ArticleInvestigative ophthalmology & visual science2013
Photoreceptor and postreceptor responses in congenital stationary night blindness.
Article in Investigative ophthalmology & visual science, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed.
- Genetic Loss of RORα Impairs Visual Function With Rod Bipolar Cell Degeneration In Mice.Investigative ophthalmology & visual science · 2025Article
- Heterogeneity and molecular landscape of melanoma: implications for targeted therapy.Molecular biomedicine · 2024Review
- Extracting the ON and OFF contributions to the full-field photopic flash electroretinogram using summed growth curves.Experimental eye research · 2019Article
- ISCEV extended protocol for the stimulus-response series for light-adapted full-field ERG.Documenta ophthalmologica. Advances in ophthalmology · 2019Article
- ISCEV extended protocol for the stimulus-response series for the dark-adapted full-field ERG b-wave.Documenta ophthalmologica. Advances in ophthalmology · 2019Article
- Disinhibition of intrinsic photosensitive retinal ganglion cells in patients with X-linked congenital stationary night blindness.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2019Article
- Phenotypic characterization of complete CSNB in the inbred research beagle: how common is CSNB in research and companion dogs?Documenta ophthalmologica. Advances in ophthalmology · 2018Article
- Deferoxamine-induced electronegative ERG responses.Documenta ophthalmologica. Advances in ophthalmology · 2018Article
- ISCEV extended protocol for the photopic On-Off ERG.Documenta ophthalmologica. Advances in ophthalmology · 2018Article
- Cone dystrophy and ectopic synaptogenesis in a Cacna1f loss of function model of congenital stationary night blindness (CSNB2A).Channels (Austin, Tex.) · 2018Article
- The importance of genetic testing as demonstrated by two cases ofMolecular vision · 2017Article
- Genotype-phenotype correlation in British families with X linked congenital stationary night blindness.The British journal of ophthalmology · 2003Article
- Optical Strategy Utilizing Contrast Modulation to Slow Myopia.Ophthalmology scienceReview
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4 authors.
Funding
Abstract
purposeTo investigate photoreceptor and postreceptor retinal function in patients with congenital stationary night blindness (CSNB).
methodsForty-one patients with CSNB (ages 0.19-32 years) were studied. ERG responses to a series of full-field stimuli were obtained under scotopic and photopic conditions and were used to categorize the CSNB patients as complete (cCSNB) or incomplete (iCSNB). Rod and cone photoreceptor (R(ROD), S(ROD), R(CONE), S(CONE)) and rod-driven postreceptor (V(MAX), log σ) response parameters were calculated from the a- and b-waves. Cone-driven responses to 30 Hz flicker and ON and OFF responses to a long duration (150 ms) flash were also obtained. Dark-adapted thresholds were measured. Analysis of variance was used to compare data from patients with cCSNB, patients with iCSNB, and controls.
resultsWe found significant reduction in saturated photoreceptor amplitude (R(ROD), R(CONE)) but normal photoreceptor sensitivity (S(ROD), S(CONE)) in both CSNB groups. Rod-driven postreceptor response amplitude (V(MAX)) and sensitivity (log σ) were significantly reduced in CSNB. Log σ was significantly worse in cCSNB than in iCSNB; this was the only scotopic parameter that differed between the two CSNB groups. Photopic b-wave amplitude increased monotonically with stimulus strength in CSNB patients rather than showing a normal photopic hill. The amplitude of the 30-Hz flicker response was reduced compared with controls, more so in iCSNB than in cCSNB. The mean dark-adapted threshold was significantly elevated in CSNB, more so in cCSNB than in iCSNB.
conclusionsThese results are evidence of normal photoreceptor function (despite the low saturated photoresponse amplitude) and anomalous postreceptor retinal circuitry.
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