Reduction of Oscillatory Potentials and Photopic Negative Response in Patients with Autosomal Dominant Optic Atrophy with<i>OPA1</i>Mutations

Miyata, Kanji; Nakamura, Makoto; Kondo, Mineo; Lin, Jian; Ueno, Shinji; Miyake, Yoichi; Terasaki, Hiroko

doi:10.1167/iovs.06-0845

Cited by 68 publications

(55 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…12,15 PhNR is also selectively reduced in eyes with optic nerve atrophy, which is induced by trauma, compression, inflammation, ischemia, or gene mutation. [8][9][10] Our results provide additional evidence that focal macular PhNR is immediately and strongly attenuated by the onset of optic neuritis and that PhNR did not improve even after patients were treated with systemic corticosteroids, which resulted to improved visual acuity and visual field but not optic atrophy.…”

Section: Discussionmentioning

confidence: 61%

“…[1][2][3][4][5][6][7][8][9] In clinical studies using full-field ERGs, the PhNR amplitude was reduced in various diseases that involves RGC damageFfor example, glaucoma, [3][4][5] retinal vascular disease, 6,7 and optic nerve diseases. [8][9][10] Several researchers measured PhNR by using focal macular ERG in humans and monkeys. [11][12][13][14][15] Focal macular PhNR detected early glaucoma with higher sensitivity and specificity than full-field PhNR.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Focal macular photopic negative response in patients with optic neuritis

Nakamura

Miyamoto

Yokota

et al. 2011

Eye

View full text Add to dashboard Cite

Purpose To investigate, by focal macular electroretinography (ERG), the change of photopic negative response (PhNR) in the recovery of visual function in patients with optic neuritis. Methods Focal macular ERG was recorded from nine patients with acute optic neuritis (38.6±10.2 years). The photostimulator device projected 151 visual angle spotlight onto the macula. Focal macular ERG recording was performed at the onset and at 1 month and 6 months after the onset of optic neuritis. The results were compared between each recording for seven of the patients. Results All patients decreased in the vision below 20/100 and had central scotoma. Vision improved more than 20/20 within 1 month and full-visual field recovered within 6 months after the onset in all patients. The amplitude of the a-wave, b-wave, and PhNR of focal macular ERG at the onset was significantly attenuated in eyes with optic neuritis (66.8±15.5, 65.8±17.7, and 65.2±14.4% of normal control, respectively). The amplitude of the a-wave and b-wave increased gradually after steroid pulse therapy. The increase in a-wave amplitude was significant at 6 months (P ¼ 0.046), whereas the PhNR amplitude did not show any significant change over 6 months after the onset of optic neuritis. Conclusions Our results suggest that inflammation at the onset of optic neuritis leads to functional deficits that extend to at least the inner nuclear layers of the retina, and that all but the ganglion cell layers of retina recover.

show abstract

Section: Discussionmentioning

confidence: 61%

Section: Introductionmentioning

confidence: 99%

Focal macular photopic negative response in patients with optic neuritis

Nakamura

Miyamoto

Yokota

et al. 2011

Eye

View full text Add to dashboard Cite

show abstract

“…9,31 In addition, the electroretinograms in patients with ADOA are, in general, normal. 1,[41][42][43] Thus, the photoreceptor layer is most likely not affected in this disease. The overall sensory retinal thickness at the fovea was normal in patients with ADOA probably because the RNFL and ganglion cell layer are essentially absent in this area.…”

Section: Discussionmentioning

confidence: 99%

Reduction of Inner Retinal Thickness in Patients with Autosomal Dominant Optic Atrophy Associated withOPA1Mutations

Ito¹,

Nakamura²,

Yamakoshi³

et al. 2007

Invest. Ophthalmol. Vis. Sci.

Self Cite

View full text Add to dashboard Cite

PURPOSE.To determine the morphologic changes in the retina in the macula and around the optic disc in patients with autosomal dominant optic atrophy (ADOA) associated with a mutation in the OPA1 gene. METHODS. Cross-sectional images of the macular area of the retina were obtained by optical coherence tomography (OCT) in patients with ADOA who had a heterozygous mutation in the OPA1 gene. There were 15 eyes of eight patients from five families: four men and four women. The average age of the patients was 48.1 years. In the OCT images, the cross sections of the sensory retina were divided manually into four areas. The thickness of the overall sensory retina and the divided areas were measured at 1 and 2 mm on the temporal, nasal, superior, and inferior sides of the fovea as well as at the fovea. The thickness of the retinal nerve fiber layer (RNFL) around the optic discs was measured by taking circular scans (3.4 mm in diameter) centered on the optic disc. The results in the patients with ADOA were compared with those from 11 normal control subjects. RESULTS. The overall thickness of the sensory retina in the macular area was significantly thinner in the patients with ADOA than in the control subjects at all points except the fovea (P Ͻ 0.0001). The RNFL in the macular area in the patients with ADOA was significantly thinner than that in control subjects at all points (P Ͻ 0.0001), especially at 1 mm from the fovea. The circumpapillary RNFL was significantly thinner at the temporal, superior, and inferior areas in patients with ADOA but not in the nasal area. The total cross-sectional area of the circumpapillary RNFL was significantly correlated with visual acuity. The thickness of the combined ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer plexiform layer in the macular area was significantly thinner in the patients (P Ͻ 0.0056). The thickness of the outer nuclear layer and the photoreceptor inner segments and the thickness of the photoreceptor outer segments were not significantly different between the patients with ADOA and normal control subjects. CONCLUSIONS. The RNFL and the layer including the ganglion cell layer are significantly thinner in patients with ADOA associated with an OPA1 gene mutation, whereas the photoreceptor layers are not affected morphologically. The inner retina is the main area of the retina altered in ADOA. (Invest Ophthalmol Vis Sci. 2007;48:4079 -4086)

show abstract

“…Furthermore, it has been demonstrated that the ERG obtained in response to a long-duration red flash of moderate intensity provides optimal delineation of the PhNR ON and PhNR OFF components. 23,26,27 The brief-flash PhNR is attenuated in patients with ADOA 28 and in the Opa1 Q285STOP mutant mouse. 29 In the mouse model, the defect is seen before any changes in visual acuity on optokinetic drum testing and before morphologic changes on retinal histology.…”

Section: Purposementioning

confidence: 99%

“…Miyata et al 28 and Barnard et al 29 highlight the diagnostic potential of the PhNR in ADOA; however, the investigators used a brief white flash (broadband stimulus) to evoke the PhNR, which provides a poor signal-to-noise ratio compared with monochromatic stimuli, 26 and cannot distinguish ON and OFF components. Furthermore, the studies elicited full-field (global) PhNRs that, in contrast to the focal PhNR, are less sensitive in detecting focal retinal lesions, such as those seen in early to moderate glaucoma.…”

Section: Purposementioning

confidence: 99%

Electrophysiological ON and OFF Responses in Autosomal Dominant Optic Atrophy

Morny

Margrain

Binns

et al. 2015

Invest. Ophthalmol. Vis. Sci.

View full text Add to dashboard Cite

METHODS. Twelve participants from six families with OPA1 ADOA and 16 age-matched controls were recruited. Electrophysiological assessment involved pattern ERGs (PERGs), focal (208) and full-field long-duration (250 ms) flash ERGs using a red light-emitting diode flash on a rod-saturating blue background, and full-field brief (300 ls) xenon flash ERGs using a red filter over a continuous rod saturating blue background. Amplitudes and implicit times of the ERG components were analyzed and the diagnostic potential of each electrophysiological technique was determined by generating receiver operating characteristic (ROC) curves.RESULTS. Mean amplitudes of the N95 and all PhNRs, except the full-field PhNR ON , were significantly reduced in participants with ADOA (P < 0.01). Subtraction of the group-averaged focal ERG of ADOA participants from that of controls showed an equal loss in the focal PhNR ON and PhNR OFF components, whereas in the full-field ERG the loss in the PhNR OFF was greater than that in the PhNR ON component. The areas under the ROC curve (AUC) for the focal PhNR ON (0.92), focal PhNR OFF (0.95), and full-field PhNR OFF (0.83), were not significantly different from that of the PERG N95 (0.99).CONCLUSIONS. In patients with ADOA, the PhNR ON and PhNR OFF components are nearly symmetrically reduced in the long-duration ERG, suggesting that ON-and OFF-RGC pathways may be equally affected.

show abstract

Reduction of Oscillatory Potentials and Photopic Negative Response in Patients with Autosomal Dominant Optic Atrophy withOPA1Mutations

Abstract: These results suggested that there are functional impairments not only in the ganglion cell layer but also in the inner nuclear and plexiform layers, including the amacrine cells of ADOA patients with OPA1 mutations.

Cited by 68 publications

References 30 publications

Focal macular photopic negative response in patients with optic neuritis

Focal macular photopic negative response in patients with optic neuritis

Reduction of Inner Retinal Thickness in Patients with Autosomal Dominant Optic Atrophy Associated withOPA1Mutations

Electrophysiological ON and OFF Responses in Autosomal Dominant Optic Atrophy

Contact Info

Product

Resources

About