98th Annual Meeting DOG 2000

K 315

Early detection of retinal dysfunction in Best’s disease using the multifocal ERG (MF-ERG)

A. M. Palmowski, R. Allgayer, B. Heinemann-Vernaleken, V. Scheerer

Purpose: To describe the MF-ERG as an additional tool in the diagnosis of Best’s disease.

Methods: Patient 1, a 30 year old female presented with loss of visual acuity (VA). A sister was known to have Best’s disease. Patient 2, a 36 yo female presented with visual obscurations. Clinical workup included perimetry, color vision testing, fluorescence angiographyy (FLA), ERG and EOG according to ISCEV standard. A MF-cone ERG (VERIS TM, 103 hexagons, m sequence 2^15, Lmax 200 cd/m^2, Contrast 99%) of the central 50 degrees was obtained.

Results: Ophthalmoscopy showed perimacular mottling of the RPE in both patients. Pat.1: VA: OD: 0.2, OS: 0.2. Farnsworth panel D15: protane/deutane anomaly. Pat.2: VA: 1.25 OU. Farnsworth panel D15: normal. Pat.1&2: Perimetry (Octopus d32) showed minor perifoveal relative scotomata. While the photopic and scotopic ERG were unremarkable, the Arden ratio was reduced. FLA revealed perifoveolar hyperfluorescence OU. When the relative contribution of concentrically averaged amplitudes to the overall response was analyzed, the first order of the MF-ERG, was remarkable for amplitude contributions outside the range of normal (age matched control: n=20) for N1P1 (first negative peak to first positive peak) and for P1N2 (P1 to the second negative peak) for all eccentricities OU.

Discussion: The MF-ERG can detect retinal dysfunction in Best disease prior to conventional Ganzfeld ERG. In contrast to other diseases, amplitudes rather than latencies seem to be affected in the MF-ERG of early Best’s disease.

Supported by DFG Pa 609/2
Universitäts- Augenklinik, D-66421 Homburg