Electrophysiological findings show generalised post-photoreceptoral deficiency in macular telangiectasia type 2

Ledolter, Anna A.; Holder, Graham E.; Ristl, Robin; Schmidt‐Erfurth, Ursula; Ritter, Markus

doi:10.1136/bjophthalmol-2017-310228

Cited by 5 publications

(6 citation statements)

References 31 publications

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“…A detailed clinical description of the patient cohort has previously been published. (Ledolter et al 2018) In summary, according to Gass and Blodi’s classification for MacTel (Gass & Blodi 1993), 6 eyes were at stage 1 of the disease, 1 eye at stage 2, 10 eyes at stage 3, 7 eyes at stage 4 and 5 eyes at stage 5. Six eyes of 6 patients had no clinically detectable retinal changes.…”

Section: Resultsmentioning

confidence: 99%

“…Multifocal electroretinography (mfERG) provides objective functional measures of localised macular function facilitating structure‐function correlations. Data have been published that show alterations of some electrophysiological parameters in MacTel (Gass & Oyakawa 1982; Barthelmes et al 2007; Narayanan et al 2013; Ledolter et al 2018; Okada et al 2018; Goel et al 2020). A reduction in mfERG N1 and P1 amplitudes and increased peak times has been reported (Narayanan et al 2013).…”

Section: Introductionmentioning

confidence: 99%

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Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure

Ledolter

Ristl

Palmowski-Wolfe

et al. 2021

Acta Ophthalmologica

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To assess the impact of neurodegenerative morphologic alterations due to macular telangiectasia type 2 (MacTel) on microperimetry (MP) and multifocal electroretinography (mfERG). Methods: Thirty-five eyes of 18 patients with MacTel were examined using spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), mfERG and MP. Software was used to match SD-OCT B-scans with the corresponding retinal sensitivity map and multifocal electroretinograms (mfERGs), thus enabling direct structure/function correlation. Results: Loss of the ellipsoid zone (EZ) had the strongest negative association with retinal sensitivity (16.77 dB versus 4.58 dB, adj. p < 0.001) of all parameters examined, and a limited negative effect on mfERGs (0.32 SD versus À1.97 SD adj. p = 0.121). Ellipsoid zone (EZ) irregularity was associated with reduced MP values but preserved mfERGs. There was a significant association between areas of inner retinal hyporeflectivity and loss of MP sensitivity (adj. p < 0.001) but the reduction in sensitivity was less than in locations with EZ loss. Areas of mfERG abnormality showed similar sensitivity loss with either inner retinal hyporeflectivity or EZ loss (adj. p = 0.063). In areas with EZ loss alone, preservation of the external limiting membrane (ELM) was associated with higher MP values than in areas with additional ELM loss; the integrity of the ELM alone was not associated with changes either in MP or mfERG. Increased FAF was observed in 51% of eyes, mixed/reduced FAF in 40%, and no abnormality was detected in 9% of eyes. Conclusion:The data suggest both MP and mfERG to be useful non-invasive modalities for detecting localised macular dysfunction in MacTel. The findings suggest a different sensitivity of the two modalities to inner and outer retinal changes in macular function and are therefore complementary.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure

Ledolter

Ristl

Palmowski-Wolfe

et al. 2021

Acta Ophthalmologica

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“…Ledolter performed full-eld ERG and pattern ERG in mac Tel eyes and suggested inner retinal dysfunction in rods and cones attributable to muller cell dysfunction. 16 Goel et al compared the mean P1 amplitudes and implicit time (IT) in R1 to R5 between Mac Tel eyes and controls and found that mean P1 amplitudes were signi cantly decreased in Mac Tel eyes compared to controls in R1. The P1 amplitudes also decreased gradually from R1 to R5.…”

Section: Discussionmentioning

confidence: 99%

Multimodal characterization of macular telangiectasia type 2 and correlation with multifocal electroretinogram

Ayachit

Reddy

Joshi

2021

Preprint

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1. Purpose: To study the correlation of multimodal imaging in macular telangiectasia (Mac Tel) with foveal function on multifocal electroretinogram (mfERG)2. Methods: Eyes with non- proliferative Mac Tel diagnosed based on clinical examination, optical coherence tomography (OCT), autofluorescence (AF), fluorescein angiography (FA), OCT angiography (OCTA). Control group with normal eye exam included for multifocal electroretinogram.Staging of OCT, OCTA, AF and FA done in Mac Tel subjects. mfERG done in study subjects and controls. Correlation of imaging modalities and P1 amplitudes at fovea (ring 1) studied in terms of correlation co-efficient.3. Results: Twenty nine eyes of 16 patients of Mac Tel and 25 eyes of 19 controls were included. BCVA was 0.38 ± 0.266 in study eyes and 0 in control eyes. On OCT it was observed that 41.4% Mac Tel eyes (n = 12) belonged to stage 3, 37.9%(n = 11)) eyes belonged to stage 2 and 20.7% (n = 6) eyes belonged to stage 1. AF- Stage 3 comprised of 75.9% eyes (n = 22); 4 eyes belonged to stage 2 and 3 eyes to stage 1. On FA, 18 eyes belonged to stage 3 (62.1%); stage 2 was seen in 1 (3.4%) eye and stage 1 was seen in 10 (34.5%) eyes. There was decrease in P1 amplitudes from R1(p < 0.001), R2( 0.001), R3 (< 0.001) and R4 (0.001) in Mac Tel eyes compared to control eyes but not in R5 (p 0.785). SD- OCT had positive correlation with FAF (CC 0.747,p < 0.001) FFA (CC 0.775, p < 0.001) and R1P1 (CC 0.682, p < 0.001). With OCTA there was no significant correlation (CC 0.318, p 0.093). There was positive and significant correlation of OCT (0.682,<0.001), OCTA (0.379,p 0.042) AF ( 0.635, p < 0.001) and FA (0.495, p < 0.006) with R1P1.4. Conclusions: Existing multimodal imaging systems can be reliable indicators of foveal function as on mfERG.

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“… 5 , 6 , 26 We cannot, however, completely exclude some contributions of d3 mechanism loss, which would be predicted based on electrophysiological observations in MacTel. 27 – 30 It will be noted that increment threshold testing could also be used to determine the contribution of postreceptoral losses to threshold elevation. 23 …”

Section: Discussionmentioning

confidence: 99%

“…5,6,26 We cannot, however, completely exclude some contributions of d3 mechanism loss, which would be predicted based on electrophysiological observations in MacTel. [27][28][29][30] It will be noted that increment threshold testing could also be used to determine the contribution of postreceptoral losses to threshold elevation. 23 A filter mechanism of sensitivity loss acting alone would be predicted to result in either no alteration in kinetics (for small changes in bleaching) or a reduction in t (for larger changes, based on empirical measurements; Figure 1).…”

Section: Discussionmentioning

confidence: 99%

Impairments in Cone Pigment Regeneration and Absolute Threshold in Macular Telangiectasia Type 2

Simunovic

Hess

Gillies

2021

Retina

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Electrophysiological findings show generalised post-photoreceptoral deficiency in macular telangiectasia type 2

Abstract: Both scotopic (rod system dominated) and photopic ERGs (cone system) are consistent with post-photoreceptoral dysfunction. There was no electrophysiological evidence of dysfunction at the level of the photoreceptor.

Cited by 5 publications

References 31 publications

Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure

Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure

Multimodal characterization of macular telangiectasia type 2 and correlation with multifocal electroretinogram

Impairments in Cone Pigment Regeneration and Absolute Threshold in Macular Telangiectasia Type 2

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