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Documenta Ophthalmologica
Published in: Documenta Ophthalmologica 1/2017

Open Access 01-02-2017 | Original Research Article

The morphology of human rod ERGs obtained by silent substitution stimulation

Authors: J. Maguire, N. R. A. Parry, J. Kremers, I. J. Murray, D. McKeefry

Published in: Documenta Ophthalmologica | Issue 1/2017

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Abstract

Purpose

To record transient ERGs from the light-adapted human retina using silent substitution stimuli which selectively reflect the activity of rod photoreceptors. We aim to describe the morphology of these waveforms and examine how they are affected by the use of less selective stimuli and by retinal pathology.

Methods

Rod-isolating stimuli with square-wave temporal profiles (250/250 ms onset/offset) were presented using a 4 primary LED ganzfeld stimulator. Experiment 1: ERGs were recorded using a rod-isolating stimulus (63 ph Td, rod contrast, C rod = 0.25) from a group (n = 20) of normal trichromatic observers. Experiment 2: Rod ERGs were recorded from a group (n = 5) using a rod-isolating stimulus (C rod = 0.25) which varied in retinal illuminance from 40 to 10,000 ph Td. Experiment 3: ERGs were elicited using 2 kinds of non-isolating stimuli; (1) broadband and (2) rod-isolating stimuli which contained varying degrees of L- and M-cone excitation. Experiment 4: Rod ERGs were recorded from two patient groups with rod monochromacy (n = 3) and CSNB (type 1; n = 2).

Results

The rod-isolated ERGs elicited from normal subjects had a waveform with a positive onset component followed by a negative offset. Response amplitude was maximal at retinal illuminances <100 ph Td and was virtually abolished at 400 ph Td. The use of non-selective stimuli altered the ERG waveform eliciting more photopic-like ERG responses. Rod ERGs recorded from rod monochromats had similar features to those recorded from normal trichromats, in contrast to those recorded from participants with CSNB which had an electronegative appearance.

Conclusions

Our results demonstrate that ERGs elicited by silent substitution stimuli can selectively reflect the operation of rod photoreceptors in the normal, light-adapted human retina.
Literature
1.
Kremers J (2003) The assessment of L- and M-cone specific electroretinographical signals in the normal and abnormal human retina. Prog Ret Eye Res 22(5):79–605CrossRef
2.
Berson EL, Gouras P, Gunkel RD (1968) Rod responses in retinitis pigmentosa, dominantly inherited. Arch Ophthalmol 80:58–67CrossRefPubMed
3.
Berson EL, Gouras P, Gunkel RD, Myrianthopoulos NC (1969) Rod and cone responses in sex-linked retinitis pigmentosa. Arch Ophthalmol 81:215–225CrossRefPubMed
4.
Gouras P, Eggers HM, MacKay CJ (1983) Cone dystrophy, nyctalopia and supernormal rod responses. A new retinal degeneration. Arch Ophthalmol 101:718–724CrossRefPubMed
5.
6.
Scholl HPN, Langrova H, Weber BH, Zrenner E, Apfelstedt-Sylla E (2001) Clinical electrophysiology of two rod pathways: normative values and clinical application. Graefes Arch Clin Exp Ophthalmol 239(2):71–80CrossRefPubMed
7.
Petzold A, Plant GT (2006) Clinical disorders affecting mesopic vision. Ophthal Physiol Opt 26(3):326–341CrossRef
8.
Marmor M, Fulton AB, Holder GE et al (2009) ISCEV standard for full-field clinical electroretinography (2008 update). Doc Ophthalmol 118(1):69–77CrossRefPubMed
9.
10.
Estevez O, Spekreijse H (1982) The “silent substitution” method in visual research. Vis Res 22(6):681–691CrossRefPubMed
11.
Shapiro AG, Pokorny J, Smith VC (1996) Cone–rod receptor spaces with illustrations that use CRT phosphor and light-emitting-diode spectra. J Opt Soc Am A 13(12):2319–2328CrossRef
12.
13.
Maguire J, Parry NRA, Kremers J et al (2016) Rod electroretinograms elicited by silent substitution stimuli from the light adapted human eye. Trans Vis Sci Tech 5(4):10CrossRef
14.
Allen AE, Lucas RJ (2016) Using silent substitution to track the mesopic transition from rod- to cone-based vision in mice. Invest Ophthalmol Vis Sci 57:276–287CrossRefPubMed
15.
Alpern M, Falls HF, Lee GB (1960) The enigma of typical total monochromacy. Am J Ophthalmol 50:996–1012CrossRefPubMed
16.
Kohl S, Marx T, Giddings I et al (1998) Total colour blindness is caused by mutations in the gene encoding the alpha-subunit of the cone photoreceptor cGMP-gated cation channel. Nat Genet 19:257–259CrossRefPubMed
17.
Khan NW, Wissinger B, Kohl S, Sieving PA (2007) CNGB3 achromatopsia with progressive loss of residual cone function and impaired rod-mediated function. Invest Ophthalmol Vis Sci 48:3864–3871CrossRefPubMed
18.
Zeitz C, Robson AG, Audo I (2015) Congenital stationary night blindness: an analysis and update of genotype-phenotype correlations and pathogenic mechanisms. Prog Ret Eye Res 45:58–110CrossRef
19.
Miyake Y, Yagasaki K, Horiguchi M, Kawase Y, Kanda T (1986) Congenital stationary night blindness with negative electroretinogram. A new classification. Arch Ophthalmol 104:1013–1020CrossRefPubMed
20.
Dryja TP, McGee TL, Berson EL et al (2005) Night blindness and abnormal cone electroretinogram ON responses in patients with mutations in the GRM6 gene encoding mGluR6. Proc Natl Acad Sci USA 102:4884–4889CrossRefPubMedPubMedCentral
21.
Sergouniotis PI, Robson AG, Li Z et al (2011) A phenotypic study of congenital stationary night blindness (CSNB) associated with mutations in the GRM6 gene. Acta Ophthalmol 90:192–197CrossRef
22.
Stockman A, MacLeod DI, Johnson NE (1993) Spectral sensitivities of the human cones. J Opt Soc Am A 10(12):2491–2521CrossRef
23.
Wyszecki G, Stiles WS (1982) Color science; concepts and methods, quantitative data and formulae, 2nd edn. Wiley, New York
24.
Gouras P, Gunkel RD (1964) The frequency response of normal, rod achromat and nyctalope ERGs to sinusoidal monochromatic light stimulation. Doc Ophthalmol 18:137–150CrossRefPubMed
25.
Stockman A, Sharpe LT, Ruther K, Nordby K (1995) Two signals in the human rod visual system: a model based on electrophysiological data. Vis Neurosci 12(5):951–970CrossRefPubMed
26.
Bijveld MMC, Kappers AML, Riemslag FCC et al (2011) An extended 15 Hz ERG protocol (1): the contributions of primary and secondary rod pathways and the cone pathway. Doc Ophthalmol 123(3):149–159CrossRefPubMed
27.
Bijveld MM, Riemslag FC, Kappers AM, Hoeben FP, van Genderen MM (2011) An extended 15 Hz erg protocol (2): data of normal subjects and patients with achromatopsia, csnb1 and csnb2. Doc Ophthalmol 123(3):161–172CrossRefPubMed
28.
Robson JG, Frishman LJ (1998) Dissecting the dark-adapted electroretinogram. Doc Ophthalmol 95:187–215CrossRefPubMed
29.
Sieving PA, Frishman LJ, Steinberg R (1986) Scotopic threshold response of proximal retina in cat. J Neurophysiol 56:1049–1061PubMed
30.
Sieving PA, Murayama K, Naarendorp F (1994) Push–pull model of the primate photopic electroretinogram: a role for hyperpolarizing neurons in shaping the b-wave. Vis Neurosci 11:519–532CrossRefPubMed
31.
Wang I, Khan NW, Branham K, Wissinger B, Kohl S, Heckenlively JR (2012) Establishing baseline rod electroretinogram values in achromatopsia and cone dystrophy. Doc Ophthalmol 125:229–233CrossRefPubMed
32.
Audo I, Robson AG, Holder GE, Moore AT (2008) The negative ERG: clinical phenotypes and disease mechanisms of inner retinal dysfunction. Surv Ophthalmol 53:16–40CrossRefPubMed
33.
Remmer MH, Rastogi N, Ranka MP, Ceisler EJ (2015) Achromatopsia: a review. Curr Opin Ophthalmol 26:333–340CrossRefPubMed
34.
Moskowitz A, Hansen RM, Akula JD, Eklund SE, Fulton AB (2009) Rod and rod-driven function in achromatopsia and blue cone monochromatism. Invest Ophthalmol Vis Sci 50:950–958CrossRefPubMed
35.
Genead MA, Fishman GA, Rha J, Dubis AM, Bonci DMO, Dubra A, Stone EM, Neitz M, Carroll J (2011) Photoreceptor structure and function in patients with congenital achromatopsia. Invest Ophthalmol Vis Sci 52:7298–7308CrossRefPubMedPubMedCentral
36.
Stockman A, Sharpe LT (2006) Into the twilight zone: the complexities of mesopic vision and luminous efficiency. Ophthal Physiol Opt 26:225–239CrossRef
37.
Smith RG, Freed MA, Sterling P (1986) Microcircuitry of the dark-adapted retina: functional architecture of the rod-cone network. J Neurosci 6:3505–3517PubMed
38.
Bloomfield SA, Dacheux RF (2001) Rod vision: pathways and processing in the mammalian retina. Prog Ret Eye Res 20:351–384CrossRef
39.
Sterling P, Freed M, Smith RG (1988) Architecture of rod and cone circuits to the On-beta ganglion cell. J Neurosci 8:623–642PubMed
40.
Slaughter MM, Miller RF (1985) Characterization of an extended glutamate receptor of the ON bipolar neuron in the vertebrate retina. J Neurosci 5:224–233PubMed
41.
Witkovsky P, Dudek FE, Ripps H (1975) Slow PIII component of the carp electroretinogram. J Gen Physiol 65:119–134CrossRefPubMed
42.
Frishman LJ, Steinberg R (1990) Origin of negative potentials in the light-adapted ERG of cat retina. J Neurophysiol 63:1333–1346PubMed
43.
44.
Granit R (1947) Sensory mechanisms of the retina. Oxford University Press, London
45.
Eksandh L, Kohl S, Wissinger B (2002) Clinical features of achromatopsia in Swedish patients with defined genotypes. Ophthalmic Genet 23:109–120CrossRefPubMed
46.
Nishiguchi KM, Sandberg MA, Gorji N, Berson EL, Dryja TP (2005) Cone cGMP-gated channel mutations and clinical findings in patients with achromatopsia, macular degeneration, and other hereditary cone diseases. Hum Mutat 25:248–258CrossRefPubMed
47.
Brown KT, Murakami M (1967) Delayed decay of the late receptor potential of monkey cones as a function of stimulus intensity. Vis Res 7:179–189CrossRefPubMed
48.
49.
Scholl HPN, Kremers J (2001) Electroretinograms in s-cone monochromacy using s-cone and rod isolating stimuli. Color Res Appl 26:S136–S139CrossRef
50.
Chen C, Zuo C, Piao C, Miyake Y (2005) Recording rod ON and OFF responses in ERG and multifocal ERG. Doc Ophthalmol 111:73–81CrossRefPubMed
51.
Hood DC, Finkelstein MA (1986) Sensitivity to light. In: Boff K, Kaufman L, Thomas J (eds) Handbook of Perception and Human Performance, vol 1. Wiley, New York, p 5-1–5-66
52.
Aguilar M, Stiles W (1954) Saturation of the rod mechanism of the retina at high levels of stimulation. J Mod Opt 1:59–65
53.
Cameron MA, Lucas RJ (2009) Influence of the rod photoresponse on light adaptation and circadian rhythmicity in the cone ERG. Mol Vis 15:2209–2216PubMedPubMedCentral
54.
Frumkes TE, Naarendorp F, Goldberg SH (1986) The influence of cone adaptation upon rod mediated flicker. Vis Res 26:1167–1176CrossRefPubMed
55.
Heikkinen H, Vinberg F, Nymark S, Koskelainen A (2011) Mesopic background lights enhance dark-adapted cone ERG flash responses in the intact mouse retina: a possible role for gap junctional decoupling. J Neurophysiol 105:2309–2318CrossRefPubMed
56.
Farrow K, Teixeira M, Szikra T et al (2013) Ambient illumination toggles a neuronal circuit switch in the retina and visual perception at cone threshold. Neuron 78:1–14CrossRef
57.
Volgyi B, Deans MR, Paul DL, Bloomfield SA (2004) Convergence and segregation of the multiple rod pathways in mammalian retina. J Neurosci 24:11182–11192CrossRefPubMedPubMedCentral
58.
Metadata
Title
The morphology of human rod ERGs obtained by silent substitution stimulation
Authors
J. Maguire
N. R. A. Parry
J. Kremers
I. J. Murray
D. McKeefry
Publication date
01-02-2017
Publisher
Springer Berlin Heidelberg
Published in
Documenta Ophthalmologica / Issue 1/2017
Print ISSN: 0012-4486
Electronic ISSN: 1573-2622
DOI
https://doi.org/10.1007/s10633-017-9571-4

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