LED-generated Multifocal ERG On- and Off-responses in Complete Congenital Stationary Night Blindness – A Case Report
- 67 Downloads
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°, a ring area of 7.5–21.9° and a peripheral ring of 21.9–31.1°). 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.
KeywordsLED multifocal ERG night blindness on- and off-response
Unable to display preview. Download preview PDF.
- 3.Rosenberg, T, Haim, M, Piczenik, Y, Simonsen, SE 1991Autosomal dominant stationary night-blindness. A large family rediscoveredActa Ophthalmol (Copenhagen)69694702Google Scholar
- 4.Pusch, CM, Zeitz, C, Brandau, O, Pesch, K, Achatz, H, Feil, S, Scharfe, C, Maurer, J, Jacobi, FK, Pinckers, A, Andreasson, S, Hardcastle, A, Wissinger, B, Berger, W, Meindl, A 2000The complete form of X-linked congenital stationary night blindness is caused by mutations in a gene encoding a leucine-rich repeat proteinNat Genet263247PubMedGoogle Scholar
- 5.Bech-Hansen, NT, Naylor, MJ, Maybaum, TA, Sparkes, RL, Koop, B, Birch, DG, Bergen, AA, Prinsen, CF, Polomeno, RC, Gal, A, Drack, AV, Musarella, MA, Jacobson, SG, Young, RS, Weleber, RG 2000Mutations in NYX, encoding the leucine-rich proteoglycan nyctalopin, cause X-linked complete congenital stationary night blindnessNat Genet2631923CrossRefPubMedGoogle Scholar
- 9.Kondo, M, Miyake, Y, Horiguchi, M, Suzuki, S, Tanikawa, A 2003 MarRecording multifocal electroretinogram on and off responses in humansInvest Ophthalmol Vis Sci3957480Google Scholar