Assessment of retinal atrophy in mixed breed dogs using spectral domain optical coherence tomography (SD-OCT) and electroretinography.

The aim of the study was to characterize retinal atrophy (RA) with progressive retinal atrophy symptoms in mixed breed dogs using ophthalmoscopy, spectral domain optical coherence tomography (SD-OCT) and electroretinography (ERG).The study was performed on 13 mixed breed dogs affected by retinal atrophy (11 males and 2 females that were 1.5-14 years old). Depending on the advancement of RA, SD-OCT examinations identified retinal abnormalities ranging from layer disorganisation to advanced atrophy. The most advanced RA occurred ventral to the optic disc. Total retinal thickness in both eyes (mean ± SD) was lower in dogs with RA compared to controls dorsally (77.7 ± 39.5 µm vs 173.5 ± 13.3 µm), ventrally (33.4 ± 29.9 µm vs 139.5 ± 10.8 µm), nasally (65.0 ± 34.5 µm vs 163.9 ± 11.0 µm) and temporally (61.8 ± 41.7 µm vs 171.9 ± 11.1 µm) to the optic disc. In dogs with locally normal architecture of inner retina, loss of definition of outer retinal layers occurred in many regions. Dark and light-adapted ERGs were reduced in 2 dogs with RA and were unrecordable in 11 dogs. Lesions evident in SD-OCT scans of mixed breed dogs affected with retinal atrophy initially appear ventrally to the optic disc and ventro-dorsally in advanced RA. In all mixed breed dogs with retinal atrophy, clinical signs and SD-OCT results correlate with ERG findings.

The Use of the RETeval Portable Electroretinography Device for Low-Cost Screening: A Mini-Review.

Electroretinography (ERG) provides crucial insights into retinal function and the integrity of the visual pathways. However, ERG assessments classically require a complicated technical background with costly equipment. In addition, the placement of corneal or conjunctival electrodes is not always tolerated by the patients, which restricts the measurement for pediatric evaluations. In this short review, we give an overview of the use of the RETeval portable ERG device (LKC Technologies, Inc., Gaithersburg, MD, USA), a modern portable ERG device that can facilitate screening for diseases involving the retina and the optic nerve. We also review its potential to provide ocular biomarkers in systemic pathologies, such as Alzheimer's disease and central nervous system alterations, within the framework of oculomics.

Assessment of the relationship between structural and functional tests in patients with idiopathic intracranial hypertension.

PURPOSE: To assess the relationship between structural and functional tests in mild and moderate idiopathic intracranial hypertension (IIH). METHODS: Patients with mild and moderate IIH and a control group were enrolled. Best-corrected visual acuity (BCVA), macular ganglion cell layer (MGCL) thickness, peripapillary retinal nerve fiber layer (pp RNFL) thickness, perimetric mean deviation (MD), and photopic negative responses (PhNR) of the electroretinogram were recorded. The associations between structural (pp RNFL and MGCL thickness) and functional (PhNR amplitude, MD and BCVA) parameters were assessed. RESULTS: 154 eyes from 78 subjects (74 eyes from IIH patients and 80 eyes from healthy subjects) were included in this comparative observational study. The MGCL thickness, VA, pp RNFL, and PhNR base-to-trough (BT) amplitude were significantly worse in moderate IIH. The BCVA and MD were associated with MGCL thickness only in moderate IIH. The relationship between MD and MGCL thickness started when MD fell below -5.7 dB. CONCLUSIONS: The association between functional and structural parameters varies between mild and moderate IIH. The MD and MGCL thickness outperformed in assessing disease severity in mild and moderate IIH, respectively. The association between MD and MGCL thickness could be considered in IIH severity categorization.

Assessment of Visual Function with Cotoretigene Toliparvovec in X-Linked Retinitis Pigmentosa in the Randomized XIRIUS Phase 2/3 Study.

PURPOSE: Cotoretigene toliparvovec (BIIB112/AAV8-RPGR) is an investigational vector-based gene therapy designed to provide a full-length, codon-optimized retinitis pigmentosa GTPase regulator (RPGR) protein to individuals with RPGR-associated X-linked retinitis pigmentosa (XLRP). We assessed efficacy and tolerability of cotoretigene toliparvovec subretinal gene therapy. DESIGN: Part 2 of the XIRIUS trial (ClinicalTrials.gov identifier, NCT03116113) was a phase 2/3, 12-month, randomized (1:1:1) dose-expansion study. PARTICIPANTS: Male patients ≥10 years of age with RPGR-associated XLRP were included. METHODS: Participants were randomized 1:1:1 to receive low-dose subretinal cotoretigene toliparvovec (5 × 1010 vector genomes/eye), high-dose cotoretigene toliparvovec (2.5 × 1011 vector genomes/eye) or to be an untreated control participant. MAIN OUTCOME MEASURES: The primary end point was the percentage of participants meeting microperimetry responder criteria (≥ 7-dB improvement at ≥ 5 of 16 central loci). Secondary end points included change from baseline in retinal sensitivity at the central 16 loci and the entire 68 loci at 12 months and change from baseline in low-luminance visual acuity (LLVA) at 12 months, as well as the proportion of eyes with a ≥ 15-Early Treatment Diabetic Retinopathy Study ETDRS letter LLVA and ≥ 10-ETDRS letter LLVA change from baseline at month 12. RESULTS: Because of the impact of the COVID-19 pandemic, enrollment ended before reaching the initial target, leaving the trial underpowered. Twenty-nine participants were included (low-dose group, n = 10; high-dose group, n = 10; control group, n = 9). At month 12, the percentage of participants meeting microperimetry responder criteria was not significantly different between either cotoretigene toliparvovec group (low dose, 37.5% [P = 0.3181]; high dose, 25.0% [P = 0.5177]) and the control group (22.2%). However, the mean change from baseline in microperimetry sensitivity improved significantly with the low-dose group versus the control group at month 12 (P = 0.0350). Significant improvement in LLVA occurred in the low-dose group versus the control group at month 12 (33.3% difference [80% confidence interval, 14.7%-55.2%]; P = 0.0498). Three ocular-related serious adverse events (SAEs) occurred in the low-dose group versus 7 SAEs in the high-dose group. CONCLUSIONS: The primary microperimetry end point was not met. Significant improvements in LLVA and mean microperimetry were observed compared with controls and fewer SAEs occured with low-dose compared with high dose cotoretigene toliparvovec. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Videoocular assessment of eye movement activity in an ataxia-telangiectasia: a case study.

PURPOSE: Ataxia-telangiectasia (A-T) is an autosomal recessive disorder characterized by progressive neurological deficits, including prominent oculomotor dysfunction. We report 5 cases of eye movement assessment in children 9-15 years old with A-T. METHODS: Three different oculomotor tasks (gaze holding, visually guided saccades and visual search) were used, and video-oculography was performed. Additionally, the scale for the assessment and rating of ataxia (SARA) score was used to assess severity of the cerebellar ataxia. RESULTS: Unstable gaze holding, nystagmus and saccadic intrusions were found. In addition to psychophysiological assessment results, we provide quantitative analysis of oculomotor activity, revealing a specific abnormal oculomotor pattern, consisting of (i) marked saccade hypermetria, (ii) unstable gaze holding, and (iii) gaze-evoked nystagmus. CONCLUSION: Our study opens the prospect to evaluate efficacy and safety of alternative methods for supporting the patient and improving his/her life quality.

Retinal Assessment Using In Vivo Electroretinography and Optical Coherence Tomography in Rodent Models of Diabetes.

Electroretinography and optical coherence tomography imaging allow for non-invasive quantitative assessment of the retina. These approaches have become mainstays for identifying the very earliest impact of hyperglycemia on retinal function and structure in animal models of diabetic eye disease. Moreover, they are essential for assessing the safety and efficacy of novel treatment approaches for diabetic retinopathy. Here, we describe approaches for in vivo electroretinography and optical coherence tomography imaging in rodent models of diabetes.

Neurophysiological visual assessment in patients with idiopathic intracranial hypertension: visual evoked potential and multifocal field electroretinography.

BACKGROUND: Determining the cause of visual deterioration in idiopathic intracranial hypertension (IIH) patients is of clinical necessity. This study aimed to study the effect of chronic increased ICP on the retina and optic nerve through objective electrophysiological measures in chronic IIH patients. METHODS: Thirty patients with chronic IIH and thirty age and sex-matched healthy controls were included in this study. Papilledema grade and CSF pressure were evaluated in the patients' group. Both groups were submitted to visual evoked potentials (VEP) and multifocal electroretinogram (mfERG). RESULT: The mean value of P100 latencies of the right and left on two check sizes, 1 deg and 15m in chronic IIH patients, was significantly delayed than controls (P-value < 0.001 for each). Chronic IIH patients showed a significantly lower amplitude of the right and left R1, R2, R3, R4 & R5 compared to controls (P-value < 0.001, < 0.001) (P-value < 0.001, < 0.001) (P-value < 0.001, < 0.001) (P-value < 0.001, = 0.001) (P-value = 0.002, < 0.001), respectively. Also, patients showed a significantly delayed peak time of the right and left R1 and R2 compared to controls (P-value < 0.001, < 0.001) (P-value = 0.001, = 0.009), respectively. There was a significant positive correlation between each of CSF pressure and papilledema grade with right and left PVEP latencies. In contrast, there was no statistically significant correlation between either CSF pressure or papilledema grade and PVEP amplitudes in both eyes. CONCLUSION: In chronic IIH patients, both optic nerve dysfunction and central retinal changes were identified, supported by VEP and the mfERG findings.

Assessment of the uniform field electroretinogram for mouse retinal ganglion cell functional analysis.

PURPOSE: The uniform field electroretinogram (UF-ERG) has been suggested as an alternative to the pattern electroretinogram (PERG) for non-invasive assessment of retinal ganglion cell (RGC) function in primates. We evaluated the validity of the UF-ERG to assess mouse RGC activity in vivo. METHODS: Unilateral optic nerve crush (ONC) was performed on adult C57BL/6J mice. Contralateral eyes with uncrushed optic nerves and eyes from surgically naive mice served as experimental controls. Electrophysiological visual assessment was performed at 12 weeks post-ONC. Flash-mediated visual-evoked cortical potentials (VEPs) were measured to confirm the robustness of the ONC procedure. Full-field flash ERGs were used to interrogate photoreceptor and retinal bipolar cell function. RGC function was assessed with pattern ERGs. Summed onset and offset UF-ERG responses to alternating dark and light uniform field flash stimuli of different intensities and wavelengths were recorded from ONC and control eyes, and relative differences were compared to the PERG results. Following electrophysiological analysis, RGC loss was monitored by immunohistochemical staining of the RGC marker protein, RBPMS, in post-mortem retinal tissues. RESULTS: ONC dramatically impacts RGC integrity and optic nerve function, demonstrated by reduced RGC counts and near complete elimination of VEPs. ONC did not affect scotopic ERG a-wave and b-wave amplitudes, while PERG amplitudes of eyes subjected to ONC were reduced by approximately 50% compared to controls. Summation of ON and OFF UF-ERG responses did not reveal statistically significant differences between ONC and control eyes, regardless of visual stimulus. CONCLUSIONS: PERG responses are markedly impaired upon ONC, while UF-ERG responses are not significantly affected by surgical trauma to RGC axons in mice. The more closely related pattern and uniform field ERGs recorded in primates suggests species-specific differences in RGC features or subpopulations corresponding to PERG and UF-ERG response generators, limiting the utility of the UF-ERG for mouse RGC functional analysis.

Assessment of factors affecting flicker ERGs recorded with RETeval from data obtained from health checkup screening.

PURPOSE: To determine the factors significantly associated with the amplitudes and implicit times of the flicker electroretinograms (ERGs) recorded with the RETeval system by analyzing the comprehensive data obtained during a health checkup screening. METHODS: Flicker ERGs were recorded with the RETeval system from 373 individuals who had a normal fundus and optical coherence tomography images. The sex, age, anthropometric, ophthalmologic, and hematologic data were collected from all participants who were 40- to 89-years-of-age. Univariable and multivariable linear mixed effects regression analyses were performed to identify factors that were significantly associated with the implicit times and amplitudes of the RETeval flicker ERGs. RESULTS: Univariable linear mixed effects regression analysis showed significant correlations between the implicit times and the best-corrected visual acuity, the age, the axial length, the blood sugar level, and the blood urea nitrogen level. Analyses by multivariable linear mixed effects regression identified that the axial length (ß = 0.28), the age (ß = 0.24), and the blood sugar level (ß = 0.092) were three independent factors that were significantly correlated with the implicit times of the RETeval flicker ERGs. Univariable linear mixed effects regression analysis also showed significant correlations between the amplitudes of the RETeval flicker ERGs and the age, the platelet count, and the creatinine level. Multivariable linear mixed effects regression models identified the age (ß = -0.092), the platelet count (ß = 0.099), and the creatinine level (ß = -0.12) as three independent factors that were significantly correlated with the amplitudes of the RETeval flicker ERGs. However, the smoking habits, body mass index, and the blood pressure were not significantly correlated with either the implicit times or amplitudes of the RETeval flicker ERGs. CONCLUSIONS: Our results indicate that the age and some ophthalmologic and hematologic findings but not the anthropometric findings were significantly associated with the implicit times and amplitudes of the RETeval flicker ERGs. Thus, clinicians should remember these factors when analyzing the RETeval flicker ERGs.

Electrophysiological assessment of nutritional optic neuropathy: a case report.

PURPOSE: To report an unexpectedly asymmetric, progressive nutritional optic neuropathy associated with vitamin A deficient optic canal hyperostosis in a 15-year-old female with a long history of a restricted diet. METHODS: We performed comprehensive ophthalmic assessments in a fifteen-year-old female with a long history of restricted eating who presented with suspected nutritional optic neuropathy, predominantly affecting the right eye vision. RESULTS: A review of computerised tomography and magnetic resonance imaging revealed bilateral optic canal hyperostosis likely associated with vitamin A deficiency. Electrodiagnostic tests and optical coherence tomography provided structure-function evidence of bilateral retinal ganglion cell dysfunction and notably revealed severe loss of temporal fibres in the left eye which showed cecocentral scotoma but normal visual acuity. Although selective damage of the papillomacular bundle has been well-documented in nutritional and toxic optic neuropathies, compressive optic canal hyperostosis secondary to nutritional deficiency has been rarely reported. CONCLUSIONS: Nutritional deficiencies are increasing in high-income countries and may be linked to the rise of gastrointestinal disorders, strict vegan and vegetarian diets and avoidant restrictive food intake disorder (ARFID) associated with conditions such as depression and autism spectrum syndrome (ASD). Our findings highlight the value of electrodiagnostic testing alongside imaging in complex nutritional optic neuropathies to help monitor, guide treatment and preserve remaining sight in a child.

Assessment of digital light processing (DLP) projector stimulators for visual electrophysiology.

INTRODUCTION: Visual electrophysiology tests require the use of precise and calibrated visual display units (VDUs). Existing VDUs for presenting structured stimuli are now mostly obsolete, with modern solutions limited or unsuitable for clinical testing. Digital light processing (DLP) laser projectors have recently become commercially available and this study aimed to assess their suitability as VDUs for visual electrophysiology testing. METHODS: This study consisted of two sections. The first was a photometric study of two DLP laser projectors (Viewsonic LS831WU and HiSense 100L5FTUK) to assess luminance, contrast, spectral and temporal characteristics of the stimulus. The second was a physiological study comparing pattern electroretinograms (PERG) and visual evoked potentials (PVEPs) amplitudes and peak-times recorded using a DLP laser projector, photometrically and spatially matched to existing plasma VDUs at our institution (Pioneer Electronics Corporation, PDP422MXE). RESULTS: The Viewsonic DLP laser projector was capable of high luminance levels (0-587.5 cd/m2) whilst maintaining contrast above 93%. The temporal properties showed fast rise and fall times of 0.5-1 ms and 0.5-1 ms, respectively, without any transient luminance change with reversals. The device required a warm-up time of at least 2 min until reaching near maximal luminance. The second (Hisense) device was observed to have a detrimental input lag jitter so was not used for any further analysis. PERGs and PVEPs showed high agreement and correlation (r = 0.766-0.905) between the Viewsonic DLP device and existing plasma VDUs. No significant differences were observed for P50 and P100 peak-time (p = > 0.05), however P50, N95 and P100 amplitudes were all significantly larger for the DLP device (p = < 0.05). DISCUSSION: The DLP laser projector tested in this study is a viable and practical replacement VDU for clinical electrophysiology tests of vision. The device is easily capable of meeting ISCEV standards, and showed PERG and PVEP amplitudes larger than existing systems despite photometric and spatial matching. The DLP laser projectors are capable of very large field sizes so are beneficial for paediatric testing or those wishing to examine large field responses. Importantly, it was observed that some devices may suffer input lag jitter, therefore, individual calibration and assessment of DLP projection systems is an important consideration before clinical implementation.

Role of pattern electroretinogram in assessment of retinal dysfunction in hypertensive patients.

BACKGROUND: Hypertension is a major health problem in both developing and developed countries. Hypertension causes retinal structural and functional impairment within the ganglion cell layer. Pattern electroretinogram (PERG) offers an objective simple tool for assessment of retinal ganglion cell function.Aim of the work: To assess retinal dysfunction in hypertensive patients with or without signs of retinopathy using PERG. MATERIALS AND METHODS: This is a case control study, including ninety-eight eyes. Twenty-eight eyes of healthy subjects served as a control group (group I) and seventy eyes of patients with systemic hypertension, who were further subdivided into group II including 39 eyes of hypertensive patients with normal fundus and group III including 31 eyes of patients with signs of hypertensive retinopathy. All subjects were subjected to ophthalmological examination and electrophysiological assessment using PERG. RESULTS: PERG implicit times were significantly prolonged and amplitudes were significantly reduced in patients with established hypertensive retinopathy. PERG abnormalities were detected in 96.8% of hypertensive retinopathy patients and 79.5% of hypertensive patients with normal fundus. CONCLUSIONS: PERG can objectively assess retinal dysfunction in hypertensive patients and may be considered a promising tool for early detection of hypertensive retinopathy.

[Multifocal electroretinography in assessment of the functional state of the central retina in patients with retinitis pigmentosa]. / Mul'tifokal'naya elektroretinografiya v otsenke funktsional'nogo sostoyaniya tsentral'noi zony setchatki pri pigmentnom retinite.

Retinitis pigmentosa (RP) is an inherited disease associated with various genetic mutations. Developments in the field of genetic engineering give relevance to the search for methods of studying retinal function, which can prove informative in the selection of patients for treatment. PURPOSE: To evaluate the information content of multifocal electroretinography (mfERG) in the diagnostics of the functional state of the central retina in retinitis pigmentosa (RP). MATERIAL AND METHODS: The study included 115 patients (228 eyes) with PR and 15 people (30 eyes) who comprised the control group. All subjects underwent standard ophthalmological examination, computer perimetry, color vision study, retinal spectral optical coherence tomography, ganzfeld electroretinography (gERG) and mfERG. The relationship between mfERG parameters and the degree of gERG changes, as well as various functional and morphological parameters of the retina was assessed. RESULTS: Visual acuity and perimetry indices varied over a wide range. GERG was unrecordable in 50.4% of cases. MfERG was registered in 214 (98.3%) eyes with varying degrees of change in visual acuity, visual field and gERG parameters. A medium degree positive relationship was revealed between the biopotential density of the retina in the foveal and parafoveal zones and visual acuity (rs=0.68; 0.63), a high degree - between the density of ttotal biopotential of the central retina (DValue) and the average light sensitivity (rs=0.9), a weak degree - between DValue and the thickness and volume of the peripheral retina (rs=0.37; 0.42), a medium negative correlation was found between the average defect in light sensitivity and the biopotential density in the periphery (Rings 4-5) on mfERG, DValue (rs= -0.67; -0.65; -0.69). CONCLUSION: MfERG detects retinal dysfunctions at an early stage of RP, in eyes with high visual acuity, normal parameters of the central visual field and gERG, as well as in low visual acuity, a pronounced decrease in light sensitivity, unrecordable gERG. MfERG can be informative in the selection of patients with RP for gene therapy.

Visual evoked potentials as a method for the prospective assessment of tacrolimus neurotoxicity in patients after kidney transplantation.

INTRODUCTION: Neurotoxicity, including optic nerve injury, is one of the most common adverse effects of tacrolimus, the principal calcineurin inhibitor used after kidney transplantation (KTx). The electrophysiologic measurements of both pattern visual evoked potentials (PVEP) and flash visual evoked potentials (FVEP) are valuable when drug-induced optic neuropathy is suspected. OBJECTIVES: To determine whether VEP measurement is a sensitive and repeatable method for monitoring tacrolimus neurotoxicity. MATERIAL AND METHODS: This prospective study focused on 35 patients (20 M, 15F, 69 eyes, mean age 43 ± 11 years) who were at a median of 3.0 (IQR, 2.2-3.7) months after KTx at the time of the initial VEP evaluation and were treated with tacrolimus since KTx. The follow-up VEP examination was done after a median of 24 (22-27) months (both VEP measurements followed the ISCEV standards). The P100 wave latency and amplitude for the 1° and 15' PVEP simulations, and the P2 wave latency and amplitude for the FVEP were analyzed. RESULTS: For the 1° checks, the P100 wave latency and amplitude values were significantly worse in the follow-up examination compared to the early post-transplant time-point. Independent associations between FVEP parameters and the tacrolimus blood trough level were observed in the follow-up examination but not at the early post-transplant period. The P2 wave latency correlated with the tacrolimus trough level only in patients treated with the twice-daily, but not the once-daily, tacrolimus formulation. The brain derived neurotrophic factor (BDNF) level correlated with the P100 (15') latency (R = 0.499; p = 0.005) and the P2 latency (R = 0.409; p = 0.025) only in patients treated with the once-daily, but not the twice-daily, tacrolimus formulation. CONCLUSION: The observations in this study may support the rationale for the use of VEP measurements as non-invasive monitoring of subclinical tacrolimus neurotoxicity.

Electrophysiological Assessment in Birdshot Chorioretinopathy: Flicker Electroretinograms Recorded With a Handheld Device.

Purpose: The flicker electroretinogram (ERG) is a sensitive indicator of retinal dysfunction in birdshot chorioretinopathy (BCR). We explored recordings from a handheld device in BCR, comparing these with conventional recordings in the same patients and with handheld ERGs from healthy individuals. Methods: Non-mydriatic flicker ERGs, using the handheld RETeval system (LKC Technologies), were recorded with skin electrodes at two centers. At one center (group 1), the stimuli (85 Td·s, 850 Td background) delivered retinal illuminance equivalent to international standards; at the other center (group 2), a different protocol was used (32 Td·s, no background). Patients also underwent international standard flicker ERG recordings with conventional electrodes following mydriasis. Portable ERGs from patients were also compared with those from healthy individuals. Results: Thirty-two patients with BCR (mean age ± SD, 56.4 ± 11.3 years) underwent recordings. Portable and standard ERG parameters correlated strongly (r > 0.75, P < 0.01) in both groups. Limits of agreement for peak times were tighter in group 1 (n = 21; -4.3 to +2.0 ms [right eyes], -3.9 to 1.5 ms [left eyes]) than in group 2 (n = 11; -3.4 to +6.9 ms [right eyes], -4.8 to +9.0 ms [left eyes]). Compared with healthy controls (n = 66 and n = 90 for groups 1 and 2, respectively), patients with BCR showed smaller mean amplitudes and longer peak times. Conclusions: Portable ERGs correlated strongly with conventional recordings, suggesting potential in rapid assessment of cone system function in office settings. Translational Relevance: Flicker ERGs, known to be useful in BCR, can be obtained rapidly with a portable device with skin electrodes and natural pupils.

[Electrophysiological and psychophysical studies in assessment of visual functions in patients with hereditary optic neuropathy]. / Elektrofiziologicheskie i psikhofizicheskie issledovaniya v otsenke zritel'nykh funktsii pri nasledstvennoi opticheskoi neiropatii.

PURPOSE: To study the capabilities of electrophysiological and psychophysical examination methods for assessment of the functional state of ganglion cells, retina and optic nerve in patients with hereditary optic neuropathy (HON). MATERIAL AND METHODS: The study included 60 patients (118 eyes) with a genetically confirmed diagnosis of HON. All study patients underwent visual field test (VFT), spectral optical coherence tomography (OCT), flash and pattern visual evoked potentials (VEP) (Flash-VEP, FVEP; Pattern-VEP, PVEP), photopic electroretinography with photonegative response (PhNR) registration and the color vision test. In 24 patients (46 eyes), these parameters were assessed before the start of treatment and one year later. The treatment involved the mitochondria-targeted antioxidant SkQ1 - plastoquinonyl-decyl-triphenylphosphonium bromide (PDTP) in the form of eye drops. RESULTS: The main PVEP components for 1.0° and 0.3° were registered in 20% and in 14% of patient eyes with HON and high visual functions, respectively. After one year of PDTP use, a significant decrease in P100 peak latency was found only in the group with disease duration of ≤1.5 years as of the time of treatment start (p<0.05). Significant differences were observed in the PhNR amplitude (p<0.004) between patients of the main and the control groups, as well as in the PhNR amplitude between patients with visual acuity of ≤0.1 and ≥0.13 (p<0.01). Patients with high visual functions were found to have a correlation between the PhNR amplitude, GCC thickness and the global loss index (GLV). CONCLUSION: Along with VFT, OCT and color vision tests, electrophysiological studies are one of the main methods of examining patients with HON. After one year of PDTP use, there was a significant decrease in the FVEP P2 peak latency in the group with a disease duration of ≤1.5 years as of the time of treatment start. The PhNR amplitude in patients with high visual functions was found to correlate with structural changes in the ganglion cell layer and the retinal nerve fiber layer.

Assessment of retinal oxygen metabolism, visual function, thickness and degeneration markers after variable ischemia/reperfusion in rats.

After total retinal ischemia induced experimentally by ophthalmic vessel occlusion followed by reperfusion, studies have reported alterations in retinal oxygen metabolism (MO2), delivery (DO2), and extraction fraction (OEF), as well as visual dysfunction and cell loss. In the current study, under variable durations of ischemia/reperfusion, changes in these oxygen metrics, visual function, retinal thickness, and degeneration markers (gliosis and apoptosis) were assessed and related. Additionally, the prognostic value of MO2 for predicting visual function and retinal thickness outcomes was reported. Sixty-one rats were divided into 5 groups of ischemia duration (0 [sham], 60, 90, 120, or 180 min) and 2 reperfusion durations (1 h, 7 days). Phosphorescence lifetime and blood flow imaging, electroretinography, and optical coherence tomography were performed. MO2 reduction was related to visual dysfunction, retinal thinning, increased gliosis and apoptosis after 7-days reperfusion. Impairment in MO2 after 1-h reperfusion predicted visual function and retinal thickness outcomes after 7-days reperfusion. Since MO2 can be measured in humans, findings from analogous studies may find value in the clinical setting.

Assessment of optical coherence tomography angiography and multifocal electroretinography in eyes with and without nonproliferative diabetic retinopathy.

PURPOSE: To examine (i) the retinal structure and function using optical coherence tomography angiography (OCTA) and multifocal electroretinography (mfERG), respectively, in eyes with and without nonproliferative diabetic retinopathy (NPDR), (ii) and their interrelationship between retinal structure (OCTA) and function (mfERG) in the two groups independently. METHODS: This was a prospective observational study. One hundred twenty-one eligible participants with type 2 diabetes with No DR (n = 89), or with mild or moderate NPDR (n = 32) underwent ophthalmic examination, ultrawide field-view fundus photography, OCTA, and mfERG. Group differences were assessed using a Mann-Whitney U test. Correlations were assessed using Spearman's rho. RESULTS: There were no significant differences in OCTA measures between the two groups. The mfERG P1 implicit times (rings 1-6) were significantly delayed and P1 response densities in rings 5 and 6 were significantly lower in participants with NPDR compared to those with No DR. In those with No DR, P1 implicit times in almost all rings were delayed in relation to lower vessel density and perfusion (maximum variance noted was 13%). In individuals with NPDR, the P1 response density in rings 2 and 3 showed a positive nonsignificant correlation with macular perfusion. CONCLUSION: In those with diabetes with No DR, retinal neuronal function is influenced by lower macular vessel density and perfusion. The retinal neuronal function is abnormal in individuals with NPDR compared to those with No DR and is not correlated with OCT angiometric measures, suggesting the likelihood of a different retinal structural correlate.

Visual electrophysiology in the assessment of toxicity and deficiency states affecting the visual system.

Visual disturbance or visual failure due to toxicity of an ingested substance or a severe nutritional deficiency can present significant challenges for diagnosis and management, for instance, where an adverse reaction to a prescribed medicine is suspected. Objective assessment of visual function is important, particularly where structural changes in the retina or optic nerve have not yet occurred, as there may be a window of opportunity to mitigate or reverse visual loss. This paper reviews a number of clinical presentations where visual electrophysiological assessment has an important role in early diagnosis or management alongside clinical assessment and ocular imaging modalities. We highlight the importance of vitamin A deficiency as an easily detected marker for severe combined micronutrient deficiency.

Assessment of Macular Function by Multifocal Electroretinogram in Patients with Multiple Sclerosis Treated with Fingolimod.

INTRODUCTION: This study aimed to evaluate whether treatment with fingolimod (FTY) may induce functional changes on the macular pre-ganglionic retinal elements in patients affected by relapsing-remitting multiple sclerosis (RR-MS) without optic neuritis (ON). METHODS: This case-control observational and retrospective study assessed multifocal electroretinogram (mfERG) responses from 35 healthy controls (mean age 43.58 ± 5.76 years), 41 patients with RR-MS without ON (mean age 40.64 ± 4.83 years, MS-noFTY group), and from 21 patients with RR-MS without ON (mean age 42.38 ± 12.34 years) and treated with fingolimod (Gilenya®, Novartis Europharm, 0.5 mg/day) (MS-FTY group). MfERG N1 and P1 implicit times (ITs), and N1-P1 response amplitude densities (RADs) were measured from concentric rings (R) with increasing foveal eccentricity: 0-5° (R1), 5-10° (R2), 10-15° (R3), 15-20° (R4), 20-25° (R5). We considered R1 and R2 as "central macular areas" and R3, R4 and R5 as "more eccentric retinal areas". In the MS-FTY group, mfERG recordings were performed between 6 and 12 months (mean 7.2 ± 1.5 months) from the start of FTY. RESULTS: In the MS-FTY group, the mean values of mfERG N1 and P1 ITs and RADs detected in both central macular areas (R1 and R2) and in more eccentric retinal areas (R3, R4 and R5) were not significantly different (p > 0.01) with respect to those of control and MS-noFTY groups. CONCLUSIONS: Our mfERG results suggest that the chronic use of FTY does not induce a dysfunction of pre-ganglionic retinal elements located in the 0-25° of central retina. Since FTY does not cause any retinal functional abnormality, we suggest that FTY treatment could not produce any toxic effect on pre-ganglionic retinal elements even in the absence of macular oedema.