Pattern-reversal visual-evoked response in bull's eye maculopathy associated with Stargardt's disease

Using steady-state pattern-reversal visual-evoked response (PVER), we studied the macular function in patients with bull's eye maculopathy. The results were correlated with fluorescein angiography. Study patients with an established (25 eyes of 14 patients) and a suspected (8 eyes of 4 patients) diagnosis of Stargardt's disease with bull's eye maculopathy were divided into group G (good vision group: 15 eyes with visual acuity of 20/40 or better) and group P (poor vision group: 18 eyes with visual acuity of 20/50 or worse). The diameters of the atrophic area and the normal or less affected central area of the bull's eye were measured form the fluorescein angiograms. The mean diameter of the central area in group G (2.0 degrees) was significantly larger than that in group P (0.6 degrees; d.f. = 17, p = 0.0227). The PVER amplitudes were reduced in the patient groups with all check sizes and the amplitude-check size functions were flat. Amplitude differences were observed between the patient groups with the 20' checks (d.f. = 17, p = 0.0638), probably due to the difference in the mean central diameters (2.0 degrees vs. 0.6 degrees). Patients with a perifoveal abnormality can have an abnormal PVER despite relatively good visual acuity. We also recorded the PVER in 7 normal controls tested with simulated ring-shaped scotomas, the sizes of which matched the mean diameters of the scotomas in the patient groups (7.0 degrees x 0.6 degrees and 7.0 degrees x 2.0 degrees). The controls also showed markedly reduced responses with all check sizes.(ABSTRACT TRUNCATED AT 250 WORDS)