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      ISCEV extended protocol for the photopic On–Off ERG


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          The International Society for Clinical Electrophysiology of Vision (ISCEV) standard for full-field electroretinography (ERG) describes a minimum procedure, but encourages more extensive testing. This ISCEV extended protocol describes an extension to the ERG standard, namely the photopic On–Off ERG, and outlines common clinical applications. A light stimulus duration of 150–200 ms is used in the presence of a rod-suppressing background to elicit cone-driven On- and Off-system ERG components. The On-response occurs after the stimulus onset and has a negative a-wave and positive b-wave. The Off d-wave is a positive component evoked by stimulus offset. Common diagnoses that may benefit from additional photopic On–Off ERG testing include retinal dystrophies and retinal disorders that cause dysfunction at a level that is post-phototransduction or post-receptoral. On–Off ERGs assess the relative involvement of On- and Off-systems and may be of use in the diagnosis of disorders such as complete and incomplete congenital stationary night blindness (complete and incomplete CSNB), melanoma-associated retinopathy, and some forms of autoimmune retinopathy. The photopic On–Off ERGs may also be useful in X-linked retinoschisis, Batten disease, Duchenne muscular dystrophy, spinocerebellar degeneration, quinine toxicity, and other retinal disorders.

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          Most cited references 57

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          Night blindness and abnormal cone electroretinogram ON responses in patients with mutations in the GRM6 gene encoding mGluR6.

          We report three unrelated patients with mutations in the GRM6 gene that normally encodes the glutamate receptor mGluR6. This neurotransmitter receptor has been shown previously to be present only in the synapses of the ON bipolar cell dendrites, and it mediates synaptic transmission from rod and cone photoreceptors to this type of second-order neuron. Despite the synaptic defect, best visual acuities were normal or only moderately reduced (20/15 to 20/40). The patients were night blind from an early age, and when maximally dark-adapted, they could perceive lights only with an intensity equal to or slightly dimmer than that normally detected by the cone system (i.e., 2-3 log units above normal). Electroretinograms (ERGs) in response to single brief flashes of light had clearly detectable a-waves, which are derived from photoreceptors, and greatly reduced b-waves, which are derived from the second-order inner retinal neurons. ERGs in response to sawtooth flickering light indicated a markedly reduced ON response and a nearly normal OFF response. There was no subjective delay in the perception of suddenly appearing white vs. black objects on a gray background. These patients exemplify a previously unrecognized, autosomal recessive form of congenital night blindness associated with a negative ERG waveform.
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            Push-pull model of the primate photopic electroretinogram: a role for hyperpolarizing neurons in shaping the b-wave.

            Existing models of the primate photopic electroretinogram (ERG) attribute the light-adapted b-wave to activity of depolarizing bipolar cells (DBCs), mediated through a release of potassium that is monitored by Müller cells. However, possible ERG contributions from OFF-bipolar cells (HBCs) and horizontal cells (HzCs) have not been explored. We examined the contribution of these hyperpolarizing second-order retinal cells to the photopic ERG of monkey by applying glutamate analogs to suppress photoreceptor transmission selectively to HBC/HzCs vs. DBCs. ERGs of Macaca monkeys were recorded at the cornea before and after intravitreal injection of drugs. Photopic responses were elicited by bright 200-220 ms flashes on a steady background of 3.3 log scotopic troland to suppress rod ERG components. 2-amino-4-phosphonobutyric acid (APB), which blocks DBC light responses, abolished the photopic b-wave and indicated that DBC activity is requisite for photopic b-wave production. However, applying cis-2,3-piperidine dicarboxylic acid (PDA) and kynurenic acid (KYN), to suppress HBCs/HzCs and third-order neurons, revealed a novel ERG response that was entirely positive and was sustained for the duration of the flash. The normally phasic b-wave was subsumed into this new response. Applying n-methyl-dl-aspartate (NMA) did not replicate the PDA+KYN effect, indicating that third-order retinal cells are not involved. This suggests that HBC/HzC activity is critical for shaping the phasic b-wave. Components attributable to depolarizing vs. hyperpolarizing cells were separated by subtracting waveforms after each drug from responses immediately before. This analysis indicated that DBCs and HBC/HzCs each can produce large but opposing field potentials that nearly cancel and that normally leave only the residual phasic b-wave response in the photopic ERG. Latency of the DBC component was 5-9 ms slower than the HBC/HzC component. However, once activated, the DBC component had a steeper slope. This resembles properties known for the two types of cone synapses in lower species, in which the sign-preserving HBC/HzC synapse has faster kinetics but probably lower gain than the slower sign-inverting G-protein coupled DBC synapse. A human patient with "unilateral cone dystrophy" was found to have a positive and sustained ERG that mimicked the monkey ERG after PDA+KYN, indicating that these novel positive photopic responses can occur naturally even without drug application. These results demonstrate that hyperpolarizing second-order neurons are important for the primate photopic ERG.(ABSTRACT TRUNCATED AT 400 WORDS)
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              Phenotypic variation in enhanced S-cone syndrome.

              To characterize the clinical, psychophysical, and electrophysiological phenotype of 19 patients with enhanced S-cone syndrome (ESCS) and relate the phenotype to the underlying genetic mutation. Patients underwent ophthalmic examination and functional testing including pattern ERG, full-field ERG, and long-duration and short-wavelength stimulation. Further tests were performed in some patients, including color contrast sensitivity (CCS), multifocal ERG, fundus autofluorescence imaging (FAI), optical coherence tomography (OCT), and fundus fluorescein angiography (FFA). Mutational screening of NR2E3 was undertaken in 13 patients. The fundus appearance was variable, from normal to typical nummular pigment clumping at the level of the retinal pigment epithelium in older patients. Nine patients had foveal schisis, and one had peripheral schisis. Pattern ERG was abnormal in all patients. In all patients, ISCEV Standard photopic and scotopic responses had a similar waveform, the rod-specific-ERG was undetectable and the 30-Hz flicker ERG was markedly delayed with an amplitude lower than the photopic a-wave. Most ERG responses arose from short-wavelength-sensitive mechanisms, and a majority of patients showed possible OFF-related activity. Multifocal ERG showed relative preservation of central function, but reduced responses with increased eccentricity. Mutations were identified in NR2E3 in 12 of 13 patients including four novel variants. The phenotype in ESCS is variable, both in fundus appearance and in the severity of the electrophysiological abnormalities. The ERGs are dominated by short-wavelength-sensitive mechanisms. The presence, in most of the patients, of possible OFF-related ERG activity is a finding not usually associated with S-cones.

                Author and article information

                Doc Ophthalmol
                Doc Ophthalmol
                Documenta Ophthalmologica. Advances in Ophthalmology
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                22 June 2018
                22 June 2018
                : 136
                : 3
                : 199-206
                [1 ]ISNI 0000 0004 0571 7705, GRID grid.29524.38, Eye Hospital, , University Medical Centre Ljubljana, ; Ljubljana, Slovenia
                [2 ]ISNI 0000 0000 8726 5837, GRID grid.439257.e, Department of Electrophysiology, , Moorfields Eye Hospital, ; London, UK
                [3 ]ISNI 0000 0001 2180 6431, GRID grid.4280.e, Department of Ophthalmology, , National University of Singapore, ; Singapore, Singapore
                [4 ]ISNI 0000 0004 1936 834X, GRID grid.1013.3, University of Sydney Medical School, ; Sydney, Australia
                [5 ]ISNI 0000000121901201, GRID grid.83440.3b, Institute of Ophthalmology, , University College London, ; London, UK
                [6 ]ISNI 0000 0000 9935 6525, GRID grid.411668.c, Department of Ophthalmology, , University Hospital Erlangen, ; Erlangen, Germany
                [7 ]Palo Alto, USA
                [8 ]Henan Eye Institute, Henan Eye Hospital, Zhengzhou, China
                [9 ]ISNI 0000000086837370, GRID grid.214458.e, Department of Ophthalmology and Visual Science, , University of Michigan, ; Ann Arbor, USA
                © The Author(s) 2018

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                Funded by: FundRef http://dx.doi.org/10.13039/501100000272, National Institute for Health Research;
                ISCEV Standards
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                © Springer-Verlag GmbH Germany, part of Springer Nature 2018


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