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D. Milea, B. Sander, M. Wegener, H. Jensen, B. Kjer, H. Lund-Andersen, M. Larsen; Retinal Axonal Loss in OPA1 Dominant Optic Atrophy, Measured With OCT. Invest. Ophthalmol. Vis. Sci. 2007;48(13):2465.
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© ARVO (1962-2015); The Authors (2016-present)
Dominant optic atrophy (DOA) is the most common hereditary optic neuropathy, resulting from a degeneration of the ganglion cells in the retina and subsequent ascendant neuropathy. Little is known about in vivo structural retinal changes in these patients.
In order to investigate retinal ganglion and nerve fiber layer characteristics, we compared data obtained with optical coherence tomography (OCT) in 10 affected patients and 20 control subjects.
DOA patients demonstrated diffusely attenuated peripapillary nerve fiber layers in all quadrants, the most prominent deficit being in the temporal and inferior quadrants, where nerve fiber layer thickness was 46% and 45% lower than in healthy subjects, respectively. Visual acuity in patients with DOA was correlated to the perioptic nerve fiber layer thickness in the inferior and superior regions (inferior r=0.76, p=0.01; superior r= 0.85, p=0.0022 ) and as well to macular thickness in the perifoveal region. Morphologically, the outer retinal layers had a normal appearance in DOA patients patients, in contrast to the pronounced deficit of the macular nerve fiber and ganglion cell layers. By extrapolation using regression analysis, it appears that the retinal thickness may be already decreased at birth in DOA patients and that the rate of the retinal thinning maybe higher throughout the lifetime compared to healthy individuals.
This study suggests that gradual loss of retinal ganglion cells and nerve fibers in DOA patients harbouring the same disease-linked mutation can be monitored noninvasively in vivo by OCT. Further prospective studies are needed in order to determine if retinal thinning rate is more rapid in DOA patients and if OCT may become a biomarker for optic nerve axonal integrity, quantifying the retinal axonal loss.
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