Standard for clinical electroretinography

Marmor, Michael F.; Arden, G B; Nilsson, Sven-Erik; Zrenner, Eberhart

doi:10.1007/bf00154486

Cited by 517 publications

(246 citation statements)

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“…17,19,36 The photopic flash ERG recorded in the present series was a non-standard, which limits interpretation to comparisons within the subject group. 37 The posterior pole was illuminated evenly by the flashing screen, but the remaining retina was illuminated unevenly with scattered light. The stimulus was below the luminance level for the standard photopic ERG, which is already well below the saturation level for the a-wave.…”

Section: Discussionmentioning

confidence: 99%

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

McCulloch

Garcia-Filion

Boemel

et al. 2006

Eye

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Aims Optic nerve hypoplasia (ONH), which is defined as a congenital deficiency of retinal ganglion cells, may also involve more distal layers of the retina. We investigated electrophysiological function of the retina in ONH using electroretinograms (ERGs). Methods ERGs were recorded from 48 subjects (3.5-35 months) with unilateral or bilateral ONH. Pattern reversal (41 checks) was presented under chloral hydrate sedation, using an optical system to correct a cycloplegic refraction. A photopic flash stimulus was also used. Fundus photographs were used to measure the disk diameter/disk macula ratio (DD/DM), and to document other clinical signs. Eyes were classified as moderate (0.15-0.3) or severe (o0.15) ONH, and those with DD/DM greater than 0.3 were used as reference eyes. Results Pattern ERG recording was completed in 89 eyes and was detectable in 80% of eyes with ONH (61/76 tested) and in all 13 reference eyes. Photopic flash ERGs were of good quality in all eyes. The severity of ONH correlates with the amplitude of the photopic flash b-waves and with the amplitude of the N95 component of the pattern ERG (Po0.01). However, the ERGs to large patterns were well preserved (43.5 lV) in 10 of 35 eyes with severe ONH. Tortuous retinal vessels in eyes with either moderate or severe ONH were associated with smaller amplitude photopic b-waves and markedly diminished or undetectable pattern ERGs. Conclusions This study supports the hypothesis that retinal dysfunction distal to the ganglion cells is common in ONH, but is not predictable on the basis of ONH severity alone. Additionally, tortuous retinal vessels in ONH may be a sign associated with retinal dysfunction.

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Section: Discussionmentioning

confidence: 99%

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

McCulloch

Garcia-Filion

Boemel

et al. 2006

Eye

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“…A clinical ERG protocol that conformed to the standard of the International Society for the Clinical Electrophysiology of Vision was performed at both sites [20][21][22]. Scotopic testing included rod responses to short (λ < 470 nm, blue) and long wavelength (λ > 600 nm, red) stimuli matched in intensity to produce equal rod-mediated response amplitudes in normal subjects, as well as a bright white flash (mixed rod-cone response).…”

Section: Protocolmentioning

confidence: 99%

Effect of optimal dietary therapy upon visual function in children with long-chain 3-hydroxyacyl CoA dehydrogenase and trifunctional protein deficiency

Gillingham

Weleber

Neuringer

et al. 2005

Molecular Genetics and Metabolism

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The objective of this prospective cohort study was to determine if dietary therapy including docosahexaenoic acid (DHA; C22:6ω-3) supplementation prevents the progression of the severe chorioretinopathy that develops in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency. Physical, biochemical, and ophthalmological evaluations, including electroretinogram (ERG) and visual acuity by evoked potential (VEP), were performed at baseline and annually following the initiation of 65-130 mg/day DHA supplementation and continued treatment with a low-fat diet. Fourteen children with LCHAD or TFP deficiency, 1-12 years of age at enrollment, were followed for 2-5 years. Three subjects with TFP β-subunit mutations had normal appearance of the posterior pole of the ocular fundi at enrollment and no changes over the course of the study. Eleven subjects who were homozygote and heterozygote for the common mutation, c.1528G > C, had no change to severe progression of atrophy of the choroid and retina with time. Of these, four subjects had marked to severe chorioretinopathy associated with high levels of plasma hydroxyacylcarnitines and decreased color, night and/or central vision during the study. The plasma level of long-chain 3-hydroxyacylcarnitines, metabolites that accumulate as a result of LCHAD and TFP deficiency, was found to be negatively correlated with maximum ERG amplitude (R max ) (p=0.0038, R 2 =0.62). In addition, subjects with sustained low plasma long-chain NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author Manuscript 3-hydroxyacylcarnitines maintained higher ERG amplitudes with time compared to subjects with chronically high 3-hydroxyacylcarnitines. Visual acuity, as determined with the VEP, appeared to increase with time on DHA supplementation (p=0.051) and there was a trend for a positive correlation with plasma DHA concentrations (p=0.075, R 2 =0.31). Thus, optimal dietary therapy as indicated by low plasma 3-hydroxyacylcarnitine and high plasma DHA concentrations was associated with retention of retinal function and visual acuity in children with LCHAD or TFP deficiency.

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“…It is largely produced in relation to ON-(depolarising) bipolar cell function. [8][9][10] The ISCEV Standard ERG 11 incorporates a rod-specific response to a dim light under scotopic conditions, and a 'Standard; mixed rod-cone response to a bright white flash under dark adaptation. This latter response is dominated by rod function.…”

Section: Electroretinogrammentioning

confidence: 99%

Electrophysiological assessment of optic nerve disease

Holder

2004

Eye

128

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The electrophysiological findings in optic nerve and primary ganglion cell dysfunction are reviewed. The value of the pattern reversal visual-evoked potential (VEP) in the diagnosis of optic nerve disease, and the pattern appearance VEP in the demonstration of the intracranial misrouting associated with albinism, are discussed. The pattern electroretinogram (PERG) is used in the direct assessment of ganglion cell function. The use of PERG or multifocal electroretinography (mfERG), to enable the distinction between VEP delay due to optic nerve disease and that due to macular dysfunction, is described.

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Standard for clinical electroretinography

Cited by 517 publications

References 0 publications

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

Retinal function in infants with optic nerve hypoplasia: electroretinograms to large patterns and photopic flash

Effect of optimal dietary therapy upon visual function in children with long-chain 3-hydroxyacyl CoA dehydrogenase and trifunctional protein deficiency

Electrophysiological assessment of optic nerve disease

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