Analysis of retinal function using chromatic pupillography in retinitis pigmentosa and the relationship to electrically evoked phosphene thresholds

Kelbsch, Carina; Maeda, Fumiatsu; Lisowska, Jolanta; Lisowski, Łukasz; Straßer, Torsten; Stingl, Krunoslav; Wilhelm, Barbara; Wilhelm, Helmut; Peters, Tobias

doi:10.1111/aos.13259

Cited by 26 publications

(25 citation statements)

References 29 publications

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“…Retinitis pigmentosa (RP) is an inherited retinal degenerative disease with the progressive loss of photoreceptors, which leads to the deterioration of retinal function and morphology [1]. Mutation in specific genes that encode proteins participating in visual phototransduction is the primary cause of the death of photoreceptors.…”

Section: Introductionmentioning

confidence: 99%

Protection of retinal function and morphology in MNU-induced retinitis pigmentosa rats by ALDH2: an in-vivo study

Yan

Long²,

Wei

et al. 2020

BMC Ophthalmol

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Background: Retinitis pigmentosa (RP) is a kind of inherited retinal degenerative diseases characterized by the progressive loss of photoreceptors. RP has been a conundrum without satisfactory countermeasures in clinic until now. Acetaldehyde dehydrogenase 2 (ALDH2), a major enzyme involved in aldehyde detoxification, has been demonstrated to be beneficial for a growing number of human diseases, such as cardiovascular dysfunction, diabetes mellitus and neurodegeneration. However, its protective effect against RP remains unknown. Our study explored the impact of ALDH2 on retinal function and structure in N-methyl-N-nitrosourea (MNU)-induced RP rats. Methods: Rats were gavaged with 5 mg/kg Alda-1, an ALDH2 agonist, 5 days before and 3 days after MNU administration. Assessments of retinal function and morphology as well as measurement of specific proteins expression level were conducted. Results: Electroretinogram recordings showed that Alda-1 administration alleviated the decrease in amplitude caused by MNU, rendering protection of retinal function. Mitigation of photoreceptor degeneration in MNU-treated retinas was observed by optical coherence tomography and retinal histological examination. In addition, Western blotting results revealed that ALDH2 protein expression level was upregulatedwith increased expression of SIRT1 protein after the Alda-1 intervention. Besides, endoplasmic reticulum stress (ERS) was reduced according to the significant downregulation of GRP78 protein, while apoptosis was ameliorated as shown by the decreased expression of PARP1 protein.Conclusions: Together, our data demonstrated that ALDH2 could provide preservation of retinal function and morphology against MNU-induced RP, with the underlying mechanism at least partly related to the modulation of SIRT1, ERS and apoptosis.

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Section: Introductionmentioning

confidence: 99%

Protection of retinal function and morphology in MNU-induced retinitis pigmentosa rats by ALDH2: an in-vivo study

Yan

Long²,

Wei

et al. 2020

BMC Ophthalmol

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“…16,29 On the other hand, the threshold for eliciting phosphenes by TES is elevated significantly in eyes with RP. 10,11,18 Several studies have shown that 5 to 10 times stronger currents were needed to evoke a phosphene by TES in RP patients than in the controls. 10,18 The reason for this has not been definitively determined but a degeneration of the RGCs has been suspected 18 because optic atrophy is one of features of the fundus of RP patients.…”

Section: Discussionmentioning

confidence: 99%

“…10,11,18 Several studies have shown that 5 to 10 times stronger currents were needed to evoke a phosphene by TES in RP patients than in the controls. 10,18 The reason for this has not been definitively determined but a degeneration of the RGCs has been suspected 18 because optic atrophy is one of features of the fundus of RP patients. In addition, some histologic studies have confirmed a reduction in the number of RGCs in the retina and axons in the optic nerve of eyes of RP patients.…”

Section: Discussionmentioning

confidence: 99%

“…In eyes with RP, there is a marked reduction of the amplitudes of the EEPs or significant elevations of the thresholds for eliciting EEPs or phosphenes by TES. 10,11,13,18 To date, these changes were suspected to be related to the degeneration of the RGCs due to retinal remodeling 8,9 following the photoreceptor degeneration. However, the exact mechanisms causing these alterations have not been determined.…”

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confidence: 99%

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Electrically Evoked Potentials Are Reduced Compared to Axon Numbers in Rhodopsin P347L Transgenic Rabbits With Severe Photoreceptor Degeneration

Kominami

Ueno

Nishida

et al. 2019

Invest. Ophthalmol. Vis. Sci.

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PURPOSE. To determine the relationship between the amplitudes of the electrically evoked potentials (EEPs) and the number of optic nerve axons at a late stage of retinal degeneration in rhodopsin P347L transgenic (Tg) rabbits, a model of retinitis pigmentosa. METHODS. Six eyes of six wild-type (WT) (43.8 6 7.5 months of age) and six eyes of six Tg (40.3 6 2.6 months of age) rabbits were studied. The EEPs were elicited by 1 to 5 mA of transcorneal electrical stimulation. The first positive wave, the P1 component, was analyzed. After euthanasia, the number of axons in the optic nerve was counted. RESULTS. The threshold current to elicit a P1 was significantly higher in Tg rabbits than WT rabbits. The amplitude of P1 elicited by 5 mA in Tg rabbits was about 24% of that in WT rabbits (P < 0.01). The number of axons in the optic nerve of Tg rabbits was reduced to about 59% of that of WT rabbits (P < 0.01). The correlation between the axon number and the amplitude of the P1 in Tg and WT rabbits was not significant. The mean ratio of the P1 amplitude/axon in Tg rabbits was decreased to 53% of that in WT rabbits (P < 0.05). CONCLUSIONS. The degree of reduction in the EEP in Tg rabbits is more severe than the reduction in the number of optic nerve axons. The use of transcorneal electrical stimulation to determine the suitable candidates for prosthesis at the end-stage of retinitis pigmentosa may underestimate the condition of the optic nerves.

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“…14 Several clinical studies have successfully used chromatic pupillometry to assess the function of rods, cones, and ganglion cells in various diseases including RP. [15][16][17][18][19][20] However, because full-field light stimuli were used, with a stimulus size that was equal to or larger than 90°, the pupillometry measurements reflect the massed response from the whole retina and cannot be used to assess focal changes in photoreceptor function.…”

Section: Introductionmentioning

confidence: 99%

Chromatic Pupilloperimetry Measures Correlate With Visual Acuity and Visual Field Defects in Retinitis Pigmentosa Patients

Sher

Tucker

Gurevich

et al. 2020

Trans. Vis. Sci. Tech.

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To evaluate the ability of chromatic pupilloperimetry to identify visual field (VF) defects in patients with retinitis pigmentosa (RP) and to test the correlation between pupilloperimetry impairment and retinal structural and functional measures. Methods: The pupil responses of 10 patients with RP (mean age, 41.3 ± 16.2 years) and 32 healthy age-similar controls (mean age, 50.7 ± 15.5 years) for 54 focal blue and red stimuli presented in a 24-2 VF were recorded. The pupilloperimetry measures were correlated with Humphrey VF mean deviation, best-corrected visual acuity, and ellipsoid zone area. Results: Substantially lower percentage of pupil contraction and maximal pupil contraction velocity (MCV) were recorded in patients with RP throughout the VF in response to blue and red stimuli. The mean absolute deviation (MADEV) in the latency of MCV (LMCV) was significantly larger in patients compared with controls for blue and red stimuli (P = 1.0 × 10 −7 and P = 1.0 × 10 −6 , respectively). The LMCV MADEV differentiated between patients and controls with high specificity and sensitivity (area under the receiver operating characteristic curve, 0.987 and 0.973 for blue and red, respectively). The MADEV of LMCV for blue stimuli correlated with best-corrected visual acuity (ρ = 0.938, P = 5.9 × 10 −5) and ellipsoid zone area (ρ = −0.857; P = 0.002). The MADEV of LMCV for red stimuli correlated with Humphrey VF mean deviation (ρ = −0.709; P = 0.022). Minimizing the test to 15 targets maintained a diagnosis of retinal damage in patients with RP with high sensitivity and specificity (area under the receiver operating characteristic curve, 0.927). Conclusions: The chromatic pupilloperimetry measures significantly correlated with retinal function and structure in patients with RP at various disease stages. Translational Relevance: Chromatic pupilloperimetry may enable objective assessment of visual field defects and visual acuity in RP.

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Analysis of retinal function using chromatic pupillography in retinitis pigmentosa and the relationship to electrically evoked phosphene thresholds

Cited by 26 publications

References 29 publications

Protection of retinal function and morphology in MNU-induced retinitis pigmentosa rats by ALDH2: an in-vivo study

Protection of retinal function and morphology in MNU-induced retinitis pigmentosa rats by ALDH2: an in-vivo study

Electrically Evoked Potentials Are Reduced Compared to Axon Numbers in Rhodopsin P347L Transgenic Rabbits With Severe Photoreceptor Degeneration

Chromatic Pupilloperimetry Measures Correlate With Visual Acuity and Visual Field Defects in Retinitis Pigmentosa Patients

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