Retinal Function In Patients Research Articles

Age-Related Macular or Retinal Degeneration?

Age-related macular degeneration (AMD) is an eye disease that leads to progressive vision loss. Its prevalence has been increasing due to population aging. Previously, it was commonly believed that the disease affects the central retina, that is, the macula. However, recent studies have shown that it also involves the peripheral retina. Novel imaging techniques revealed various degenerative lesions that extend beyond the central macula. While their prevalence remains unknown, they seem to be more frequent in patients with late AMD. These findings suggest that the term "age-related retinal dysfunction" might be more adequate to describe some cases of AMD. They also raise the question about the role of electroretinography (ERG) as an objective measure of retinal function. The most common types of ERG tests used in AMD are multifocal (mfERG) and full-field ERG (ffERG). mfERG is more sensitive to macular changes, but the test is difficult to perform when fixation is unstable. On the other hand, ffERG reflects the function of the entire retina, not only the macular area. It helps assess the impact of peripheral retinal lesions and overall retinal function in patients with AMD. As ffERG results are normal in early-stage AMD, any abnormalities indicate that the disease is more severe and affects the entire retina. Anti-vascular endothelial growth factor injections improve retinal function in patients with neovascular AMD, as demonstrated by an increase in their ERG responses. More research is needed to assess the association between local and general retinal dysfunction. In this review, ffERG findings in patients with AMD are described and the usefulness of ffERG is discussed based on previous studies and cases from our own clinical practice.

Full-field stimulus threshold testing for evaluation of the retinal function in patients with inherited retinal dystrophy

Objective: To analyze the characteristics of full-field stimulus thresholds (FST) in patients with middle-and late-stage inherited retinal dystrophy (IRD), and to explore the feasibility of the FST test in evaluating the retinal function for IRD patients. Methods: It was a retrospective case series study. Twenty-eight patients with IRD whose full-field electroretinogram (ERG) results showed no electrical activity (20 patients) or a serious decrease in electrical activity (8 patients) were enrolled, including 17 males and 11 females, with a median age of 32 years. Ten normal controls were also enrolled. All eyes had a measurement of best corrected visual acuity (BCVA) and an ERG test. FST stimulated by red, blue and white lights were examined with an electrophysiological instrument. The decimal value of BCVA was converted to logarithm of the minimum angle of resolution. Data of the better eye of each participant were used for statistical analysis. Independent sample t-test was used to analyze the difference in FST among patients. Linear regression was used to analyze the correlation between FST, visual acuity and ERG amplitudes. Results: The BCVA of all eyes was 0.7±0.6. Under the stimulation of red, blue and white lights, the FST were(-27.0±7.1), (-47.4±12.2) and(-41.7±11.5) dB in the 8 eyes with decreased electrical activity, (-16.3±7.0), (-27.2±13.7) and(-23.5±12.5) dB in the 20 eyes with no electrical activity, and(-39.9±4.0), (-65.8±4.0) and(-58.5±3.4) dB in the 10 control eyes, respectively. The FST of IRD patients were higher than the normal controls. The FST with red, blue and white lasers in eyes with no electrical activity were higher than those with severely reduced electrical activity (t=-3.472, -3.506, -3.433; all P=0.002). The BCVA was not correlated with the FST under the red, blue and white light stimulation in eyes with a serious reduction in electrical activity (r=0.134, 0.011, 0.010; P=0.055, 0.601, 0.611). There was a linear correlation between the red light stimulus FST and the b-wave amplitude of flicker ERG response (r=-0.591, P=0.026), but there was no significant correlation between other amplitudes and FST. Conclusions: The FST test is a supplementary method to evaluate the retinal function of patients with middle-and late-stage IRD. It can quantitatively reflect the function of rod and cone cell system. As the retinal function of IRD patients decreases, the FST gradually increase.

INTERNAL LIMITING MEMBRANE PEELING DISTORTS THE RETINAL LAYERS AND INDUCES SCOTOMA FORMATION IN THE PERIFOVEAL TEMPORAL MACULA.

To determine whether internal limiting membrane peeling damages retinal function in patients with an idiopathic macular hole. Retrospective case series. Forty-five eyes of 45 idiopathic macular hole patients who underwent vitrectomy with internal limiting membrane peeling with a minimum follow-up of 6 months. Each patient received a complete ophthalmological examination. The eyes were examined by microperimetry MP-3 in the central 20° visual field and optical coherence tomography angiography in the central 6 × 6 mm area. Six months after the surgery, macular hole closed in each patient. Retinal sensitivity decreased significantly in the perifoveal temporal ETDRS sector (from 24.97 ± 2.67-19.98 ± 5.68 dB, P = 0.001) but not in the other sectors. Six patients (13%) developed 24 scotomas, 62.5% presented in the perifoveal temporal sector. Anatomically, bumps in the outer nuclear layer were discovered concurrent with inner retinal dimples on B-scan images, predominantly (76.8%) in the perifoveal temporal sector, which have not been previously reported. The incidence of outer nuclear layer bumps was significantly higher in patients with scotomas than in those without (83% vs. 18%, P = 0.014). Internal limiting membrane peeling induced functional changes specifically in the perifoveal temporal macula. Distortion in the retinal layers is proposed to underly scotomas pathogenesis.

Predictive value of retinal function by the Purkinje test in patients scheduled for cataract surgery in Kinshasa, DR Congo.

To assess the retinal function in patients with dense cataracts in resource poor settings in Kinshasa, DR Congo. In a tertiary eye care center, the Purkinje entoptic test was performed as part of the ophthalmological examination in 98 eyes in patients with cataract, using a penlight. Totally 92 cataract patients including 86 patients suffered from unilateral and 6 from bilateral cataracts were included in the study. The investigator asked the patients about their perception of the vascular pattern most commonly described as a leafless or dead tree. Visual acuity≥6/60 was considered an indication of good visual function and visual acuity<6/60 of poor function. Following small incision cataract surgery, best corrected visual acuity (BCVA) was determined and compared with the preoperative findings. Out of 98 eyes, there were 80 (81.6%) where the Purkinje entoptic phenomenon was reported by the patients. After cataract surgery, out of the 80 eyes, there were 75 (93.8%) with a BCVA of better than 6/60, whereas in 5 eyes (6.2%), BCVA was 6/60 or less. Out of the 18 eyes (18.4%) where no Purkinje tree was recognized, there were 14 (77.8%) with BCVA of better than 6/60, whereas in 4 (22.2%) BCVA was 6/60 or less. The Purkinje entoptic test is successfully used for preoperative assessment of retinal function in patients with dense cataract. However, further investigation and refinement of the test is necessary to validate the method for use in sub-Saharan conditions.

Complete evaluation of retinal function in Major Depressive Disorder: From central slowdown to hyperactive periphery

BackgroundDeveloping easy-to-access biomarkers is crucial in Major Depressive Disorder. The retina has already been suggested as relevant. However, there is a need for a global and local assessment of whole retinal function using a reproducible, standardized protocol allowing for comparison across studies. Our aim is to assess whole retinal function in patients with actual unipolar Major Depressive Episode (MDE) using pattern, flash and multifocal electroretinogram (ERG) according to the International Society for Clinical Electrophysiology of Vision standardized protocols. MethodsWe assessed retinal function in 14 males and females with MDE, diagnosed based on the Diagnostic and Statistical Manual of Mental Disorders, and in age- and sex-matched healthy controls. ResultsComparing the patients with the controls, we observed the following using multifocal ERG: a significant increase in N1 peak time in ring 3 and a decrease in P1 amplitude in ring 2; using pattern ERG: a significant increase in P50 peak time; using flash ERG: a decrease in a- and b-wave peak time and an increase in the b-wave amplitude in dark-adapted 3.0, a decrease in a- and b-wave peak time and an increase in both wave amplitudes in light-adapted 3.0, and a decrease in the b-wave peak time in light-adapted flicker. LimitationsSample size. Contribution of pharmacological treatments to the outcomes cannot be formally excluded. ConclusionsPatients with MDE exhibit delayed signaling in the central retina and hyperreactivity to light in the periphery. Central retinal function may be a marker of psychomotor retardation and cognitive impairment in MDE.

Long-Term Investigation of Retinal Function in Patients with Achromatopsia

PurposeTo investigate the long-term natural history of retinal function of achromatopsia (ACHM).MethodsSubjects with molecularly confirmed ACHM were recruited in a prospective cohort study of mesopic microperimetry. Coefficient of repeatability and intraclass correlation coefficient (ICC) of mean sensitivity (MS) were calculated. Best-corrected visual acuity (BCVA), bivariate contour ellipse area (BCEA), contrast sensitivity (CS), MS, total volume (VTOT), and central field volume (V5°) from volumetric and topographic analyses were acquired. Correlation of functional parameters with structural findings from optical coherence tomography (OCT) was performed.ResultsEighteen subjects were recruited. Mean follow-up was 7.2 years. The MS test–retest repeatability coefficient was 1.65 decibels (dB), and the ICC was 0.973 (95% confidence interval, 0.837–0.98). Mean MS was similar for right and left eyes (16.97dB and 17.14dB, respectively). A negative significant correlation between logMAR BCVA and the retinal sensitivity indices (MS, VTOT, V5°) was found. A significant negative correlation between logCS and MS, VTOT, and V5° was also observed. BCVA and BCEA improved during follow-up. Mean CS, MS, VTOT, and V5° at final follow-up were similar to baseline. MS was similar between CNGA3- and CNGB3-ACHM. Patients with and without the presence of a foveal ellipsoid zone on OCT had similar MS (16.64 dB and 17.17 dB, respectively).ConclusionsWe demonstrate a highly reproducible assessment of MS. Retinal function including MS, volumetric indices, and CS are stable in ACHM. Improvement of fixation stability and small changes of BCVA over time may be part of the natural history of the disease.

In vivo Genome Editing for Vision

Background & Aim Leber congenital amaurosis (LCA), one of the leading causes of childhood-onset blindness, is caused by autosomal recessive mutations in several genes including RPE65, resulting in retinal degeneration. For the recovery of retinal function in patients with LCA, it is required to repair the gene to replace intact protein and to restore physiological responses in the retinal tissues. In this study, we investigated the therapeutic potential of RPE65 replacement through CRISPR/Cas9-mediated therapeutic genome editing including homology-directed repair and in-frame deletion in rd12 mice, a model of human LCA, which have a homozygous nonsense mutation in the Rpe65 gene. Methods, Results & Conclusion Through subretinal delivery of the dual adeno-associated virus system containing transcripts for CRISPR/Cas9 and donor DNA, we achieved >1% homology-directed repair and ∼1.6% deletion of the pathogenic stop codon in Rpe65 gene in retinal pigment epithelial tissues of rd12 mice. This treatment resulted in ∼10% of Rpe65 mRNA expression and 20-30% of dark-adapted electroretinographic responses in rd12 mice at seven months after the injection. There was no definite evidence of histologic abnormalities or tumorigenesis related to potential off-target effects during seven months of follow-up periods. Taken together, we suggest the results of the therapeutic genome editing of a Rpe65 nonsense mutation by CRISPR/Cas9, providing new insight for developing therapeutics for LCA.

Vascular Density and Retinal Function in Patients with Retinitis Pigmentosa Evaluated by Swept-Source OCT Angiography and Microperimetry

Purpose: To determine the retinal and choroidal vessel density in the macular area with swept-source optical coherence tomography (OCT) angiography (SS-OCTA) in patients affected by retinitis pigmentosa (RP), to compare their data with those on healthy subjects, and to study a possible morphofunctional correlation by microperimetry (MP-1). Methods: A total of 40 eyes of 40 patients affected by RP and 24 eyes of 24 healthy subjects were included in the study. Manually moving down the segmentation line of the SS-OCTA, we have evaluated the vessel density for the superficial retinal plexus, deep retinal plexus, choriocapillaris, and three levels of the choroid. Results: Linear regression analyses were performed of the retinal structure and function. No significant correlation was detected in any case (R² = 0, p > 0.05). A comparison between RP and healthy controls revealed a significant reduction in SS-OCTA mean capillary density in the RP group (p = 0.0011). This relationship was consistent across vascular layers (p = 0.2413). A significant association between the capillary density of the various vascular complexes was detected within individual eyes (p < 0.0001). Conclusions: This study represents the first comparing MP-1 and SS-OCTA data in the largest cohort of patients. RP patients showed a reduction in both the retinal and the choroidal vascular network in the macular area compared to healthy subjects.

Determinants of Cone and Rod Functions in Geographic Atrophy: AI-Based Structure-Function Correlation

To investigate the association between retinal microstructure and cone and rod function in geographic atrophy (GA) secondary to age-related macular degeneration (AMD) by using artificial intelligence (AI) algorithms. Prospective, observational case series. A total of 41 eyes of 41 patients (75.8 ± 8.4 years old; 22 females) from a tertiary referral hospital were included. Mesopic, dark-adapted (DA) cyan and red sensitivities were assessed by using fundus-controlled perimetry ("microperimetry"); and retinal microstructure was assessed by using spectral-domain optical-coherence-tomography (SD-OCT), fundus autofluorescence (FAF), and near-infrared-reflectance (IR) imaging. Layer thicknesses and intensities and FAF and IR intensities were extracted for each test point. The cross-validated mean absolute error (MAE) was evaluated for random forest-based predictions of retinal sensitivity with and without patient-specific training data and percentage of increased mean-squared error (%IncMSE) as measurement of feature importance. Retinal sensitivity was predicted with a MAE of 4.64 dB for mesopic, 4.89 dB for DA cyan, and 4.40 dB for DA red testing in the absence of patient-specific data. Partial addition of patient-specific sensitivity data to the training sets decreased the MAE to 2.89 dB, 2.86 dB, and 2.77 dB. For all 3 types of testing, the outer nuclear layer thickness constituted the most important predictive feature (35.0, 42.22, and 53.74 %IncMSE). Spatially resolved mapping of "inferred sensitivity" revealed regions with differential degrees of mesopic and DA cyan sensitivity loss outside of the GA lesions. "Inferred sensitivity" accurately reflected retinal function in patients with GA. Mapping of "inferred sensitivity" could facilitate monitoring of disease progression and serve as "quasi functional" surrogate outcome in clinical trials, especially in consideration of retinal regions beyond areas of GA.

Multifocal electroretinogram changes in patients with retinal vein occlusion

Background Retinal vein occlusion (RVO) is one of the most common vascular retinal disorders that lead to macular edema. There is often discrepancy between visual prognosis and optical coherence tomography (OCT) changes. Multifocal electroretinogram (mfERG) provides useful information about retinal function in affected parts of the retina in patients with RVO.Purpose The aim of this study was to assess the functional changes in patients with RVO by mfERG and to correlate these changes with OCT findings in those patients.Patients and methods This is a prospective study of 30 eyes of patients with RVO, with their fellow eyes being taken as control. Thirteen eyes with central RVO, 12 eyes with branch RVO, and five eyes with hemi-RVO were included in the study. OCT to measure the central macular thickness was done in all patients. mfERG was also done in all patients. P wave amplitude and p wave implicit times were measured in the central area and all quadrants.Results mfERG responses were markedly affected in all quadrants in central RVO, in the affected quadrants in branch RVO, and in the affected hemiretina in hemi-RVO. The differences between the p amplitude and p implicit time between the affected eyes and the fellow eyes were statistically significant in all patients. There was a significant correlation between the central macular thickness and p amplitude in the affected half of the retina in hemi-RVO.Conclusion mfERG is a sensitive tool for assessment of retinal function in patients with RVO. mfERG can assess local retinal dysfunction in patients with RVO, which is sensitive to morphological changes detected by OCT.

Chromatic Full-Field Stimulus Threshold and Pupillography as Functional Markers for Late-Stage, Early-Onset Retinitis Pigmentosa Caused by CRB1 Mutations.

PurposeMutations in the CRB1 gene cause early-onset retinal degeneration (EORD). Clinical disease progression markers, such as visual fields or electrophysiology, are not reliably measurable in most patients to follow the retinal function in patients with CRB1-mutations.MethodsTen patients (five females, five males; age 22–56 years) with EORD caused by CRB1 mutations were examined in a cross-sectional manner using best corrected visual acuity (BCVA), perimetry, full-field and multifocal electroretinography, full-field stimulus threshold (FST), and pupillography to red and blue light. Disease duration was defined as the difference between the age at the first symptoms to the age at examination in years.ResultsBCVA was quantifiable in six patients and ranged from light perception to 20/50. The visual field was measurable only in three patients who had the shortest disease duration. Full-field and multifocal electroretinography were not measurable in any patient. FST to blue and red light were measurable in all patients except the one with the longest disease duration; the thresholds ranged from −16.7 to 1.5 dB for red light and from −40.2 to 2.5 dB for blue light (0 dB = 0.01 cd.s/m2) and showed correlations with disease duration (r = 0.87 for blue, r = 0.65 for red, r = 0.8 for blue–red difference). The maximal relative pupil constriction amplitude (MRA) showed low or no correlations with disease duration (r = −0.55 for blue, r = −0.3 for red light); the blue–red difference in the post-illumination pupil responses (PIPR) showed no correlation with disease duration (r = −0.05). Compared to healthy eyes, the MRA to red and blue light was significantly decreased (P < 0.001) and the blue–red PIPR difference was significantly increased (P = 0.003).ConclusionsFST features a valid clinical marker in late-stage early-onset retinitis pigmentosa caused by CRB1 mutations correlating with disease duration. This indicates the potential as a progression marker of disease. The pupil responses to full-field chromatic stimuli show significant differences from the normal population: the remaining responses, although reduced, indicate a partially preserved inner retinal function despite severe photoreceptor dysfunction.Translational RelevanceThe functional measurements presented in this study present a valid clinical progression marker in late-stage early onset retinitis pigmentosa caused by biallelic CRB1 mutations. Additionally, they can be used as outcome measures for safety and efficacy in clinical therapy trials.

Retinal Function in Patients with Adalimumab Treatment: Long-term Follow-up with Microperimetry

ABSTRACT Purpose: To examine the effects of a biological treatment (adalimumab) on visual function in patients with ankylosing spondylitis and in uveitic patients without macular edema during one-year treatment with adalimumab. Methods: Sixteen eyes of eight consecutive Caucasian patients treated with adalimumab were followed up using microperimetry (MAIA; CenterVue, Padova, Italy). Five patients had ankylosing spondylitis without uveitis, three patients had panuveitis without macular edema. Macular sensitivity and macular integrity were recorded. Results: During six-month follow-up, the average threshold did not change significantly (p = .649). Macular integrity was stable (p = .225). The macular sensitivity point analysis showed no significant effects (examination F(3,56) = 0.494 and p = .688; point*examination F(108,2016) = 0.688 and p = .994) during the follow-up. Conclusions: During one-year follow-up, adalimumab did not affect macular function, unlike the well-established maculopathy induced by hydroxychloroquine. Microperimetry may be considered when following-up macular function in patients undertaking adalimumab.

Correlation between Choriocapillaris Density and Retinal Sensitivity in Stargardt Disease.

The aim of this work was to characterize the choriocapillaris (CC) in patients with Stargardt disease (STGD) using the swept source widefield optical coherence tomography angiography (SS WF OCTA) and to compare CC perfusion density to retinal sensitivity, analyzed using microperimetry (MP). This cross-sectional study included 9 patients (18 eyes) with STGD and central CC atrophy (stage 3 STGD). The CC was analyzed using SS WF OCTA and areas of different CC impairment were quantified and correlated with retinal sensitivity analyzed using MP. The main outcome measures were the percent perfused choriocapillaris area (PPCA), retinal sensitivity, and correlation between PPCA and retinal sensitivity. Seventeen eyes of 9 patients suffering from stage 3 STGD were analyzed. SS WF OCTA revealed a vascular rarefaction in central atrophic zones and a near atrophy halo of choriocapillaris impairment. In all eyes were noticed a central atrophy (CA) area with absolute absence of CC that corresponded to 0 dB points at MP, a near atrophy (NA) zone of PPCA impairment that included points with decreased sensitivity at MP and a distant from atrophy (DA) zone with higher PPCA and retinal sensitivity values. The mean difference of PPCA and retinal sensitivity between NA and CA and DA and CA was statistical significantly different (p < 0.01), the latter showing higher values. A direct relationship between PPCA and retinal sensitivity was found (p < 0.001). Choriocapillaris damage evaluated using SS WF OCTA correlates with MP, these data suggest that CC impairment may be a predictor of retinal function in patients with STGD.

Long-term treatment with 9-cis-β-carotene rich alga Dunaliella bardawil ameliorates photoreceptor degeneration in a mouse model of retinoid cycle defect

The retinoid cycle is the enzymatic pathway that regenerates the vision chromophore, 11-cis retinal, after it is bleached during light absorption. Genetic insults to this cycle result in incurable blinding retinal dystrophies such as retinitis pigmentosa and Leber congenital amaurosis. Previous studies demonstrated that oral treatment with 9-cis-β-carotene rich Dunaliella bardawil powder significantly improved visual and retinal function in patients with fundus albipunctatus night blindness and retinitis pigmentosa. Here we examined the effect of oral treatment with the Dunaliella powder on retinal function and structure in RPE65rd12 mice, a model of a genetic defect in the retinoid cycle. Mice were fed with a control diet, vitamin A deficient diet (VAD) or VAD diet supplemented with Dunaliella powder for 13 months. Mice fed with Dunaliella presented significantly higher dark-adapted (35.7 μV ± 3.1 vs. 6.9 μV ± 2.5, p < 0.001 for VAD and vs. 4.3 μV ± 1.1, p < 0.001 for control) and light-adapted (35.1 μV ± 4.3 vs. 6.2 μV ± 3.0, p < 0.001 for VAD and vs. 4.9 μV ± 1.3, p < 0.001 for control) maximal electroretinogram a-wave amplitudes. The Dunaliella group also presented higher dark- and light-adapted maximal electroretinogram b-wave amplitudes compared with the control diet (86.5 μV ± 9.4 vs. 28.7 μV ± 6.3, p < 0.001 and 79.2 μV ± 10.4 vs. 28.3 μV ± 4.7, p = 0.001, respectively), but comparable results to the VAD group. A significantly higher number of M-cone photoreceptors was identified in the retinas of DUNA treated mice. Taken together, our study suggests that 9CBC-rich Dunaliella bardawil powder may present an effective treatment for retinal dystrophies caused by defects in the retinoid cycle.

Prolonged Cone b-Wave on Electroretinography Is Associated with Severity of Inflammation in Noninfectious Uveitis

This study sought to investigate retinal function in patients with noninfectious uveitis by using full-field electroretinography (ERG) and correlate the ERG to disease duration and severity of inflammation. Prospective cohort study. Patients (n= 200) with noninfectious uveitis and a disease duration of <1 year (group A [n= 80]) and those with a disease duration of >5 years (group B [n= 120]) were included. ERGs of the total 355 uveitis eyes were measured according to an extended International Society for Clinical Electrophysiology of Vision protocol. ERG abnormalities were related to demographics and uveitis characteristics, including anatomical classification, duration of uveitis, severity of inflammation, best corrected visual acuity (BCVA), cystoid macular edema (CME), and the highest fluorescein angiography (FA) score during the course of the disease. ERGs showed abnormalities in 235 eyes (66.2%). The most frequent and pronounced ERG abnormality was a prolonged implicit time of the cone b-wave (151 eyes [42.5%]), which was associated with vitritis (P= .005); cells in the anterior chamber (P=.007); the highest fluorescein angiography score (P= .011); age (P < 0.001); and pupil diameter (P < 0.001). BCVA was relatively good (0.05 logMAR [interquartile range {IQR}, 0.011, 0.22]) and not associated with this ERG abnormality. There were no differences between the ERG abnormalities in group A and those in group B and no significant associations between ERG abnormalities and anatomical classification or specific diagnoses. ERG results are frequently affected in cases of noninfectious uveitis of all anatomic subtypes, including anterior uveitis without apparent inflammation of the posterior segment. ERG abnormalities appear tobeassociated with the severity of inflammation fromboth the present and the past and therefore may be seen even when signs of retinal inflammation have disappeared.

Relationship of mean retinal sensitivity with vision-related quality of life in patients with idiopathic macular hole

Objective To evaluate the relationship of retinal sensitivity (MS) with vision-related quality of life in patients with idiopathic macular hole (IMH). Methods A prospective cohort study was performed in this study.Thirty-one monocular IMH patients were enrolled in Beijing Tongren Hospital from May to September of 2017.Vision-related quality of life (VR-QoL) was analyzed in 31 patients with National Eye Institute 25-Item Visual Function Questionnaire (NEI VFQ-25) (Chinese version), and MS in the affected eye and the fellow eye in these patients was detected by Microperimeter-3.MS and best corrected visual acuity (BCVA) between the affected eye and the fellow eye were compared.The correlations among BCVA, age, MS, minimum macular hole diameter, composite scores and subscales score were evatuateds.This study protocol was approved by Ethic Committee of Beijing Tongren Hospital.This study complied with Declaration of Helsinki. Results There were significant correlations between composite scores and MS in the affected eye and the fellow eye (affected eye: ρ=0.484, P=0.006; fellow eye: ρ=0.387, P=0.031). Significante correlations were shown in near activities (affected eye: ρ=-0.353, P=0.048; fellow eye: ρ=0.352, P=0.048) and distance activities (affected eye: ρ=0.496, P=0.004; fellow eye: ρ=0.415, P=0.015). No obvious correlations were shown between VR-QoL and BCVA or minimum hole diameter.The correlation between composite scores and the MS in the affected eye was the most strong. Conclusions MS can reflect the retinal function in patients of IMH more widely.The correlations VR-QoL and MS is correlated in the affected eye and fellow eye in patients of monocular IMH, however, patient's VR-QoL dependes more on the affected eye.IMH can violate the patient's near and distance activities. Key words: Macular hole idiopathic; Retinal sensitivity; Vision-related quality of life

STRUCTURAL-FUNCTIONAL CORRELATION IN PATIENTS WITH DIABETIC MACULAR EDEMA.

Previous studies have shown that patients with diabetic macular edema (DME) with relatively good visual acuity can have slow reading speed. The aim of this study was to evaluate the structural-functional correlation in a cohort of patients with DME and to assess whether the central four retinal points on microperimetry (MP4) could be used as a potentially faster and more reliable method of assessing retinal function in patients with DME than reading speed. The study was performed on patients with clinically significant DME. The best-corrected visual acuity (BCVA) was recorded with letter counting on a modified ETDRS chart, the maximal reading speed (MRS) was recorded with MNREAD, the retinal sensitivity (MP28 and MP4) was measured with Optos OCT/ Scanning Laser Ophthalmoscopy and the central subfield thickness was measured by Heidelberg Spectralis Spectral Domain Optical Coherent Topography. Of 100 eyes analyzed, 76 eyes were included in the study. The mean BCVA was 76.5 letters (Snellen equivalent 6/18), the mean MRS was 156.8 words per minute, the mean MP4 was 9.81 dB per point, and the mean central subfield thickness was 309.3 microns. It was found that faster MRS is correlated with younger age (P = 0.001), better BCVA (P < 0.0001), and better retinal sensitivity (P < 0.0001) for both MP28 and MP4, but not with central subfield thickness (P = 0.66). Central subfield thickness is correlated with MP28 (P = 0.05) but not with age (P = 0.812), BCVA (P = 0.113), or MP4 (P = 0.485). After correction for age and BCVA, MRS is still correlated with MP28 (P = 0.001) and MP4 (P = 0.015). Patients with DME can have reduced reading speed despite good visual acuity. Maximal reading speed is often reported to be difficult to perform, inconsistent, and affected by language and educational level. However, in this study, the authors found that the central MP4 points are quick and easy to test in most of the patients, and are highly correlated with MRS. Microperimetry might therefore represent a useful additional functional test that could be considered better than BCVA or reading speed in quantifying visual function in patients with DME.

Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype.

To analyze the clinical features, visual acuity, and full-field electroretinogram (ERG) findings of 15 patients with the neuronal ceroid lipofuscinosis (NCL) phenotype and to establish the role of ERG testing in NCL diagnosis. The medical records of five patients with infantile NCL, five with Jansky-Bielschowsky disease, and five with juvenile NCL who underwent full-field ERG testing were retrospectively analyzed. Progressive vision loss was the initial symptom in 66.7% of patients and was isolated or associated with ataxia, epilepsy, and neurodevelopmental involution. Epilepsy was present in 93.3% of patients, of whom 86.6% presented with neurodevelopmental involution. Fundus findings ranged from normal to pigmentary/atrophic abnormalities. Cone-rod, rod-cone, and both types of dysfunction were observed in six, one, and eight patients, respectively. In our study, all patients with the NCL phenotype had abnormal ERG findings, and the majority exhibited both cone-rod and rod-cone dysfunction. We conclude that ERG is a valuable tool for the characterization of visual dysfunction in patients with the NCL phenotype and is useful for diagnosis.

The effect of cilostazol on electrophysiologic changes in non-proliferative diabetic retinopathy patients.

To evaluate the effects of cilostazol, an antiplatelet and vasodilation agent, on the retinal function of patients with non-proliferative diabetic retinopathy (NPDR) using a full-field electroretinogram (ffERG). A total of 20 eyes from 20 patients were enrolled as the cilostazol-treated group, and 16 eyes from 16 patients were enrolled as the control group to assess the functional effects of cilostazol. Ophthalmologic examinations including fundus fluorescein angiography (FFA), fundus color photography, optical coherence tomography (OCT), and ffERG responses were recorded at baseline and after 1year of cilostazol treatment. The number of microaneurysms on FFA, the number of exudates on fundus photographs, and central macular thickness (CMT) on OCT were compared between the two groups. Recording of ffERG was also performed at baseline and repeated after 1year of treatment. The mean implicit times and amplitudes of a- and b-waves in each ffERG response were analyzed to evaluate the retinal function. CMT and the numbers of microaneurysms and exudates showed no significant change in the cilostazol-treated group. There was no significant change in ffERG parameters between baseline and 1year after the treatment in each group. The mean changes in implicit times from the cilostazol-treated group were significantly less than in the control group in b-waves from dark-adapted 3 ERG (p=0.017) and 10 ERG responses (p=0.047). On the other hand, the mean changes in amplitudes were not significant after 1year of cilostazol treatment, but there were slight increases in amplitudes of dark-adapted 0.01 ERG and 10 ERG in the cilostazol-treated group. These results suggest that cilostazol administration could reduce the implicit times of ffERG in patients with NPDR. It may be beneficial to preserve the retinal function in the diabetic retina, and additional research with larger populations and extended duration are needed to clarify the efficacy and safety of cilostazol for these patients.

Evaluation of cone function by a handheld non-mydriatic flicker electroretinogram device.

PurposeFull-field electroretinograms (ERGs) are used to evaluate retinal function in patients with various types of hereditary and acquired retinal diseases. However, ERG recordings require relatively invasive procedures, including pupillary dilation and the use of contact lens electrodes. Thus, it would be helpful to have a simpler and noninvasive screening method. The purpose of this study was to determine whether a new, handheld, portable ERG device, RETeval™, can be used to screen patients for cone dysfunction.Patients and methodsThirty-five eyes of 35 patients who had reduced cone responses ascertained by a conventional ERG system using contact lens electrodes were studied. The causative diseases included achromatopsia, cone dystrophy, cone-rod dystrophy, retinitis pigmentosa, choroidal dystrophy, autoimmune retinopathy, and Stargardt disease. The flicker ERGs were recorded with the RETeval™ under undilated conditions with skin electrodes (stimulus strength, 3.0 cd·s/m2; frequency, 28.3 Hz), and the responses were compared to that of 50 healthy eyes. The amplitudes and implicit times of the fundamental component of the flicker ERGs were analyzed in three age groups: Group A, ≤20 years; Group B, 21–40 years; and Group C, ≥41 years.ResultsIn all of the age groups, the amplitudes of the ERGs were significantly smaller and the implicit times significantly longer in patients with cone dysfunction than in the control eyes. All but one of the patients had flicker amplitudes lower than the mean −2.0 standard deviation of control eyes.ConclusionThe RETeval™ has a potential of being used to screen for cone dysfunction. The entire examination takes <5 minutes and does not require pupil dilatation or a contact lens electrode. Although the flicker responses do not provide information on the scotopic functions, the RETeval™ device can be used to determine which patients require additional full-field ERG testing with dilated pupils under both scotopic and photopic conditions.