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  • Title: The negative ERG is not synonymous with nightblindness.
    Author: Cibis GW, Fitzgerald KM.
    Journal: Trans Am Ophthalmol Soc; 2001; 99():171-5; discussion 175-6. PubMed ID: 11797304.
    Abstract:
    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.
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