Clinical electrophysiology of two rod pathways: normative values and clinical application.
BACKGROUND
The scotopic 15-Hz flicker electroretinogram (ERG) has two limbs (slow and fast ERG rod signals), and these have been attributed to two retinal rod pathways (the ON rod bipolar and AII amacrine pathway and the rodcone gap-junction pathway). The aim of this study was to provide normative values of the scotopic 15-Hz flicker ERG, to estimate the inter-individual variability, and to apply this method to a clinical setting.


METHODS
Twenty-two normal subjects, one patient with retinitis pigmentosa (RP), and two patients with Stargardt's mascular dystrophy (SMD) participated in the study. The SMD patients were screened for mutations in the 50 exons of the ABCA4 (formerly ABCR) gene. We measured ERG response amplitudes and phases to flicker intensities ranging from -3.37 to -0.57 log scotopic trolands s at a flicker frequency of 15 Hz.


RESULTS
The normal scotopic 15-Hz flicker ERG showed a biphasic amplitude pattern with a minimum at about-1.57 log scotopic trolands s, where there was an abrupt phase shift of about 180 deg. The inter-individual variability in ERG amplitude ranged from 47% to 67% for the slow and from 41% to 64% for the fast rod signal. Both the RP patient and the SMD patients (who were compound heterozygotes for mutations in the ABCA4 gene) showed reduced amplitudes for the two rod ERG pathways.


CONCLUSIONS
The inter-individual variability might be explained by anatomical differences between individual retinae. In the RP patient, the amplitude reductions corresponded well with the standard rod ERG. In the SMD patients, however, the scotopic 15-Hz flicker ERG revealed rod dysfunction, whereas the standard rod ERG was within normal limits. The scotopic 15-Hz flicker method may be more sensitive than the standard rod ERG.
Scholl
H P
HP
University Eye Clinic, Schleichstrasse 12-16, 72076 Tübingen, Germany. hendrik.scholl@uni-tuebingen.de
Langrová
H
H
Weber
B H
BH
Zrenner
E
E
Apfelstedt-Sylla
E
E
D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D000328:Adult; D003623:Dark Adaptation; D004596:Electroretinography; D005260:Female; D006801:Humans; D008268:Macular Degeneration / Q000235:genetics / Q000503:physiopathology*; D008297:Male; D009154:Mutation; D012016:Reference Values; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000503:physiopathology*; D012374:Rod Cell Outer Segment / Q000503:physiopathology*; D014795:Visual Pathways / Q000503:physiopathology*
C056475:ABCA4 protein, human; D018528:ATP-Binding Cassette Transporters