ,Retinal Ganglion Cells ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Mitochondrial Diseases ,genetic structures ,Adolescent ,Nerve fiber layer ,Visual Acuity ,Optic Atrophy, Hereditary, Leber ,Retinal ganglion ,Optic neuropathy ,03 medical and health sciences ,Cellular and Molecular Neuroscience ,Young Adult ,0302 clinical medicine ,Nerve Fibers ,Ophthalmology ,Optic Atrophy, Autosomal Dominant ,medicine ,Humans ,Aged ,Retrospective Studies ,Retina ,business.industry ,Leber's hereditary optic neuropathy ,Middle Aged ,medicine.disease ,eye diseases ,Sensory Systems ,medicine.anatomical_structure ,Cross-Sectional Studies ,Retinal ganglion cell ,Acute Disease ,Chronic Disease ,030221 ophthalmology & optometry ,Optic nerve ,Female ,sense organs ,Mitochondrial optic neuropathies ,business ,030217 neurology & neurosurgery ,Tomography, Optical Coherence - Abstract
Background: Mitochondrial optic neuropathies such as Leber's Hereditary Optic Neuropathy (LHON) and Dominant Optic Atrophy (DOA) have been shown to produce an optic neuropathy secondary to retinal ganglion cell loss with thinning of the retinal ganglion cell complex (RGCC). Methods: We performed a retrospective analysis assessing the thicknesses of the peripapillary retinal nerve fiber layer (pRNFL) along with the macular retinal ganglion cell-inner plexiform layer (RGC-IPL) using optical coherence tomography (OCT). We compared these changes among acute and chronic LHON, DOA, and normal healthy control patients. Results: Patients with chronic LHON exhibited statistically significant thinning of the RNFL in the superior, nasal, and inferior quadrants of the retina. In acute LHON, the RNFL was relatively thicker in all but the temporal quadrant when compared with respective quadrants in normal eyes; however, statistical significance was not achieved. In DOA, the RNFL was thinnest in the superior and inferior quadrants of the retina, measuring between acute and chronic LHON thickness values. In chronic LHON and DOA, both the pRNFL and RGC-IPL were significantly thinner in all four retinal quadrants relative to controls. Conclusions: This article represents the first comparative study of the RGCC between LHON and DOA. Our findings demonstrated significant thickness reductions in pRNFL and macular RGC-IPL in patients with LHON and DOA, with different specific patterns consistent with the general patterns of thinning classically observed. This study suggests the usefulness of the RGCC as a potential in vivo biomarker for assessing disease in patients with LHON and DOA.
- Published
- 2019