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K. Miyata, M. Nakamura, M. Kondo, S. Ueno, H. Terasaki; Reduction of Oscillatory Potentials and Photopic Negative Response in Patients With Dominant Optic Atrophy With OPA1 Mutations . Invest. Ophthalmol. Vis. Sci. 2006;47(13):3755.
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© ARVO (1962-2015); The Authors (2016-present)
To determine the electrophysiological findings in patients with dominant optic atrophy (DOA) with OPA1 gene mutations.
Eight DOA patients (age, 24–55 years; mean, 41.0 years) with OPA1 mutations were examined. In addition to routine ophthalmological tests, full–field ERGs, including the rod responses, mixed rod–cone responses, oscillatory potentials (OPs), single–flash cone response, 30–Hz flicker ERGs, and photopic negative responses (PhNR) were recorded. The results were compared to those from 30 age–matched controls. Analysis of retinal nerve fiver layer (RNFL) thickness by Stratus OCT and visual fields by static perimetry (Humphrey 30–2 program) were also performed.
The amplitude of the PhNR and OPs were significantly smaller for DOA patients than for normal controls (P <0.05). Other ERG components also tended to be smaller for DOA patients than for controls, but the differences were not statistically significant. The ratios of the amplitudes of the PhNR and OPs to the b–wave were also reduced for DOA patients. There was no significant correlation between the amplitudes of PhNR or OPs and the visual acuity and the RNFL thickness.
These results indicate that there are functional impairments of the retina of DOA patients with OPA1 mutations. The significant reduction of the amplitude of the PhNR and OPs suggest that the inner retinal function is most affected in these patients.
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