ISCEV extended protocol for the photopic On–Off ERG

Šuštar, Maja; Holder, Graham E.; Kremers, Jan; Barnes, Claire S.; Lei, Bo; Khan, Naheed W.; Robson, Anthony G.

doi:10.1007/s10633-018-9645-y

Cited by 56 publications

(50 citation statements)

References 52 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…PERG is undetectable (4-12 and 4-14), shows a P50 component of short peak time (5-5 and 5-12) and a reduced N95:P50 ratio (5-5, 5-12; and Family 3, 2-3). PVEPs are abnormal in all cases and FVEPs undetectable in 1 (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). See text for details.…”

Section: Sanger Sequencingmentioning

confidence: 99%

“…Broken lines replace blink artifacts that occur soon after b-waves in DA10 ERGs and in the On-Off ERGs in Family 3, 2-3. All 5 cases show evidence of generalized retinal dysfunction with either similar severity of rod and cone involvement (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) or rod-cone dystrophy (4-12, 5-2, 5-5; Family 3, 2-3). There is evidence of progression in 5-5 between the ages of 18 and 26 years.…”

Section: Sanger Sequencingmentioning

confidence: 99%

“…[4][5][6][7] Family 2 underwent flash visual evoked potentials (VEP)s and pattern reversal VEPs to a 30-minute check in 3 of 4 cases. An extended protocol for photopic On-Off ERG and short-wavelength flash (S-cone) ERG were also performed.…”

Section: Study Population and Clinical Investigationmentioning

confidence: 99%

“…An extended protocol for photopic On-Off ERG and short-wavelength flash (S-cone) ERG were also performed. [4][5][6][7] Family 2 underwent flash visual evoked potentials (VEP)s and pattern reversal VEPs to a 30-minute check in 3 of 4 cases. Full-field ERGs (maximum dark-adapted and light adapted flash strength 2.25 cdÁs/m 2 ) were performed in all 4 individuals; large-field-pattern ERGs were analyzed in 1.…”

Section: Study Population and Clinical Investigationmentioning

confidence: 99%

See 3 more Smart Citations

SSBP1 mutations in dominant optic atrophy with variable retinal degeneration

Jurkutė

Leu

Pogoda

et al. 2019

Annals of Neurology

Self Cite

View full text Add to dashboard Cite

Objective: Autosomal dominant optic atrophy (ADOA) starts in early childhood with loss of visual acuity and color vision deficits. OPA1 mutations are responsible for the majority of cases, but in a portion of patients with a clinical diagnosis of ADOA, the cause remains unknown. This study aimed to identify novel ADOA-associated genes and explore their causality. Methods: Linkage analysis and sequencing were performed in multigeneration families and unrelated patients to identify disease-causing variants. Functional consequences were investigated in silico and confirmed experimentally using the zebrafish model. Results: We defined a new ADOA locus on 7q33-q35 and identified 3 different missense variants in SSBP1 (NM_001256510.1; c.113G>A [p.(Arg38Gln)], c.320G>A [p.(Arg107Gln)] and c.422G>A [p.(Ser141Asn)]) in affected individuals from 2 families and 2 singletons with ADOA and variable retinal degeneration. The mutated arginine residues are part of a basic patch that is essential for single-strand DNA binding. The loss of a positive charge at these positions is very likely to lower the affinity of SSBP1 for single-strand DNA. Antisense-mediated knockdown of endogenous ssbp1 messenger RNA (mRNA) in zebrafish resulted in compromised differentiation of retinal ganglion cells. A similar effect was View this article online at wileyonlinelibrary.com.Additional supporting information can be found in the online version of this article.

show abstract

Section: Sanger Sequencingmentioning

confidence: 99%

Section: Sanger Sequencingmentioning

confidence: 99%

Section: Study Population and Clinical Investigationmentioning

confidence: 99%

Section: Study Population and Clinical Investigationmentioning

confidence: 99%

See 2 more Smart Citations

SSBP1 mutations in dominant optic atrophy with variable retinal degeneration

Jurkutė

Leu

Pogoda

et al. 2019

Annals of Neurology

Self Cite

View full text Add to dashboard Cite

show abstract

“…ISCEV has also established an extended protocol for the photopic On-Off ERG to long duration (i.e. light onset/offset) that may provide complementary information in such conditions [13]. The method described here utilises a range of brief flashes that include the ISCEV standard LA 3.0 ERG.…”

Section: Scope and Applicationsmentioning

confidence: 99%

ISCEV extended protocol for the stimulus–response series for light-adapted full-field ERG

et al. 2019

Self Cite

View full text Add to dashboard Cite

The International Society for Clinical Electrophysiology of Vision (ISCEV) standard for full-field electroretinography (ERG) describes a minimum protocol for clinical testing but encourages additional ERG testing when appropriate. This ISCEV extended protocol describes methods to record and evaluate a light-adapted (LA) ERG stimulus-response series with increasing flash strengths. The LA ERG stimulus-response series (also referred to as the luminance-response or intensity-response series in the published literature) can characterise generalised cone system function more comprehensively than the ISCEV standard LA ERGs alone. The amplitude of LA ERG a-waves, arising from cones and cone offbipolar cells, typically shows a saturating function. The LA ERG b-wave amplitudes, which arise primarily from activity of retinal bipolar cells, show an amplitude peak followed by a nonzero plateau (the ''photopic hill'' phenomenon). This ISCEV extended protocol specifies a stimulus-response series

show abstract

Outcomes Associated With Sustained-Release Intraocular Fluocinolone Implants in a Case of Melanoma-Associated Retinopathy Treated Without Systemic Immunosuppression

2019

Self Cite

View full text Add to dashboard Cite

IMPORTANCE Melanoma-associated retinopathy (MAR) is a paraneoplastic syndrome in which antiretinal antibodies crossreact with retinal ON-bipolar cells, resulting in night blindness and progressive visual field loss. Current therapeutic options include cytoreductive surgery in combination with immunoglobulin, corticosteroids, or plasmapheresis, but their effectiveness is limited and may be contraindicated, given the possible protective role of circulating autoantibodies against metastatic spread. We report 3-year follow-up of the first case (to our knowledge) of MAR treated with intravitreal long-acting steroid implants.OBJECTIVE To report on a patient with MAR who was treated with intravitreal fluocinolone acetonide implants in the absence of systemic immunosuppression. DESIGN, SETTING, AND PARTICIPANTSThis is a 3-year follow-up of a 73-year-old woman with a history of surgical excision of a malignant melanoma of the left pinna who presented with visual symptoms of shimmering and nyctalopia. Fundus examination, fundus autofluorescence, and optical coherence tomography were normal, with no evidence of cystoid macular edema. Automated perimetry showed a reduction in visual field and full-field electroretinography (ERG) demonstrated findings consistent with generalized ON-bipolar cell dysfunction, typical of MAR. The patient was treated with bilateral fluocinolone acetonide intravitreal implants. MAIN OUTCOMES AND MEASURESVisual acuity, visual field, and electroretinography testing for 3 years after treatment. RESULTSVisual fields improved in this 73-year-old patient from 20/30 (Snellen measured as 6/9) OD and 20/16 (6/5) OS at baseline to 20/20 OU within 1 week of treatment. Detailed electroretinography monitoring indicated characteristic abnormalities that partly resolved after treatment, consistent with improved inner retinal ON-bipolar cell function. Bilateral cataracts developed approximately 2 years after injection; cataract surgery was performed uneventfully. At 3 years posttreatment, the patient remained visually stable and in systemic disease remission, with best-corrected visual acuity remaining at 20/20 OU. CONCLUSIONS AND RELEVANCEWe report what is, to our knowledge, the first case of MAR treated with intravitreal slow-release corticosteroid implants, which shows improvements in visual symptoms, visual fields, and retinal function. Sustained-release intraocular steroid implants may offer an effective and safe alternative to systemic immunosuppression in MAR, although results from 1 case should be generalized with abundant caution.

show abstract

ISCEV extended protocol for the photopic On–Off ERG

Cited by 56 publications

References 52 publications

SSBP1 mutations in dominant optic atrophy with variable retinal degeneration

SSBP1 mutations in dominant optic atrophy with variable retinal degeneration

ISCEV extended protocol for the stimulus–response series for light-adapted full-field ERG

Outcomes Associated With Sustained-Release Intraocular Fluocinolone Implants in a Case of Melanoma-Associated Retinopathy Treated Without Systemic Immunosuppression

Contact Info

Product

Resources

About