Chang-Hua Piao

Multifocal electroretinogram in occult macular dystrophy

PURPOSE Occult macular dystrophy (OMD) is an unusual macular dystrophy presenting with an essentially normal fundus and fluorescein angiography but with progressive central visual loss. The authors studied the function of local retinal areas in the posterior pole of patients with OMD using multifocal electroretinograms (ERGs). METHODS Multifocal ERGs were recorded using the Visual Evoked Response Imaging System with 61 hexagonal elements within a visual field of 30 degrees radius from 8 OMD patients and 20 age-matched, normal subjects. The amplitudes and implicit times of the patients and normal control subjects were compared at the various retinal eccentricities. RESULTs. The amplitudes of the multifocal ERGs in the OMD patients were markedly reduced in the central 7 degrees of the fovea. The difference of the ERG amplitudes between OMD and normal subjects became smaller toward the peripheral retina. Most OMD patients had slight but significantly delayed implicit times across the whole testing field, and the differences between the OMD and the normal subjects did not change with retinal eccentricity. CONCLUSIONS Our results for multifocal ERG amplitudes support the idea that OMD patients have localized retinal dysfunction distal to the ganglion cells in the central retina. The delayed implicit times across the whole test field suggest that the retinal dysfunction has a broader boundary than expected by ERG amplitudes and psychophysical perimetric results.

Correlation between Macular Volume and Focal Macular Electroretinogram in Patients with Retinitis Pigmentosa

PURPOSE To determine whether a significant correlation exists between the morphology of the macula measured by optical coherence tomography (OCT) and the amplitude of focal macular electroretinograms (fmERGs) in patients with retinitis pigmentosa (RP). METHODS fmERGs were recorded in 43 patients with RP and 43 age-similar normal subjects, with a 15 degrees stimulus spot, 5.6 to 5.8 mm in diameter on the fundus. The sum of the volume of the neural retina in the central 6 mm (total macular volume) was measured with the OCT system. The length of the photoreceptor inner segment/outer segment junction (IS/OS line) in a 6-mm diameter macular area was also measured in the OCT images. RESULTS There was a weak correlation between the total macular volume and the fmERG amplitudes (correlation coefficient, 0.46 for the a-wave and 0.54 for the b-wave). The fmERG amplitudes in the patients with RP with IS/OS line longer than 2 mm were significantly larger than those in patients with RP with IS/OS line shorter than 2 mm, but the correlations between these two factors were weak. One major reason for the low correlations between the macular morphology and fmERGs was that there were some patients with RP who had normal macular volume and long IS/OS line, but had severely reduced focal macular ERGs. CONCLUSIONS Although the macular volume and length of the IS/OS line correlated weakly with the amplitude of the fmERGs, a preserved macular morphology does not necessarily guarantee normal-amplitude fmERGs in patients with RP.

Amplitude Decrease of Photopic ERG b-Wave at Higher Stimulus Intensities in Humans

PURPOSE The b-wave of the human photopic electroretinogram (ERG) elicited by a short-flash increases in amplitude with increasing stimulus intensities at lower stimulus levels, but then decreases at higher stimulus intensities. The purpose of the present study was to explore this phenomenon in more detail, using short- and long-flash stimuli. METHODS The intensity-response functions of the b-wave elicited by short- and long-flashes were compared from threshold to higher stimulus intensities in 5 normal subjects. Short- and long-flash ERGs were elicited under rod-saturating background levels using white light-emitting diodes built into a contact lens electrode. RESULTS Whereas the amplitude of the short-flash b-wave decreased at higher intensities, the amplitude of the long-flash ERG b-wave did not decrease but plateaued. The long-flash ERG d-wave or OFF-response decreased at higher stimulus levels as did the short-flash elicited b-wave. CONCLUSIONS Because it is widely accepted that the b-wave and the OFF-response d-wave interact to produce a single positive response, our results suggest that the decrease in the b-wave amplitude at high stimulus intensity is caused by the decrease of the d-wave at the higher stimulus intensities.

Foveal Thickness in Occult Macular Dystrophy

PURPOSE Occult macular dystrophy (OMD) is an inherited macular dystrophy characterized by a progressive macular dysfunction without any visible fundus abnormality. We studied the foveal thickness in patients with OMD using optical coherence tomography (OCT). DESIGN Observational case series. Foveal thickness by OCT images. METHODS Foveal thickness obtained from 22 eyes of 11 patients with OMD was compared with that from 27 eyes of 20 age-matched normal controls. RESULTS Mean foveal thickness in the patients group (96.5 +/- 19.5 microm) was significantly thinner than that in the normal controls (133.3 +/- 9.0 microm, P <.0001, Mann-Whitney U test). Eighteen of 22 eyes with OMD had foveal thickness that were thinner than the lower limit of the normal range. There was no statistically significant correlation between the foveal thickness and visual acuity, age, or duration from onset. CONCLUSION These results demonstrated that there are significant anatomic changes in the macula of patients with OMD.

Relationship Between Retinal Layer Thickness and Focal Macular Electroretinogram Components After Epiretinal Membrane Surgery

PURPOSE To study the effect of epiretinal membrane (ERM) removal on the function and structure of the retina, and to determine whether the functional changes were correlated with the changes in the thickness of different retinal layers. METHODS Focal macular electroretinography (FMERG) and spectral-domain optical coherence tomography (SD-OCT) were performed on 17 eyes of 15 patients before and after ERM surgery. The parafoveal retina was divided into an inner layer, a middle layer, and an outer layer in the OCT images. The thickness of each layer was measured before and after the ERM surgery. The a-wave, b-wave, and oscillatory potentials (OPs) of the FMERGs were analyzed before and after the ERM surgery. RESULTS The thickness of the inner and middle retinal layers was significantly reduced after surgery (by 39% and 23%, respectively). The mean amplitudes of the b-waves and OPs at 6 months postoperatively were significantly larger than those recorded preoperatively (by 21% and 61%, respectively). The ratios of the pre- to postoperative b-wave and OP amplitudes were correlated with the thickness reduction of the middle retinal layer (b-wave, r = -0.51, P < 0.05; OPs, r = -0.82, P < 0.01). CONCLUSIONS The significant correlations between the reduction in the thickness of the middle retinal layer and increase in the amplitude of the b-waves and OPs suggest that the improvement of macular function after ERM peeling is due to the decrease in the thickness of the middle retinal layer.

Significant Correlation between Electroretinogram Parameters and Ocular Vascular Endothelial Growth Factor Concentration in Central Retinal Vein Occlusion Eyes

PURPOSE Central retinal vein occlusion (CRVO) leads to retinal ischemia, which then induces an upregulation of vascular endothelial growth factor (VEGF). The aim of this study was to determine whether a significant correlation exists between the ocular VEGF levels and the amplitudes and implicit times of different components of the electroretinogram (ERG) in eyes with a CRVO. METHODS The medical records of the 20 consecutive patients who had macular edema secondary to CRVO and were examined at the Nagoya University Hospital from November 2008 to February 2010 were reviewed. Because all the patients were scheduled to receive an intravitreal injection of bevacizumab (IVB), it was possible to collect samples of the aqueous humor before the IVB. The correlation between the different components of the ERGs and the VEGF concentration in the aqueous was determined. RESULTS The mean VEGF concentration of the aqueous humor was 416 pg/mL with a range of 100-1260 pg/mL. The b/a ratio of the single flash ERGs (P = 0.049; ρ = -0.45), implicit times of the cone a-wave (P = 0.028; ρ = 0.50), cone b-wave (P = 0.0059; ρ = 0.63), and 30 Hz flicker ERGs (P = 0.0058; ρ = 0.63) were significantly correlated with the VEGF concentration in the aqueous. CONCLUSIONS The significant correlations between the different components of the ERGs and the aqueous VEGF concentration indicate that full-field ERGs can be used to detect the CRVO patients at a high risk of developing neovascularization of the iris.

Focal macular electroretinograms in eyes with wet-type age-related macular degeneration.

PURPOSE To study the properties of the focal macular electroretinograms (fmERGs) in eyes with untreated wet-type age-related macular degeneration (AMD). METHODS fmERGs were recorded from 157 eyes of 157 consecutive patients with untreated wet-type AMD (113 men, 44 women; age, 71.3 +/- 8.0 years). The fmERGs were recorded under direct fundus observation using a modified infrared fundus camera and a 15 degrees stimulus spot. Amplitudes and implicit times of the fmERGs recorded from the AMD patients were compared with those from 21 age-similar healthy controls. RESULTS The amplitudes of fmERGs in the AMD patients were significantly smaller (P < 0.001) and the implicit times were significantly longer (P < 0.001) than the corresponding values in the control eyes. There was a significant correlation between amplitude and implicit time of the fmERG and visual acuity (logMAR), but the degree of correlation was weak. The difference in the b/a amplitude ratio between the AMD patients and healthy controls was not significant. CONCLUSIONS The significant reduction in amplitude and the severe delay in implicit times of a- and b-waves of the fmERGs indicated significant functional alterations in the inner and the outer retinal layers of the macular area of eyes with wet-type AMD.

Focal Macular Electroretinograms after Intravitreal Injections of Bevacizumab for Age-Related Macular Degeneration

PURPOSE To evaluate the changes in the best-corrected visual acuity (BCVA), macular thickness, and focal macular electroretinograms (FMERGs) after three intravitreal injections of bevacizumab for a choroidal neovascularization (CNV) associated with age-related macular degeneration (AMD). METHODS The medical records of 18 eyes of 18 patients who had received three consecutive monthly intravitreal injections of bevacizumab were retrospectively studied. The BCVA, macular thickness determined by optical coherence tomography (OCT), and FMERGs were measured before the first injection, and 10 days after each of the intravitreal bevacizumab injections. RESULTS The number of eyes with improvement in BCVA after the first injection was one (6%), after the second injection was four (22%), and after the third injection was five (28%). The number of eyes with reduction in macular thickness was 4 (33%), 8 (44%), and 10 (56%) after each of the three injections. The number of eyes with increase in b-wave amplitude of the FMERGs was 7 (38%), 6 (33%), and 10 (56%) after each of the three each injections. The mean macular thickness was significantly thinner after the first injection, and the mean BCVA was significantly improved after the second injection. The mean amplitude and implicit time of the b-wave of the FMERGs were significantly improved only after the third injection (P<0.05). CONCLUSIONS All parameters improved but the best was after the third injection, indicating that three monthly intravitreous injections with bevacizumab may be an effective treatment regimen for AMD.

Comparison of focal macular cone ERGs in complete-type congenital stationary night blindness and APB-treated monkeys☆

Focal macular cone electroretinograms (ERGs) and multifocal ERGs were recorded to study the macular function in patients with the complete-type of congenital stationary night blindness (cCSNB). The waveforms of the focal macular cone ERGs and the on- and off-responses of the multifocal ERGs in the cCSNB patients were similar to those recorded from monkey retinas treated with L-2 amino-4-phosphonobutyric acid (APB), suggesting that patients with cCSNB have a complete defect of the on-pathway even in the central retina. The results also demonstrated that there was a paradoxical positive response in the central retina of cCSNB patients, as compared to the negative full-field ERGs in the same subjects.

Restoration of Cone Interdigitation Zone Associated With Improvement of Focal Macular ERG After Fovea-Off Rhegmatogenous Retinal Reattachment

PURPOSE To determine whether a correlation exists between the parameters of the focal macular ERGs (FMERGs) and the microstructural changes of the photoreceptors after successful surgery for fovea-off rhegmatogenous retinal detachment (RRD). METHODS Twenty eyes of 20 patients who had undergone successful surgery to reattach the retina in eyes with fovea-off RRD were studied. Focal macular ERGs and spectral-domain OCT (SD-OCT) were recorded at 1 and 6 months after the surgery. Changes of the components of the FMERGs, as well as changes of the SD-OCT parameters including the length of the external limiting membrane (ELM), ellipsoid zone (EZ), cone interdigitation zone (CIZ), and size of the outer photoreceptor area (between ELM and RPE), were determined. RESULTS During the postoperative period, the mean amplitudes of the a-waves increased by 1.4 times and the b-waves by 1.7 times. Spectral-domain OCT showed that the mean length of the EZ and CIZ and the size of the outer photoreceptor area had increased significantly at 6 months. The degree of the increase in the CIZ and outer photoreceptor area was significantly correlated with the increase in the amplitudes of the b-waves of the FMERGs (r = 0.56, P = 0.042, r = 0.57, P = 0.040, respectively; Spearman rank correlation test). However, the length of the EZ was not significantly correlated with the increase of the b-waves. CONCLUSIONS A restoration of the EZ alone might not be enough to improve the FMERGs, and a restoration of the EZ accompanied by that of the CIZ was essential for the recovery of the FMERGs after fovea-off RRD.