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200 papers
A practical introduction to wavelet analysis in electroretinography.
Shwetar YJ, Lalush DS, Zhang AY … +3 more , McAnany JJ, Jeffrey BG, Haendel MA
Doc Ophthalmol · 2026 Feb · PMID 41398515 · Full text
PURPOSE: To provide a conceptual understanding of the continuous and discrete wavelet transforms (CWT, DWT) for clinical electroretinography (ERG) analysis, and how these methods uncover time-frequency features that comp...
PURPOSE: To provide a conceptual understanding of the continuous and discrete wavelet transforms (CWT, DWT) for clinical electroretinography (ERG) analysis, and how these methods uncover time-frequency features that complement traditional time-domain analysis. METHODS: A technical overview without the use of mathematical formula describing the basics of CWT and DWT and implementation considerations. We also review an example of four standard ISCEV full-field ERG (ffERG) recordings from a healthy 32-year-old male. RESULTS: Wavelet analysis uncovered time-frequency signatures absent in raw traces. In DA 0.01 cd s/m DWT scalogram, energy localized in the 2-5 Hz range, with CWT scalograms corroborating these findings. In DA 3.0 cd s/m, a broader frequency response is seen across 10, 20 and 40 Hz center frequencies. A similar progression was found in the LA 3.0 cd s/m, with additional low energy indices at 80 and 160 Hz. For the LA 30 Hz flicker, all frequency and time-frequency profiles effectively replicated the 30 Hz response of the cone system. CONCLUSIONS: CWT and DWT provide complementary and objective insight into ERG responses. Open-source MATLAB toolkit and step-by-step tutorial provided herein lower technical barriers and enable use by the broader community.
The reproducibility of handheld ERGs recorded with skin electrode and natural pupils.
Sommer C, Turgut F, Saad A … +7 more , Becker MD, Al-Sheikh M, Davis CQ, Rahn U, Barboni MTS, DeBuc DC, Somfai GM
Doc Ophthalmol · 2025 Nov · PMID 41288885 · Publisher ↗
PURPOSE: To investigate the reproducibility of hand-held full-field electroretinogram (ERG) to determine the minimum change required in longitudinal measurements to reach statistical significance. METHODS: The study incl...
PURPOSE: To investigate the reproducibility of hand-held full-field electroretinogram (ERG) to determine the minimum change required in longitudinal measurements to reach statistical significance. METHODS: The study included 27 healthy volunteers, aged 45-65. Light-adapted (ISCEV standard) full-field ERGs measured with the RETeval device (LKC Technologies, Germantown, MD, USA) were recorded using non-invasive skin electrodes, followed by a second examination 1-14 days later. Intersession variability of the a- and b-waves, the flicker responses (light intensity 85 Td·s, frequency 28.3 Hz) and the photopic negative response (PhNR, 38 Td·s on 380 Td blue) were assessed. RESULTS: The mean standard deviations were 0.86, 0.83, 0.40 and 3.24 ms for a-wave, b-wave, flicker and PhNR peak times, respectively. Coefficient of variations (CV) were 32%, 22%, 18%, and 19.2% for the amplitudes of the a-wave, b-wave, flicker and PhNR, respectively. CONCLUSION: While flicker ERGs had the smallest variability, ISCEV standard a-wave and b-wave times also had variability less than 1 ms, indicating excellent reproducibility. Amplitudes were more variable, with the a-wave amplitude having the most variability. While it depends on disease, longitudinal studies utilizing ERG timing are expected to be more likely to show statistically significant results due to low inter-session variability.
Short-term variability of the multifocal ERG in clinical settings.
Kahlon S, Ebedes D, Tzekov R
Doc Ophthalmol · 2026 Feb · PMID 41252054 · Publisher ↗
PURPOSE: The purpose of this study was to evaluate the effect of repeat testing on N1 and P1 amplitudes, signal-to-noise (SNR) ratios, and amplitude ring ratios (RR) in multifocal electroretinography (mfERG). METHODS: Th...
PURPOSE: The purpose of this study was to evaluate the effect of repeat testing on N1 and P1 amplitudes, signal-to-noise (SNR) ratios, and amplitude ring ratios (RR) in multifocal electroretinography (mfERG). METHODS: This was a retrospective review of mfERG records from 08/2022 to 05/2023. Patients were tested binocularly with the Espion system (Diagnosys LLC). Only records from patients with repeat mfERG tests at the same appointment were included. N1 and P1 amplitudes, signal-to-noise ratio (SNR), and amplitude ring ratios were evaluated for the first recording (run #1), the second recording (run #2), and the combination of run #1 and run #2 (combined run). RESULTS: Data was collected for 93 eyes from 47 patients (5 males, 42 females) with a mean patient age of 56.1 ± 17.3 years. No change was observed between run #1 and run #2 for N1 or P1 amplitudes, however amplitudes of the combined run decreased significantly (p < 0.05) compared to run #1 amplitudes for all rings, right and left eyes (except for ring 1 in right eyes). SNR increased significantly from run #1 to run #2 for rings 2-5 (~10%), but not for ring 1. The number of blinks recorded during testing decreased from run #1 to run #2 (p < 0.001). Amplitude ring ratios R5/R4 and R5/R3 did not change significantly from run #1 to run #2, while amplitude R1/R2 ratio decreased significantly (p < 0.05). CONCLUSION: The change in signal quality from run #1 to run #2 suggests a superior quality signal in the second run. Furthermore, the decreased P1 amplitude in the combined run compared to run #1 should be considered when a clinician uses the combined run for their final report.
A rapid pupillometry protocol for clinical use: effect of age and test-retest repeatability.
Park JC, McAnany JJ
Doc Ophthalmol · 2025 Nov · PMID 41240211 · Full text
PURPOSE: Pupillometry is most commonly performed in laboratory settings using specialized, non-portable instruments that require lengthy test protocols. The purpose of this study was to develop and evaluate a rapid, clin...
PURPOSE: Pupillometry is most commonly performed in laboratory settings using specialized, non-portable instruments that require lengthy test protocols. The purpose of this study was to develop and evaluate a rapid, clinically-applicable pupillometry protocol using a commercially available, portable, handheld instrument. METHODS: Thirty-seven healthy individuals (ages 21-61 years) participated in three experiments. In each experiment, the pupillary light reflex (PLR) was elicited by full-field, 500 ms chromatic flashes (470 nm and 621 nm; 12,000 Td). Experiment I evaluated the minimum dark adaptation (DA) time needed to achieve maximum PLRs. Experiment II determined the effect of age. Experiment III estimated PLR test-retest repeatability. For all experiments, baseline pupil size (BL; 1 s before flash onset), maximum pupil constriction (MPC) following the flash, and post-illumination pupillary response (PIPR; median size 6-8 s after flash offset) were quantified. RESULTS: Experiment I showed that from 1 to 3 min of DA, BL and MPC increased slightly (0.27 mm and 5%, respectively), whereas the PIPR increased considerably (17%). The responses did not change appreciably after 3 min, therefore a 3 min DA period was used for Experiments II and III. Experiment II showed a trend for BL and MPC to decrease with age, but correlations with age were not statistically significant (all p > 0.05). PIPR was independent of age (r = - 0.01; p = 0.96). Experiment III showed test-retest repeatability of approximately 1 mm for BL, and 10% for MPC and PIPR, indicating good repeatability. CONCLUSION: The proposed approach is useful for measuring the MPC and PIPR across a broad range of ages and baseline pupil sizes. Given the device portability and short test duration (approximately 5 min including DA), this approach has promising clinical utility.
Bilateral retinal dysfunction in Posner-Schlossman syndrome: subclinical abnormalities revealed by full-field electroretinography.
Ge X, Li J, Xie X … +3 more , He X, Lu Z, Feng Y
Doc Ophthalmol · 2026 Apr · PMID 41239123 · Publisher ↗
PURPOSE: The aim of this study was to investigate whether Posner-Schlossman Syndrome (PSS), is strictly unilateral, to determine the presence of subclinical abnormalities in the contralateral eye, and to analyze the rela...
PURPOSE: The aim of this study was to investigate whether Posner-Schlossman Syndrome (PSS), is strictly unilateral, to determine the presence of subclinical abnormalities in the contralateral eye, and to analyze the relationship between retinal function and the corneal sub-basal nerve in such patients. METHODS: The patients included in this study were diagnosed with PSS. 14 patients with PSS and 21 healthy controls were recruited for this study. Both eyes of the patients and one eye of the healthy controls underwent full-field electroretinography (ffERG) examination. Additionally, in vivo confocal microscopy (IVCM) was performed on the affected eyes of the patients to assess the status of their corneal sub-basal nerve. RESULTS: Compared with healthy controls, patients with PSS exhibited significantly reduced amplitudes and abnormal peak times in dark-adapted oscillatory potentials, light-adapted 3 ERG, and light-adapted 30 Hz flicker ERG in both eyes. However, no statistically significant differences were observed in dark-adapted 0.01 ERG, dark-adapted 3.0 ERG, and dark-adapted 10.0 ERG. All ffERG parameters in the affected eyes of patients showed no significant correlations with the total nerve length of corneal sub-basal nerve. CONCLUSIONS: The results demonstrate that PSS is not a strictly unilateral disease. Through ffERG, it is possible to identify cases of bilateral involvement that are difficult to detect, thereby enabling early intervention against potential disease progression.
Unilateral pigmentary retinopathy in an Asian population.
Thomas GN, Ong SC, Chan HW … +4 more , Tien M, Stanley PF, Koh AHC, Holder GE
Doc Ophthalmol · 2026 Apr · PMID 41239122 · Publisher ↗
PURPOSE: To report a series of Asian patients with unilateral pigmentary retinopathy. METHODS: Retrospective case series. Clinical features, fundus photography, fundus autofluorescence imaging (FAF), visual fields, optic...
PURPOSE: To report a series of Asian patients with unilateral pigmentary retinopathy. METHODS: Retrospective case series. Clinical features, fundus photography, fundus autofluorescence imaging (FAF), visual fields, optical coherence tomography (OCT) and electrophysiology were reviewed in 6 patients referred to two Singaporean inherited retinal disease clinics with suspected "unilateral retinitis pigmentosa". RESULTS: Four patients presented with unilateral blurring of vision; 2 were asymptomatic. All index eyes and one fellow eye had FAF abnormalities consistent with visible fundus abnormalities. Visual fields were abnormal in all index and 2 fellow eyes. Macular OCT showed disruption of outer retinal layers in all index eyes and 1 fellow eye. Five index eyes had abnormal rod and cone full field electroretinogram (ERG) amplitudes, while one index eye had localized rod ERG amplitude abnormality. Two index eyes had 30 Hz flicker peak time delay. Three index eyes displayed cone greater than rod system involvement. Pattern electroretinography (PERG) demonstrated macular dysfunction in 5 index eyes and 2 fellow eyes. One case was diagnosed with possible RP. Alternative diagnoses (trauma and autoimmune retinopathy) were ascertained in 4 cases. CONCLUSION: Electrophysiology was more sensitive than clinical examination and imaging in detecting retinopathy or maculopathy. In only 1 of 6 patients referred for possible unilateral RP was that diagnosis sustainable; four could be attributed to an identified acquired etiology. Although the diagnosis was unclear in the remaining case, the clinical findings and investigations were not compatible with an inherited disorder.
Characterization of functional and structural impairments in best vitelliform macular dystrophy using visual electrophysiology and optical coherence tomography in pediatric and adult patients.
Polosa A, Lu M, Dorfman AL … +3 more , Masis-Solano M, Costantino S, Qian CX
Doc Ophthalmol · 2026 Apr · PMID 41222609 · Publisher ↗
PURPOSE: To assess functional and anatomical impairments in pediatric patients and their affected adult family members with juvenile-onset Best vitelliform macular dystrophy (BVMD). METHODS: Fifteen genetically confirmed...
PURPOSE: To assess functional and anatomical impairments in pediatric patients and their affected adult family members with juvenile-onset Best vitelliform macular dystrophy (BVMD). METHODS: Fifteen genetically confirmed BVMD patients (10 pediatric and 5 adults) underwent a comprehensive ophthalmic examination with multimodal imaging and electrophysiological testing. We quantified BVMD lesion volumes using sequential spectral-domain OCT (SD-OCT) scans, facilitating correlation with clinical parameters and electroretinogram (ERG) findings. RESULTS: Children presented less advanced stages of BVMD than adults, had better visual acuity [Log MAR 0.13 ± 0.17] than adults [Log MAR 0.91 ± 0.52, p < 0.001], and displayed lower lesion volumes [0.93 ± 0.71 mm] compared to adults [1.80 ± 0.79 mm, p < 0.001]. Interestingly, in 3 eyes in Stage 3 disease (pseudohypopyon stage), despite a high lesion volume (1.74 ± 0.66 mm), a good visual acuity was still observed (LogMAR of 0.00 in all 3 eyes). Multifocal ERG (mfERG) revealed macular dysfunction in all patients mostly pronounced in the 3 central rings [% of P1 reduction from control in Ring 1 in children and adults: 51% and 51%, Ring 2: 17% and 36% and Ring 3: 8% and 15%, respectively]. Furthermore, higher lesion volumes showed more affected mfERG responses. Full-field flash ERGs were normal in children, while reduced amplitudes and delayed responses were observed in some adults. CONCLUSION: While juvenile presentations of BVMD are less severe and more circumscribed, adult presentations demonstrate more widespread functional abnormalities in both amplitude and implicit times correlated with anatomical progression and clinical progression. Interestingly, mfERG responses in children with 20/20 vision also revealed functional impairment, correlating more strongly with the degree of vitelliform liquefaction on OCT than with visual acuity. Our findings suggest that mfERG may serve as an early, reliable indicator of functional defects in BVMD.
Comparison of visual evoked potential variability in eyes affected by optic neuritis and fellow eyes.
Chutná M, Kremláček J, Kuba M … +4 more , Kubová Z, Szanyi J, Vít F, Langrová J
Doc Ophthalmol · 2026 Feb · PMID 41214379 · Full text
PURPOSE: This study compared the variability of visual evoked potential (VEP) in response to stimulation of eyes affected by unilateral optic neuritis with that of fellow (non-affected) eyes. METHODS: Pattern-reversal VE...
PURPOSE: This study compared the variability of visual evoked potential (VEP) in response to stimulation of eyes affected by unilateral optic neuritis with that of fellow (non-affected) eyes. METHODS: Pattern-reversal VEP (PVEP) and motion-onset VEP (MVEP) recordings from thirty-six subjects with unilateral optic neuritis at different intervals from disease onset were retrospectively evaluated, and differences in the following parameters were compared: signal‒to‒noise ratio (SRN), interquartile range of the response jitter (jitter IQR), and number of trials corresponding to the average response (corresponding N). RESULTS: In the PVEP recordings, the P1 peak times of the fellow eyes were significantly shorter than those of the affected eyes (Cohen's d = -1.470, p < 0.001). P1 amplitudes were significantly greater in fellow eyes (d = 1.17, p < 0.001). Significant differences were found in the SNR (d = 0.782, p < 0.001), jitter IQR (d = -0.874, p < 0.001), and corresponding N (d = 0.700, p < 0.001). MVEP presented significantly shorter N2 peak times in fellow eyes than in affected eyes (d = 0.840, p < 0.01) and significantly greater amplitudes (d = 0.494, p = 0.002). There was a significant difference in the SNRs (d = 0.440, p = 0.01) and corresponding N values (d = 0.415, p = 0.01). There was no difference in the jitter IQR (d = 0.143, p = 0.230). CONCLUSIONS: The increased variability in eyes affected by optic neuritis compared with fellow eyes (in particular, in pattern-reversal VEP, which predominantly represents the activity of the macular-papillary fibers of the optic nerves) may represent important pathophysiologic features and may add valuable information to diagnostics via VEP examinations.
Incomplete congenital stationary night blindness associated with a novel variant in the CACNA1F gene.
Loo SP, Shipton C, Hamilton M … +5 more , Brown A, Millar E, Malik I, Craig M, Hamilton R
Doc Ophthalmol · 2026 Jun · PMID 41201761 · Publisher ↗
PURPOSE: Incomplete congenital stationary night blindness (icCSNB) is a subtype of inherited, non-progressive retinal diseases. Most cases of icCSNB result from mutations in the X-linked gene CACNA1F. We describe the cli...
PURPOSE: Incomplete congenital stationary night blindness (icCSNB) is a subtype of inherited, non-progressive retinal diseases. Most cases of icCSNB result from mutations in the X-linked gene CACNA1F. We describe the clinical findings of two male siblings diagnosed with icCSNB, both carrying a novel variant c.4008 + 5G > T in CACNA1F inherited from their mother. METHODS: We carried out a comprehensive ophthalmic assessment, including fundus imaging, optical coherence tomography (OCT) scanning and electroretinography. We performed genetic testing with next generation sequencing, in-silico and functional analyses to further characterise the novel variant. RESULTS: Two male siblings presented with high myopia and reduced visual acuities at age three. Examination and OCT demonstrated no significant abnormalities in both siblings. Full-field electroretinogram (ffERG) testing demonstrated markedly reduced amplitude to weak flashes and an electronegative waveform to strong flashes in dark-adapted ERGs, resembling that of icCSNB, leading to its diagnosis in both children. Next generation sequencing in the older sibling identified a novel hemizygous c.4008 + 5G > T variant in CACNA1F. In-silico analysis of this variant predicted that it would disrupt normal splicing of CACNA1F, though it was not possible to confirm this by RNA sequencing. This same variant was found in the younger sibling, as well as in their mother who had normal examination and ffERG findings. CONCLUSIONS: We report a novel CACNA1F variant not previously identified in the literature in three patients. Although functional analyses were unable to confirm pathogenicity of this variant, in-silico tools predicted that its effect is consistent with the pathogenesis of icCSNB. Reporting of this family further widens the genotypic spectrum of icCSNB.
Impact of pulsed stimulation on objective and subjective visual acuity measurements in nystagmus.
Quanz EV, Al-Nosairy KO, Stolle FH … +4 more , Kuske J, Heinrich SP, Bach M, Hoffmann MB
Doc Ophthalmol · 2026 Feb · PMID 41186874 · Full text
PURPOSE: Quanz et al. (Sci Rep 14:16797, 2024) reported that participants with nystagmus had higher objective visual evoked potential visual acuity estimates (VA) by 0.12 logMAR relative compared to standard psychophysic...
PURPOSE: Quanz et al. (Sci Rep 14:16797, 2024) reported that participants with nystagmus had higher objective visual evoked potential visual acuity estimates (VA) by 0.12 logMAR relative compared to standard psychophysical VA (VA). The cause of this modest, but significant VA overestimation remains unclear. Here we investigated its association with the pattern-pulse stimulation mode applied for steady state VEP recording for VA estimation. Specifically, we tested whether psychophysical visual acuity to pulsed optotypes (VA) also exceeds standard optotype VA. METHODS: Twelve participants with nystagmus were included in this analysis. VA was determined for pattern-pulse steady-state VEP stimulation (Quanz et al. in Sci Rep 14:16797, 2024) using EP2000, psychophysical VA was determined to stationary (VA) and to pulsed (VA) Landolt-C optotypes employing a modified version of the Freiburg Vision Test (FrACT). Pulsed stimulus timing was identical for VEP and VA (40 ms on and 93 ms off, i.e. at 7.5 Hz). In a separate measurement, fixation stability within the central 4° was determined using microperimetry (Nidek MP-1), and the eye with the stronger fixation instability was selected for the analysis (12 eyes). LogMAR differences were assessed with a paired t-test and the correlation of fixation stability and VA differences (ΔVA = VA - VA) was tested. RESULTS: VA (0.43 ± 0.06 logMAR) and VA (0.45 ± 0.06 logMAR, P = 0.15) did not differ from each other, but from VA (0.26 ± 0.08 logMAR, P = 0.02 and P = 0.01, respectively). There was no correlation of ΔVA with fixation instability (r = 0.002, P = 0.89). CONCLUSION: Pulsed stimulation appears not to be the reason for the VA overestimation in nystagmus. Further research should address whether differences in the spatial stimulus properties might be of relevance, as VA is tested with optotypes, VA with extended patterns.
Molecular analysis of foveoschisis in females reveals a novel case of segmental uniparental disomy in X-linked retinoschisis.
Elbagoury NM, Essawi ML, Fathy HM … +4 more , Eid OM, Nabih M, Mohamed AM, Tawfik CA
Doc Ophthalmol · 2026 Apr · PMID 41123868 · Full text
BACKGROUND: Foveoschisis refers to the splitting of retinal layers involving the macula that may have different causes with variable structural-functional natural histories. Idiopathic cases are seen in the absence of in...
BACKGROUND: Foveoschisis refers to the splitting of retinal layers involving the macula that may have different causes with variable structural-functional natural histories. Idiopathic cases are seen in the absence of inherited or acquired predisposing conditions and referred to as stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR). Our study aimed to clinically and genetically characterize females presenting with foveoschisis (including affected male siblings where present). METHODS: Five patients (3 females and 2 males) from 3 consanguineous families presenting with foveoschisis underwent complete ophthalmological evaluation, multimodal imaging including color, infrared, fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT), electroretinogram (ERG), and molecular evaluation including Sanger sequencing of the RS1 gene and whole exome sequencing (WES). Main outcome measures were age at first visit, best-corrected visual acuity (BCVA), peripheral retinal changes, FAF pattern, ERG findings, and RS1 variants. RESULTS: The mean age was 21.8 years. The BCVA ranged from 20/100 to 20/20. Peripheral retinal changes ranged from a tapetal reflex, peripheral retinoschisis, vitreous veils, to vitreoretinal traction. A ring of increased signal was the most common FAF abnormality, while one patient exhibited a double-ring hyperautofluorescence. All patients demonstrated an electronegative ERG. One female was considered to have a molecularly undiagnosed inherited retinal disease (IRD). Another female was considered stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) after exclusion of other causes. Three patients showed a novel nonsense variant in the RS1 gene; homozygous in the female sibling and hemizygous in the male siblings. Familial segregation revealed an unaffected father and a carrier mother. Trio SNP array confirmed maternal segmental uniparental isodisomy (seg UPiD). CONCLUSION: This is the first reported X-linked retinoschisis (XLRS) case with seg UPiD. We emphasize the significance of SNP arrays in elucidating non-Mendelian inheritance cases. We report a novel variant, which is the first to be detected in the RS1 domain in a female.
Optimizing cyclopean stimuli for the evaluation of stereo vision by steady-state visual evoked potentials.
Radó J, Mikó-Baráth E, Hegyi P … +3 more , Nemes VA, Jandó G, Buzás P
Doc Ophthalmol · 2026 Feb · PMID 41105358 · Full text
PURPOSE: This study aimed to optimize dynamic random dot correlogram (DRDC) and stereogram (DRDS) stimuli to evoke steady-state visual evoked potentials (ssVEP) on multiple EEG channels for the objective assessment of st...
PURPOSE: This study aimed to optimize dynamic random dot correlogram (DRDC) and stereogram (DRDS) stimuli to evoke steady-state visual evoked potentials (ssVEP) on multiple EEG channels for the objective assessment of stereopsis. METHODS: EEG recordings were conducted on 22 healthy adults (mean age: 30.2 ± 5.8 years) while viewing cyclopean and control stimuli. DRDC and DRDS were presented at three temporal frequencies (0.9375, 1.875, and 3.75 cycles per second, cps) using anaglyphic channel separation. The ssVEP responses were analyzed using T statistical test to determine the most effective stimulus for eliciting significant cortical activity. RESULTS: DRDC at 1.875 cps evoked significant ssVEP responses in 93% of participants on at least one occipital electrode (O1, Oz, O2) and in 100% when including parietal-occipital electrodes. DRDS at similar frequencies also produced robust responses but required additional parietal electrode monitoring. Monocular control measurements confirmed that responses were stereo-specific. CONCLUSIONS: DRDC at 1.875 cps was the most effective stimulus for objective electrophysiological assessment of stereopsis, demonstrating high reliability with minimal electrode setups. These findings support the integration of optimized ssVEP protocols into clinical assessments, particularly for non-verbal or pediatric populations.
Chromatic pupil campimetry as objective diagnostic tool for progressive optic neuropathies.
Edelmayer MV, Strasser T, Jung R … +7 more , Sonntag A, Jendritza R, Tonagel F, Peters T, Wilhelm H, Wilhelm B, Kelbsch C
Doc Ophthalmol · 2025 Oct · PMID 41094347 · Publisher ↗
PURPOSE: This study assessed the diagnostic potential of chromatic pupil campimetry (CPC) using relative maximal constriction amplitude (relMCA), pupillary light response (PLR) latency, and pupillary escape to differenti...
PURPOSE: This study assessed the diagnostic potential of chromatic pupil campimetry (CPC) using relative maximal constriction amplitude (relMCA), pupillary light response (PLR) latency, and pupillary escape to differentiate optic neuropathies (ON) from healthy individuals and identify specific ON subtypes. METHODS: CPC testing used red and blue stimuli at central (0°) and peripheral (20°) locations to measure relMCA, latency, and pupillary escape. Patients with various ON etiologies, including glaucoma (n = 20), optic nerve compression by meningioma (n = 18), chiasm compression (n = 4), Leber hereditary optic neuropathy (LHON; n = 4), and autosomal dominant optic atrophy (ADOA; n = 3), were tested. Linear mixed-effects models and post hoc Tukey tests were used to analyze differences across subgroups of ON etiologies and a healthy control group (n = 40), regarding signal eccentricities and locations. RESULTS: Pupillary escape was significantly higher in ON patients during central red stimulation (p = 0.0007). Glaucoma and meningioma groups showed reduced relMCA and prolonged latency for both stimuli compared to controls (p < 0.0001 to p = 0.0058). RelMCA during blue stimulation was lower in glaucoma patients than in ADOA (p = 0.0183). LHON patients exhibited significantly prolonged PLR latency during blue stimulation compared to healthy (p = 0.0284). CONCLUSION: CPC effectively distinguished glaucoma and meningioma from healthy controls but was less reliable for differentiating ON subtypes. Our results indicate, that central pupillary escape is associated with inner retinal dysfunction.
Dioptric blur and VEP-based visual acuity - clinical implications in resource-limited settings: correspondence.
Matovu D
Doc Ophthalmol · 2025 Oct · PMID 41091361 · Publisher ↗
Abstract loading — click title to view on PubMed.
Best vitelliform macular dystrophy caused by a BEST1 p.(Ser246Asn) variant coexisting with diabetic retinopathy.
Tatemoto Y, Hayashi T, Mizobuchi K … +2 more , Den S, Nakano T
Doc Ophthalmol · 2026 Jun · PMID 41051727 · Publisher ↗
PURPOSE: To report a 42 year-old male patient with Best vitelliform macular dystrophy (BVMD) complicated by diabetic retinopathy, who harbored a missense variant in the BEST1 gene. METHODS: Comprehensive ophthalmological...
PURPOSE: To report a 42 year-old male patient with Best vitelliform macular dystrophy (BVMD) complicated by diabetic retinopathy, who harbored a missense variant in the BEST1 gene. METHODS: Comprehensive ophthalmological examinations, including full-field electroretinography (ERG) and electrooculography (EOG), were performed. Whole exome sequencing (WES) was conducted to identify potential disease-causing variant(s), and Sanger sequencing was used for confirmation. RESULTS: Fundus photography and fluorescein angiography revealed macular degeneration and non-proliferative diabetic retinopathy with macular leakage. Although the light peak/dark trough (Arden) ratio on EOG was relatively preserved, a reduced light rise and attenuated dark trough amplitudes were observed. ERG demonstrated normal rod and cone system function. Based on optical coherence tomography findings, the BVMD stage was classified as a stage between the Vitelliruptive and Atrophic stages in the right eye, and as the Pseudohypopyon stage in the left eye. WES identified a previously unreported BEST1 variant, c.737G > A: p.(Ser246Asn) heterozygously, confirmed by Sanger sequencing. CONCLUSIONS: This case emphasizes the importance of EOG assessment and genetic analysis in establishing an accurate diagnosis of BVMD, particularly in patients with coexisting conditions such as diabetic retinopathy.
Optimizing latency calculation for robust evaluation of the pupillary light response in chromatic pupillography.
Jendritza R, Jung R, Strasser T … +6 more , Sonntag A, Edelmayer M, Peters T, Wilhelm B, Wilhelm H, Kelbsch C
Doc Ophthalmol · 2025 Oct · PMID 41045437 · Publisher ↗
PURPOSE: To optimize latency calculation in chromatic pupillography for a more robust evaluation of pupillary light response (PLR) dynamics in a normative collective. METHODS: The PLR of 150 healthy participants aged 18-...
PURPOSE: To optimize latency calculation in chromatic pupillography for a more robust evaluation of pupillary light response (PLR) dynamics in a normative collective. METHODS: The PLR of 150 healthy participants aged 18-79 years (median 46 years, 94 females) measured by L-cone- and rod-favoring stimulation protocols in Chromatic Pupil Campimetry (CPC) was analyzed. Three calculation methods of latency to constriction onset after light stimulus were tested. 1: intersection of mean baseline pupil diameter and linear fit through the descending part of the pupillogram (20 data points) at each stimulus position in the central visual field (30°), 2: intersection of a linear fit through the baseline and linear fit using less (15) data points through the pupillary contraction phase at each stimulus position and 3: mean per eccentricity gained by averaged pupillograms. Equivalence testing (two one-sided t-tests, TOST) was used for comparison of the methods. RESULTS: The longest mean latencies were found with calculation 1 in both photopic and scotopic stimulation, followed by calculation 2. Latency calculation per eccentricity (3) resulted in the shortest mean latencies. The differences in latency results of the three calculation methods increased with increasing eccentricity in both stimulation protocols. Calculation 2 and 3 were equivalent up to 12° eccentricity in photopic and up to 20° eccentricity in scotopic stimulation. CONCLUSIONS: The use of the intersection of a linear fit through the baseline with a linear fit containing an adjusted number of data points adapted to the characteristics of the pupillary contraction phase appears to be suitable to provide consistent latency calculation, particularly for small constriction amplitudes and noisy data as they may occur in patients with e.g. hereditary retinal degenerations. The evaluation of mean latency per eccentricity is equivalent and may be advantageous in difficult clinical test results with low amplitudes.
A unilateral presentation of pigmented paravenous chorioretinal atrophy: a case report.
Seraj H, Alharazi SK, Magharbil ES … +3 more , Albalawi HB, Alali NM, Magliyah MS
Doc Ophthalmol · 2025 Dec · PMID 41023504 · Publisher ↗
PURPOSE: This case report aims to describe an atypical presentation of pigmented paravenous chorioretinopathy (PPCRA). METHODS: Detailed clinical ophthalmologic examinations, multimodal imaging and electroretinography of...
PURPOSE: This case report aims to describe an atypical presentation of pigmented paravenous chorioretinopathy (PPCRA). METHODS: Detailed clinical ophthalmologic examinations, multimodal imaging and electroretinography of a 33-year-old woman who presented with unilateral PPCRA. RESULTS: A 33-year-old female who is known to have hypothyroidism and had previous bariatric surgery, referred for retinal evaluation following incidental findings during a refractive surgery consultation. Fundus examination revealed unilateral segmental perivascular hyperpigmentation, vascular sclerosis, and areas of chorioretinal atrophy, raising the differential diagnosis of pigmented paravenous chorioretinopathy (PPCRA) versus resolved retinal vasculitis. The patient reported no significant ocular symptoms apart from decreased night vision in one eye and denied a history of acute visual loss or photophobia. Systemic workup, including autoimmune and infectious serologies, imaging, and a detailed clinical history, was unremarkable. The patient reported consanguinity within the family. CONCLUSION: This report underscores the challenge of distinguishing PPCRA, a rare, typically bilateral hereditary condition, from resolved vasculitis, which often presents unilaterally with a history of systemic inflammation. Fluorescein angiography and optical coherence tomography were instrumental in identifying the lack of active inflammation and vascular leakage, favoring the diagnosis of PPCRA.
Effects of flickering light stimulation on retinal blood flow and full-field electroretinogram in mice.
Rai M, Lakshmanan Y, Choi KY … +1 more , Chan HH
Doc Ophthalmol · 2025 Dec · PMID 40996493 · Full text
PURPOSE: To investigate the effects of brief flickering light stimulation (FLS) on retinal electrophysiology and retinal blood flow (RBF) in normal C57BL6J mice. METHODS: RBF and full-field electroretinography (ffERG) we...
PURPOSE: To investigate the effects of brief flickering light stimulation (FLS) on retinal electrophysiology and retinal blood flow (RBF) in normal C57BL6J mice. METHODS: RBF and full-field electroretinography (ffERG) were measured before and after a 60 second FLS (12 Hz, 0.1 cd·s/m) in a cohort of 8-12-weeks old C57BL6J mice (n=10) under anaesthetic and light-adapted conditions. A separate set of age-matched mice (n=9) underwent RBF and ffERG measurements before and after steady light stimulation (SLS) at 1 cd/m under similar conditions. The changes in RBF (arterial and venous flow) as well as the amplitudes and implicit times of the a-wave, b-wave, oscillatory potentials (OPs), and photopic negative response (PhNR) were analyzed. RESULTS: FLS significantly increased both arterial (p=0.003) and venous (p=0.018) blood flow as well as b-wave amplitudes (p=0.017) compared to SLS, which did not have any significant changes in either RBF or ERG. However, no significant differences were found in other ffERG responses (amplitudes and implicit times of a-wave, OPs, and PhNR, as well as b-wave implicit time) between the two groups after light stimulation. An increase in b-wave amplitude was positively associated with an increase in both arterial (r=0.655, p=0.040) and venous blood flow (r=0.638, p=0.047) in the FLS group. CONCLUSIONS: Our results suggest that transient FLS not only increases RBF but also enhances electro-retinal responses of the middle retinal layer, as shown by ffERG, thus demonstrating its substantial effects on both the vascular and neuronal components of retinal neurovascular coupling in mice.
An in-depth characterization of development-related electroretinographical and morphological changes in Landrace pigs.
Skrzypczyk L, Ackermann BC, Augustin VA … +4 more , Rahn U, Uhl P, Auffarth GU, Hammer M
Doc Ophthalmol · 2025 Dec · PMID 40993473 · Full text
PURPOSE: Landrace pigs are increasingly used as a large-animal model in ophthalmic research due to their cone-enriched visual streak and anatomical similarity to the human eye. However, they are commonly studied at 16-20...
PURPOSE: Landrace pigs are increasingly used as a large-animal model in ophthalmic research due to their cone-enriched visual streak and anatomical similarity to the human eye. However, they are commonly studied at 16-20 weeks of age, a timeframe in which the animals double their weight and development-related physiological changes may occur. This study aims to characterize retinal function and morphology and establish reference values for future translational studies. METHODS: Landrace pigs (16-20 weeks old) underwent standardized examinations of the left eye at baseline (16 weeks), 18 and 20 weeks. The left eye was examined by optical coherence tomography (OCT), fundus photography, histology, and full-field electroretinography (ffERG) under light- and dark-adapted conditions, using the ISCEV-compliant 6-step Dog, Cat, Nonhuman Primate protocol. RESULTS: A total of 30 animals were included. Retinal morphology remained stable throughout the study period, with no significant changes in retinal thickness observed by OCT (baseline: 252 ± 24 µm; week 20: 249 ± 11 µm; p = 0.17) or by histology. ffERG revealed increased amplitudes under light- and dark-adapted conditions at 20 weeks compared to baseline at 16 weeks of age (e.g. light-adapted b-wave: + 65 µV, + 18.4%, p < 0.01), while latencies remained stable without clinically relevant changes. CONCLUSIONS: During this phase of rapid development, Landrace pigs undergo significant functional retinal maturation without corresponding morphological changes emphasizing importance of functional testing in retinal assessments. This study provides reference data in a large number of animals.
Comparative analysis of pupillometry in two scheimpflug-based measurement systems.
Atalay K, Kocak I, Sayin N … +2 more , Sanliturk MA, Zirtiloglu S
Doc Ophthalmol · 2025 Sep · PMID 40944850 · Publisher ↗
OBJECTIVE: The primary aim of this study was to compare pupillometry measurements obtained from the Sirius™ and AL-Scan™ devices under different lighting conditions. Additionally, the secondary aim was to assess and comp...
OBJECTIVE: The primary aim of this study was to compare pupillometry measurements obtained from the Sirius™ and AL-Scan™ devices under different lighting conditions. Additionally, the secondary aim was to assess and compare the topographic (keratometric) measurements provided by these systems. MATERIALS AND METHODS: A non-interventional and prospective study was conducted on normal patients aged 18-45 years. Patients who did not smoke and had a spherical equivalent ranging from + 3.00 D to - 5.0 were included. Pupillometry measurements were taken after 1 minute of dark adaptation. Only the right eyes were compared for keratometry. All eyes were compared for pupillometry. We compared the K1 (D), K2 (D), KAvg (D), photopic pupillometry (PP), and mesopic pupillometry (MP) values. RESULTS: We examined a total of 30 women (66.7%) and 15 men (33.3%). AL Scan showed an average K1 of 42.99 ± 1.50 D, an average K2 of 44.04 ± 1.74, and an average Kavg of 43.58 ± 1.62. Sirius topography yielded the following averages: 42.94 ± 1.51 for K1, 43.90 ± 1.75 for K2, and 43.41 ± 1.61 for Kavg. The average PP and MP measures obtained with the Nidek Al-Scan instrument were 3.97 ± 1.08 mm and 6.04 ± 1.08 mm, respectively. The Sirus device yielded average PP and MP values of 5.05 ± 1.04 mm and 6.01 ± 1.08 mm, respectively. Bland-Altman statistics showed a good degree of correlation between the MP measurements of both devices (p = 0.65). However, K1, K2, KAvg, and PP data showed no significant agreement between both devices (p < 0.05). CONCLUSION: In our research, Sirius and Nidek Al-Scan equipment yield comparable MP data; however, PP is inconsistent. Device selection with repeated pupillometry data requires more investigation.
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