LED-generated multifocal ERG on- and off-responses in complete congenital stationary night blindness -- a case report

We report on the application of a light emitting diode (LED) screen to elicit multifocal ERG on- and off-responses in a patient presenting with the complete type of congenital stationary night blindness (cCSNB): A 63-years old woman was diagnosed with cCSNB by means of standard ERG procedures and dark adaptometry. To confirm this diagnosis and to investigate topographical differences of on- and off-responses a multifocal approach employing long-duration stimuli was added. Results of mfERG-testing were averaged in three groups (a central area of 7.5 degrees , a ring area of 7.5-21.9 degrees and a peripheral ring of 21.9-31.1 degrees ). When compared to normal controls (n = 4) on-responses (P1-amplitudes) were severely reduced symmetrically at all eccentricities, while off-responses showed no reduction resulting in an increased off/on-ratio. Furthermore on-latencies of P1 were delayed symmetrically at all eccentricities, whereas off-latencies were normal. To our knowledge this is the first report of multifocal on- and off-responses in a CSNB-patient. Stimulus-generation with a LED-screen provides the advantage of a stable luminance during the long-duration on-phase.