Visual evoked potentials can be reliably recorded using noninvasive epidermal electrodes in the anesthetized rat

Purpose

Visual evoked potentials (VEPs) are a powerful tool to evaluate nervous conduction along the visual pathways, both in humans and in animal models. Traditionally, epidural screw electrodes are used to record VEPs in preclinical research. Here we tested the feasibility in the preclinical setting of the same noninvasive technique used for clinical VEP acquisition, by using epidermal cup electrodes with no surgical procedures.

Methods

Monocular flash VEPs were recorded bilaterally under sevoflurane anesthesia once a week for 6 weeks in 14 dark Agouti rats, 7 with implanted epidural screws and 7 with epidermal 6 mm Ø Ag/AgCl cups.

Results

VEP traces obtained with the two techniques were morphologically comparable. There were no significant differences in latency of the main visual component between screw-recorded VEPs (sVEPs) and cup-recorded VEPs (cVEPs). Amplitude values with epidermal cups were significantly lower than those with epidural screws. Both techniques provided latencies and amplitudes which were stable over time. Furthermore, with regard to latency both methods ensured highly repeatable measurements over time, with epidermal cups even providing slightly better results. On the other hand, considering amplitudes, cVEPs and sVEPs provided fairly acceptable repeatability.

Conclusions

Epidermal cup electrodes can provide comparable results to those obtained with the “gold standard” epidural screws, while representing a simpler and less invasive technique to test nervous conduction along the visual pathways in the preclinical setting.

     

New photic stimulating system with white light-emitting diodes to elicit electroretinograms from zebrafish larvae

Purpose

The zebrafish is an established animal model commonly used in biological, neuroscience, and genetic research. We have developed a new light stimulating system using white light-emitting diodes (LEDs) to elicit ERGs from zebrafish larvae. The purpose of this study was to record full-field ERGs and to evaluate the inter-trial reliability of the ERGs recorded with our system from zebrafish larvae.

Methods

The stimulating device used white LEDs that were attached to a stereomicroscope, and the location of the recording electrode on the cornea could be monitored while the eye was being stimulated. Full-field scotopic and photopic ERGs were recorded from larvae at the age of 5–7 days post-fertilization (dpf). Intensity–response curves were constructed from the ERGs. Inter-trial reliability of the ERGs recorded by our system was evaluated.

Results

This stimulating system could be used for efficient and reliable ERG recordings from 5–7 dpf larvae. The amplitudes, implicit times, and the waveforms of the scotopic and photopic ERGs were similar to those reported in earlier studies. Inter-trial reliability of the amplitudes of the photopic ERG b-waves was excellent with an intra-class correlation coefficient of 0.98.

Conclusion

We conclude that this new light stimulation system using white LEDs attached to a stereomicroscope will be helpful in recording reliable ERGs from zebrafish larvae.

     

Pattern- and motion-related visual evoked potentials in HIV-infected adults

Purpose

The goal of the current study was to explore visual function in virally suppressed HIV patients undergoing combined antiretroviral therapy (cART) by using pattern-reversal and motion-onset visual evoked potentials (VEPs).

Methods

The pattern-reversal and motion-onset VEPs were recorded in 20 adult HIV+ patients with a mean age of 38 years and CD4 cell counts ≥230 × 10⁶ cells/L of blood.

Results

Nine out of 20 patients displayed VEP abnormalities. Pattern-reversal VEPs pathology was observed in 20% of subjects, and 45% HIV patients had impaired motion-onset VEPs. Five out of 16 neurologically asymptomatic HIV patients had prolonged motion-onset VEP latencies in both eyes. Four neurologically symptomatic patients displayed simultaneously abnormal motion-onset and pattern-reversal VEP latencies: monocular involvement was observed in two patients with Lyme and cytomegalovirus unilateral optic neuritis. Binocular involvement was noted in two patients with cognitive deficits. Correlation analysis between disease duration, CD4 cell count, HIV copies in plasma, MoCA and electrophysiological parameters did not show any significant relationships.

Conclusions

The functional changes of the visual system in neurologically asymptomatic virally suppressed HIV patients displayed higher motion-onset VEP sensitivity than in standard pattern-reversal VEP examinations. This promising marker, however, has no significant association with clinical conditions. Further exploration is warranted.

     

Imitating the effect of amblyopia on VEP-based acuity estimates

Purpose

Acuity testing based on visual evoked potentials (VEPs) overestimates acuity in patients with amblyopia. We hypothesized that distortion and fragmentation of the stimulus in amblyopia impede recognition of optotypes, while it leaves the pattern onset response in the VEP mostly unaffected, resulting in overestimation of acuity.

Methods

Acuity VEPs were recorded in visually normal participants with the stimulus degraded by patterned polymethyl methacrylate panes, which induce distortion and fragmentation. For comparison, frosted panes were used to induce blur through wide-angle scattering. Standard psychophysical optotype acuity was recorded under the same conditions.

Results

With the distorted and fragmented stimuli, the VEP consistently overestimated acuity relative to psychophysical optotype acuity. With blurred stimuli, both measures were in good agreement.

Conclusions

The data support the assumption that stimulus distortion and fragmentation leave VEP-based measures of acuity relatively unaffected, resulting in a discrepancy between measures of acuity that are based on checkerboard VEPs on one hand and psychophysical optotype recognition on the other hand. The technique of stimulus degradation described here provides a simple and efficient way of imitating effects that are known from amblyopia and may thus serve as a tool in the evaluation of vision tests.

     

VEP and PERG in patients with multiple sclerosis,with and without a history of optic neuritis

Purpose

Visual electrophysiology is routinely used to detect the visual complications of multiple sclerosis, but the analysis mostly focuses on visual evoked potential (VEP) and especially the P100 component. Our goal was to analyze the components and waveform alterations of VEPs and pattern electroretinograms (PERGs) in patients with multiple sclerosis (MS) with good vision.

Methods

The main VEP and PERG components of 85 patients with MS were analyzed in two groups: 38 patients who had optic neuritis in their history (ON group) and 47 patients who had never had optic neuritis (MS group). The results were compared against a control group of 47 healthy subjects.

Results

Both VEP and PERG alterations occurred in a greater number of patients than expected, and these alterations were not necessarily linked to ON in the history or a deterioration of visual acuity.

Conclusions

Both VEP and PERG can detect dysfunction in the visual system in MS, even if the patient has no subjective symptoms. Even if PERG is not routinely used in neuro-ophthalmology, the results suggest that PERG assessment may provide useful information describing the retinal defect in MS.

     

The relationship of nystagmus waveform on the VEP response in infantile nystagmus syndrome: a small case series

Purpose

The relationship between eye movements and the visual evoked potential (VEP) response was examined in two subjects with infantile nystagmus syndrome (INS). Changes in VEP amplitude were compared between periods of foveation versus periods of high-frequency nystagmus. An analysis is proposed that improves extraction of the checkerboard reversal VEP signal from subjects with INS.

Methods

INS subjects were 2 healthy children (12–13 years old) with 20/40 or better corrected acuity. Optical coherence tomography confirmed the optic nerves, retina, and fovea were within normal variation. VEPs were recorded to checkerboard reversal and to onset/offset of horizontal gratings while simultaneously recording the electrooculogram (EOG). VEP epochs underwent Fourier analysis, and epochs were examined for phase consistency with the mean. Foveation periods were compared to video-oculography recordings from a separate session.

Results

Optic nerve misrouting, such as crossed VEP asymmetry seen in albinism, or ipsilateral VEP asymmetry seen in achiasma, was not detected in either subject. By averaging only epochs in which EOG epochs showed foveation, VEP amplitude could be increased ≥59%. Averaging the VEP only on epochs with consistent phase at Oz increased VEP amplitude by ≥twofold; subsequent EOG epochs after this analysis mostly contained foveation periods or minimal EOG activity. Latency varied <14 ms across all analyses.

Conclusions

The checkerboard reversal VEP signal is dependent on foveation periods in subjects with INS despite good visual acuity. Reduction in VEP amplitude due to retinal image motion induces noise and/or lack of phase locking in the VEP epochs. Selective averaging of epochs based on phase consistency improves the extraction of a VEP signal, likely when retinal image motion is minimized.

     

Safety of intravitreal clindamycin in albino rabbit eyes

Purpose

To study the potential toxic effects of intravitreal clindamycin on the retina of albino rabbits, by assessing functional and morphological retinal changes.

Methods

Eight albino rabbits were included in the study. In each rabbit, 1 mg/0.1 ml clindamycin was injected into the vitreous of the right (experimental) eye, and 0.1 ml saline was injected into the vitreous of the left (control) eye. The electroretinogram (ERG) was recorded before injection, 3 days, 1, 2, and 4 weeks post-injection. The visual evoked potential (VEP) was recorded 4 weeks post-injection. Clinical examination was conducted at all time points. The eyes were enucleated at the termination of the follow-up period in order to prepare the retinas for histology in order to assess retinal structure.

Results

ERG and VEP responses that were recorded from the experimental eye at different times following intravitreal clindamycin injection were very similar to the corresponding responses that were recorded from the control eyes. Clinical examination was normal in all eyes, and no histological damage was observed.

Conclusions

Intravitreal injection of 1 mg clindamycin does not cause functional or morphological signs of retinal toxicity in albino rabbits, during a period of 4 weeks post-injection. These findings support the clinical use of 1 mg intravitreal clindamycin.

     

Effect of varying skin surface electrode position on electroretinogram responses recorded using a handheld stimulating and recording system

Purpose

A handheld device (the RETeval system, LKC Technologies) aims to increase the ease of electroretinogram (ERG) recording by using specially designed skin electrodes, rather than corneal electrodes. We explored effects of electrode position on response parameters recorded using this device.

Methods

Healthy adult twins were recruited from the TwinsUK cohort and underwent recording of light-adapted flicker ERGs (corresponding to international standard stimuli). In Group 1, skin electrodes were placed in a “comfortable” position, which was up to 20 mm below the lid margin. For subsequent participants (Group 2), the electrode was positioned 2 mm from the lid margin as recommended by the manufacturer. Amplitudes and peak times (averaged from both eyes) were compared between groups after age-matching and inclusion of only one twin per pair. Light-adapted flicker and flash ERGs were recorded for an additional 10 healthy subjects in two consecutive recording sessions: in the test eye, electrode position was varied from 2 to 10–20 mm below the lid margin between sessions; in the fellow (control) eye, the electrode was 2 mm below the lid margin throughout. Amplitudes and peak times (test eye normalised to control eye) were compared for the two sessions.

Results

Including one twin per pair, and age-matching yielded 28 individuals per group. Flicker ERG amplitudes were significantly lower for Group 1 than Group 2 participants (p?=?0.0024). However, mean peak times did not differ between groups (p?=?0.54). For the subjects in whom electrode position was changed between recording sessions, flash and flicker amplitudes were significantly lower when positioned further from the lid margin (p?<?0.005), but peak times were similar (p?>?0.5).

Conclusions

Moving the skin electrodes further from the lid margin significantly reduces response amplitudes, highlighting the importance of consistent electrode positioning. However, this does not significantly affect peak times. Thus, it may be feasible to adopt a more comfortable position in participants who cannot tolerate the recommended position if analysis is restricted to peak time parameters.

     

VEP analysis methods in children with optic nerve hypoplasia: relationship to visual acuity and optic disc diameter

Purpose

Assessing vision in young children with optic nerve hypoplasia (ONH) is challenging due to multi-directional infantile nystagmus, the range of optic nerve loss, and cognitive delay. This study examined visual evoked potential (VEP) responses and averaging techniques in children with ONH. The assumption is that EEG epochs with inconsistent temporal phase would be associated with nystagmus, signal reduction due to axon loss, and visual inattention.

Methods

A retrospective chart review was performed on 44 children (average age 2.2 years; SD 1.9). Optic disc diameter was estimated by ophthalmoscopy. Visual function was measured under binocular viewing and then compared to the eye with the larger optic disc to exclude secondary amblyopia. Visual acuity was measured by Teller cards or by recognition optotypes, and both measures were converted into log minimum angle of resolution (logMAR). VEPs were recorded to onset/offset of horizontal gratings and to reversing checkerboards. Signal-to-noise ratios (SNRs) were estimated from phase consistency across epochs in the Fourier domain. VEPs were also averaged after (1) correction of epochs for phase shifts across a limited bandwidth, or (2) selection of only epochs showing phase consistency.

Results

Optic disc diameter, logMAR, VEP amplitudes, and VEP SNR were all significantly inter-correlated. Optic disc diameter correlated best with VEP SNR (Spearman rho = 0.82; p < 0.001). Age-corrected logMAR correlated with optic disc diameter and VEP SNR (Spearman rho = ?0.695 and 0.70, respectively; p < 0.001). VEP latency poorly correlated with optic disc diameter or logMAR. Correction of phase shifts or selection of epochs based on phase consistency significantly increased VEP amplitude and SNR for children with optic disc diameters <1000 microns. Correction of phase inconsistency did not improve the correlation of VEP parameters with optic disc diameter or with logMAR.

Conclusions

In ONH, the size of the optic nerve is correlated with VEP SNR and logMAR. The results imply a direct relationship between the reduction in optic nerve axons and generalized reduction in visual function. Our calculation of VEP SNR provides objective assessment of optic nerve function that is independent of subjective scoring of VEP peaks.

     

Deferoxamine-induced electronegative ERG responses

Purpose

To report a case of deferoxamine-induced retinopathy characterized by electroretinography (ERG), optical coherence tomography angiography (OCT-A), and other multimodal imaging.

Methods

This is an observational case report of one patient. Full-field ERG was performed. OCT-A, spectral-domain optical coherence tomography (SD-OCT), color fundus photography, and fundus autofluorescence were used to characterize the retinopathy induced by deferoxamine use.

Results

A 64-year-old man with a history of β-thalassemia intermedia presented with worsening visual acuity, nyctalopia, and electronegative ERG. OCT-A revealed atrophy of the choriocapillaris in areas of hypoautofluorescence, corresponding to regions of retinal atrophy. SD-OCT showed disruption of the ellipsoid zone, granular hyperreflective deposits within the retinal pigment epithelium, thinning of the retinal layers, and extensive choroidal sclerosis and atrophy of the retinal pigment epithelium.

Conclusion

Deferoxamine-induced retinopathy can manifest with electronegative maximal ERG responses, and OCT-A can be used to detect deferoxamine toxicity.

     

The morphology of human rod ERGs obtained by silent substitution stimulation

Purpose

To record transient ERGs from the light-adapted human retina using silent substitution stimuli which selectively reflect the activity of rod photoreceptors. We aim to describe the morphology of these waveforms and examine how they are affected by the use of less selective stimuli and by retinal pathology.

Methods

Rod-isolating stimuli with square-wave temporal profiles (250/250 ms onset/offset) were presented using a 4 primary LED ganzfeld stimulator. Experiment 1: ERGs were recorded using a rod-isolating stimulus (63 ph Td, rod contrast, C _rod = 0.25) from a group (n = 20) of normal trichromatic observers. Experiment 2: Rod ERGs were recorded from a group (n = 5) using a rod-isolating stimulus (C _rod = 0.25) which varied in retinal illuminance from 40 to 10,000 ph Td. Experiment 3: ERGs were elicited using 2 kinds of non-isolating stimuli; (1) broadband and (2) rod-isolating stimuli which contained varying degrees of L- and M-cone excitation. Experiment 4: Rod ERGs were recorded from two patient groups with rod monochromacy (n = 3) and CSNB (type 1; n = 2).

Results

The rod-isolated ERGs elicited from normal subjects had a waveform with a positive onset component followed by a negative offset. Response amplitude was maximal at retinal illuminances <100 ph Td and was virtually abolished at 400 ph Td. The use of non-selective stimuli altered the ERG waveform eliciting more photopic-like ERG responses. Rod ERGs recorded from rod monochromats had similar features to those recorded from normal trichromats, in contrast to those recorded from participants with CSNB which had an electronegative appearance.

Conclusions

Our results demonstrate that ERGs elicited by silent substitution stimuli can selectively reflect the operation of rod photoreceptors in the normal, light-adapted human retina.

     

Phenotypic characterization of complete CSNB in the inbred research beagle: how common is CSNB in research and companion dogs?

Purpose

Although congenital stationary night blindness (CSNB) has been described in a Japanese beagle dog research colony, certain clinical correlates with human CSNB have not yet been described, nor has an estimate of frequency of the condition been made in inbred and outbred beagle populations.

Methods

A beagle with CSNB obtained from a commercial research dog supplier in the USA and matched control dogs (n?=?3) underwent examination, refraction, ocular imaging, assessment of visual navigation ability and detailed electroretinography (ERG). Retrospective review of ERGs in two independent groups of inbred (n?=?15 and 537, respectively) and one group of outbred dogs (n?=?36) was used to estimate CSNB frequency in these populations.

Results

In the affected dog, there were absent dark-adapted b-waves in response to dim-light flashes, severely reduced dark-adapted b-waves in response to bright-light flashes, and normal light-adapted b-waves with a-waves that had broadened troughs. Long-flash ERGs confirmed a markedly reduced b-wave with a preserved d-wave, consistent with cone ON-bipolar cell dysfunction. There was evidence of normal rod photoreceptor a-wave dark adaptation, and rapid light adaptation. In the wider beagle populations, five inbred beagles had a b/a wave ratio of <?1 in dark-adapted bright-flash ERG, whereas no outbred beagles had ERGs consistent with CSNB.

Conclusions

The identified dog had clinical findings consistent with complete type CSNB, similar to that described in the Japanese colony. CSNB appears to be a rare disorder in the wider beagle population, although its detection could confound studies that use retinal function as an outcome measure in research dogs, necessitating careful baseline studies to be performed prior to experimentation.

     

Consecutive unilateral recording of the two eyes affects dark-adapted ERG responses,when compared to simultaneous bilateral recording

Purpose

Our aim was to compare the electroretinographic (ERG) responses of two eyes obtained by consecutive unilateral recordings to those obtained by a simultaneous bilateral recording in sheep.

Methods

Eight sheep underwent two full-field ERG recordings, using two recording strategies of the standard ISCEV protocol: consecutive unilateral recordings of one eye after the other, and simultaneous bilateral recording of both eyes. The order of recording strategy within an animal (unilateral/bilateral), eye recording sequence in the unilateral session (OD/OS), and amplifier channel assignment for each eye were all randomized. To test whether duration of dark adaptation and/or anesthesia affect the results, the ISCEV protocol was recorded bilaterally in six additional eyes following 38 min of patched dark adaptation, as was done for the second eye recorded in the consecutive unilateral recordings.

Results

The second recorded eye in the unilateral session had significantly higher scotopic b-wave amplitudes compared to the first recorded eye and to the bilaterally recorded eyes. A-wave amplitudes of the dark-adapted mixed rod–cone responses to a high-intensity flash were also significantly higher in the second eye compared to the first eye recorded unilaterally and to the bilaterally recorded eyes. Light-adapted responses were unaffected by the recording strategy. When the ISCEV protocol was recorded after 38 min of dark adaptation, the scotopic responses were higher than in the first eyes, and similar to those of the second eyes recorded unilaterally, suggesting that indeed the longer duration of anesthesia and dark adaptation are responsible for the increased scotopic responses of the second eye.

Conclusions

Consecutive unilateral ERG recordings of two eyes result in higher amplitudes of the dark-adapted responses of the eye recorded second, compared to the eye recorded first and to bilaterally recorded eyes. The differences in scotopic responses can be attributed to different duration of dark adaptation and/or anesthesia of the two consecutively recorded eyes. Photopic responses are not affected. Therefore, simultaneous bilateral ERG responses should be recorded when possible, especially for evaluation of scotopic responses.

     

Case of adult-onset neuronal intranuclear hyaline inclusion disease with negative electroretinogram

Purpose

To report the findings in a 72-year-old man with neuronal intranuclear hyaline inclusion disease (NIHID) with the negative-type electroretinogram (ERG) and without night blindness.

Methods

Standard ophthalmological examinations including the medical history, measurements of the best-corrected visual acuity and intraocular pressures, slit-lamp biomicroscopy, ophthalmoscopy, spectral-domain optical coherence tomography, fundus autofluorescence, and perimetry were performed. In addition, neurological and electrophysiological examinations were performed.

Results

NIHID was confirmed by skin biopsy. The ophthalmologic examinations revealed sluggish pupillary reflexes without visual disturbances and retinal abnormalities. The amplitudes of the dark-adapted 0.01 ERG was absent, and light-adapted 3 ERG and light-adapted 30 Hz flicker ERG were reduced in amplitude and delayed in implicit time. The rod system was more severely affected than the cone system, indicating that NIHID is classified as one of rod-cone dysfunction syndrome. The dark-adapted 3 ERG consisted of a markedly reduced b-wave with larger a-wave (negative ERG), but the amplitude of a-wave was smaller than normal.

Conclusions

Since the ophthalmoscopical findings and the subjective visual functions may be essentially normal, the characteristic ERG abnormalities can be an important findings in adult-onset NIHID without night blindness.

     

Macular staphyloma in patients affected by Joubert syndrome with retinal dystrophy: a new finding detected by SD-OCT

Purpose

Joubert syndrome (JS) is an inherited autosomal recessive or X-lined disorder characterized by a congenital malformation of the mid-hindbrain and a large spectrum of clinical features. It is estimated that retinal dystrophy is present in association with the typical neurological findings in about one-third of the patients. The aim of this study is to better characterize the macular region in JS patients with and without retinal dystrophy.

Methods

We describe six individuals affected by JS as demonstrated by the presence of the typical “molar tooth sign” on MRI. The presence of retinal dystrophy was assessed by fundus examination and electrophysiology by means of full-field electroretinogram (ERG) and visual evoked potentials (VEP) at five spatial frequencies (300–15 min of arc). The macular region was examined with spectral domain optical coherence tomography (SD-OCT). All the exams were performed in awake conditions. All the patients underwent next-generation-sequencing analysis of known JS genes.

Results

Pathogenic biallelic variants in either the INPP5E gene or the AHI1 gene were detected in two pairs of siblings, all positive for retinal dystrophy. Genetic testing yielded no results in the remaining two patients, one with bilateral coloboma and retinal dystrophy and the other with normal fundus appearance. Decimal best-corrected visual acuity was between 0.1 and 1.0. In the two pairs of siblings, SD-OCT revealed a posterior staphyloma centred on the fovea, in one case associated with cystoid macular oedema. Macular morphology was just slightly altered in the fifth patient and completely normal in the last patient. Refractive error was between + 2.50 diopter sphere (DS) and ? 8 DS and ? 4 diopter cylinder ax 45°. ERG waves were markedly lower than the normal limits in both scotopic and photopic components in the two pairs of siblings and in the fifth subject, with VEP P100 latencies and amplitudes delayed and reduced in all spatial frequencies. ERG and VEP were within normal limits in the last patient.

Conclusions

To our knowledge, macular staphyloma has not been described before in JS. Further work is warranted to assess the true prevalence of staphyloma in JS and its connection to retinal dystrophy.

     

The importance of electrode position in visual electrophysiology

Purpose

The DTL fibre electrode is commonly used to record the electric potentials elicited by stimulation of the retina. Two positions are commonly used: it is placed either on the cornea along the lower lid or in the conjunctival fornix. The PERG and OPs have previously been examined and compared under both conditions. The aim of this study was to examine the ERG, flicker response and on–off responses with differing electrode positions.

Methods

Before recruitment, all subjects underwent an ophthalmological examination. We enrolled 13 normal control subjects into the study aged 13–64 years, all with a visual acuity of ≥1.0. We recorded scotopic and photopic ERGs, flicker and on–off responses, for both electrode positions. On the first day, one eye had the electrode placed on the cornea along the lower lid and the other eye had it positioned in the conjunctival sac. On a second day, the recordings were repeated with the alternative electrode placements.

Results

ERG, on–off and flicker responses were all smaller by between 20 and 25% when the DTL electrode was positioned in the conjunctival sac, compared to when it was positioned on the cornea, as did the scatter in the data points. This indicates that there is no advantage clinically for one or the other placement.

Conclusions

Our results confirm other reports examining the effect of electrode position on electrophysiological potentials. When recording with the DTL electrode, it is important to ensure that it is placed at the same position in repeat recordings or in multicentre trials and that it is stable and does not move during recording.

     

Test–retest reliability of scotopic full-field electroretinograms in rabbits

Purpose

To explore test–retest reliability of standard scotopic full-field ERG measurements in New Zealand White rabbits. The ERG is widely used for testing of retinal integrity after any ocular treatment. We here present detailed stimulus–response dependencies for single healthy and untreated animals, concentrating on test–retest reproducibility.

Materials and methods

Five New Zealand White rabbits (aged 8–10 weeks, weight about 2.0–2.5 kg) underwent binocular ERG measurements after intramuscular anesthesia and pharmaceutical pupillary dilatation at a baseline day and 10 days later. Eleven scotopic flash strengths (0.0001–10 cd s/m²) were presented. Variability was quantified via the 95% limits of agreement (LOA).

Results

The a-waves displayed the typical monotonic sigmoid amplitude increase with flash strength, and the b-waves peaked at 0.01 cd s/m², followed by a marked dip at 0.1–0.3 cd s/m². LOA of both waves went through a maximum in the dip region. LOA divided by mean amplitudes (relative LOA) was fairly flat over flash strength, around 20% beyond the dip. Intraindividual interocular variability was markedly lower, around 10%.

Conclusions

Scotopic ERG responses in rabbits display a region of high variability at 0.1–0.3 cd s/m²; beyond that region the amplitude-LOA is 20%, the interocular LOA being half that value. The use of intraindividual control eyes for testing any toxicity of ocular agents thus appears markedly more sensitive. As a rule of thumb, we found the relative 95% LOA as 33% between individuals, 20% across sessions and 10% between eyes.

     

Early postoperative evaluation of retinal function by electroretinography after vitreous surgery for idiopathic epimacular membrane

Purpose

In this study, we used the PuREC to carry out electroretinography (ERG) measurements using skin electrodes to assess changes before and after microincision vitreous surgery (MIVS) for epiretinal membrane (ERM) and evaluate the stress on retinal function soon after MIVS.

Methods

The study subjects were 18 eyes of 18 patients who underwent MIVS for ERM simultaneously with cataract surgery. ERG measurements were performed using a skin electrode on the day before vitreous surgery, on the day after surgery, and 1 week later. The amplitude and implicit time of each waveform were measured, and the changes between preoperative results and those 1 day and 1 week postoperatively were investigated.

Results

Preoperatively, the dark-adapted (DA) 0.01 ERG, the DA 3.0 ERG a-wave amplitude, and the light-adapted (LA) 3.0 ERG b-wave amplitude were significantly smaller in affected eyes compared with their fellow eyes (P < 0.05, Wilcoxon’s signed-rank test). The day after surgery, all-wave amplitude showed no significant difference compared to preoperatively (repeated-measures analysis of variance (ANOVA) post hoc test). One week after surgery, the LA 3.0 ERG for b-waves and flicker ERG amplitude had improved from the value on the day after surgery (P < 0.05, ANOVA post hoc test).

Conclusions

Cone ERG components have improved within 1 week after surgery by MIVS for ERM.

     

Juvenile open-angle Glaucoma associated with Leber’s hereditary optic neuropathy: a case report and literature review

Background

Leber’s hereditary optic neuropathy (LHON) is a maternally inherited recessive disease rarely complicated with glaucoma. We conducted a clinical and genetic retrospective case series to describe three cases of juvenile open-angle glaucoma (JOAG) and an ND4 m11778G?>?A mitochondrial DNA (mtDNA) mutation, which is pathognomonic for LHON.

Case presentation

Patient 1 was a 16-year-old boy diagnosed with bilateral JOAG and high myopia. His intraocular pressure (IOP) was poorly controlled with the use of full topical anti-glaucoma medications. His best-corrected visual acuity (BCVA) decreased gradually over 5?years. Fundoscopic examination revealed bilateral enlarged disc cupping of the optic nerves with sectorial excavation and reduction of the neural rim in the left eye. His visual field (VF) was characterized by bilateral progressive central scotoma. Pattern visual evoked potentials (VEPs) and pattern electroretinograms (ERGs) showed extinguished responses in both eyes. Because of the non-specific visual field findings and the optic neuropathy disclosed by the pattern VEPs and pattern ERGs, we arranged a genetic test for the patient, which revealed an m11778G?>?A mtDNA mutation. Patient 2, the younger brother of Patient 1, was a 15-year-old boy who had been diagnosed with bilateral JOAG in 2010. The BCVA of both eyes remained at 1.0 during the follow-up period. Fundoscopic examination revealed bilateral mildly paled optic disc with enlarged cupping and reduction of the neural rim. The pattern ERG revealed a decreased N95 amplitude bilaterally. The genetic test revealed an m11778G?>?A mtDNA mutation. Patient 3 was a 35-year-old man with bilateral JOAG. His BCVA decreased gradually over 10?years. Fundoscopic examination revealed paled optic disc with enlarged disc cupping and reduction of the neural rim in both eyes. The pattern ERG revealed a decreased N95 amplitude bilaterally. The genetic test revealed an m11778G?>?A mtDNA mutation.

Conclusions

This case series describes three patients with concomitant occurrence of JOAG and LHON. These two diseases may have a cumulative effect on oxidative stress and retinal ganglion cell death with the rapid deterioration of vision, which may occur during adolescence.