The negative ERG is not synonymous with nightblindness.
| Autor: | Cibis GW; Sections of Ophthalmology and Rehabilitative Medicine, University of Missouri, Kansas City, USA., Fitzgerald KM |
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| Jazyk: | angličtina |
| Zdroj: | Transactions of the American Ophthalmological Society [Trans Am Ophthalmol Soc] 2001; Vol. 99, pp. 171-5; discussion 175-6. |
| Abstrakt: | Purpose: To provide electroretinographic differentiation between 4 genetically distinct conditions associated with a negative. Schubert Bornschein type electroretinogram (ERG): Complete congenital stationary night blindness (cCSNB), incomplete CSNB (incCSNB), Duchenne muscular dystrophy, and a family with an autosomal dominantly inherited negative ERG. Methods: ERGs were recorded in all subjects according to the ISCEV standards. Additionally, a long-duration flash was used under photopic testing conditions to separate depolarizing (ON) and hyperpolarizing (OFF) bipolar cell contributions. Dark adaptometry was obtained in cooperative adult subjects. Results: We were unable to differentiate between these 4 genetically distinct conditions using the scotopic ERG response to the bright white flash only. The photopic, cone-derived ERG to both short- and long-duration flashes was more informative in making distinctions between these 4 disorders and understanding the possible mechanisms behind the abnormal ERG. Conclusion: None of these disorders are progressive or a result of abnormal photoreceptor phototransduction. We suggest that they each represent a signal transmission error at the photoreceptor to depolarizing bipolar cell synapse that affects both rod and cone output. We propose that vision is spared in the latter 2 conditions because of timing errors in transmission as opposed to a complete signaling block, as seen in cCSNB. |
| Databáze: | MEDLINE |
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