Faculty Bibliography

We examined the reliability of Humphrey visual field thresholds and multifocal electroretinogram (mfERG) amplitudes and timing in a group of patients with Retinitis Pigmentosa (RP). Eight patients with RP and seven control subjects were tested five times: at baseline (visit #0), at three weekly follow-up visits (visits #1 - #3), and at three months (visit #4). For the Humphrey thresholds, differences between dB values on repeat visits were obtained. Differences between log values on repeat visits were calculated for mfERG amplitude and implicit time. We used the standard deviations of these difference scores as a measure of reliability and the means of the difference scores as a measure of progression. We found that the majority of the patients' repeat data were more variable than that of the control subjects for both the Humphrey and mfERG. We found no single factor that predicted the magnitude, or the variance, of the SD of differences scores for the patients. We recommend that each patient's reliability be assessed individually. Ultimately, the choice of an outcome measure must be guided by its reliability, as well as its ability to assess the visual function of interest

The purpose of this study was to investigate atypical multifocal ERG (mfERG) responses for patients with diseases that can affect the photoreceptors. MfERGS were obtained from seven patients with retinitis pigmentosa (RP), three with progressive cone dystrophy (CD) and eight with diabetic retinopathy (DR). Both first- and second-order kernel responses were analyzed. The amplitudes and implicit times of the first-order responses were compared to those obtained from age-similar controls. For the first slice of the second-order response, the root-mean-square (RMS) and the signal-to-noise ratio (SNR) of each response were calculated. Achromatic visual fields were also obtained from each subject. For the three groups of patients, first-order responses with relatively large amplitudes, broad-shaped waveforms and markedly increased implicit times had non-measurable second-order responses. These responses were associated with areas of decreased visual field sensitivity. As RP, CD and DR affect the outer retina, the results are consistent with damage to the outer plexiform layer rather than damage to the inner retina

PURPOSE: To determine the extent of rod and cone photoreceptor dysfunction in patients with cone dystrophy using psychophysical and electrophysiological tests. METHODS: Ten patients with cone dystrophy participated. Rod and cone system psychophysical thresholds were measured as a function of retinal eccentricity. Bright-flash full-field electroretinograms were obtained under dark-adapted (rod-mediated) and light-adapted (cone-mediated) conditions. The a-wave data were fitted with a model based on photopigment transduction to obtain values for log Rmax (maximum response) and log S (sensitivity). b-Wave parameters were also examined by fitting a nonlinear, saturating function (the Naka-Rushton equation) to the rod-mediated responses. Oscillatory potentials were measured to the cone-mediated high-intensity flashes. RESULTS: On average, the rod-mediated psychophysical thresholds were elevated by 0.5 log unit. These threshold elevations did not differ significantly with retinal eccentricity. In contrast, cone-mediated psychophysical thresholds were elevated up to 3.0 log units. Threshold elevation was greatest in the central retinal locations. For rod-mediated conditions, the a-wave Rmax parameter was significantly reduced in three patients; the a-wave log S parameter was within normal limits. The rod-mediated b-wave Rmax parameter was reduced in six patients; log k was abnormal in one patient. For cone-mediated conditions, the a-wave Rmax parameter was reduced in six patients and the a-wave log S parameter was reduced in two patients. The cone system oscillatory potentials were abnormal in nine patients. CONCLUSIONS: Patients with cone dystrophy show different patterns of psychophysical rod versus cone system sensitivity losses with retinal eccentricity. The full-field electrophysiological data indicate that most of the patients had abnormal cone photoreceptor function. Some patients also showed rod photoreceptor abnormalities. The rod system changes were smaller than the cone system changes

PURPOSE: To determine the influence of age on local electroretinographic responses in humans. METHODS: Multifocal electroretinograms (mfERGs) were obtained from 62 normally sighted subjects ranging in age from 21 to 81 years. A stimulus array of 103 scaled hexagons was used to measure electrical signals within a retinal area approximately 46 degrees in diameter. Commonly reported mfERG methods were used to quantify the responses: peak-to-peak amplitudes and implicit times, scalar product amplitude, and amplitude and time scales derived from the algorithm of Hood and Li, published in 1997. RESULTS: Regression analysis showed significant linear relationships of amplitude and timing measures with age. The rates of losses were 10.5% per decade for peak-to-peak amplitude, 11.7% per decade for scalar product amplitude, and 9.5% per decade for a-scale. The rate of amplitude reduction was highest in the central 3 degrees. Age had less influence on implicit time measures. The rates of timing losses were 1.4% per decade for the N1 component and 1.0% per decade for both the P1 component and the t-scale measure. Using predicted interval ranges, the age was calculated at which 50% of the expected values would fall below the lower 95% prediction interval band of younger subjects. CONCLUSIONS: The age-associated mfERG alterations are presented to emphasize the importance of appropriate normative data in interpretation of mfERGs