Long-term follow-up of a young male who developed acute macular neuroretinopathy following COVID-19 vaccination.

This report presents the clinical findings and prognosis of a healthy male patient who developed acute macular neuroretinopathy after COVID-19 vaccination. Abnormal findings improved about 1 month after the onset and disappeared 3 months later. The subjective symptoms disappeared in 3 months, and no recurrence was observed for 1 year.

Three-Year Follow-Up of Results of Intraocular Lens Fixation in Patients with Retinitis Pigmentosa.

This is a retrospective, consecutive, noncomparative case series of 6 eyes of 5 retinitis pigmentosa (RP) patients who had undergone pars plana vitrectomy (PPV) and intraocular lens (IOL) implantation. The aim of this case series was to report the long-term outcomes of PPV with IOL implantation in patients with RP). The surgical procedures, visual function, refractive error, corneal endothelial cell density, intraocular pressure, and retinal morphology were evaluated before and 3 years after the surgery. Six eyes of 5 RP patients that had undergone PPV and IOL implantation with or without suturing for lens dislocation were studied. The visual acuity was maintained or improved at 3 years after surgery in all 6 eyes. No intraoperative complications occurred in any of the cases. The mean deviation of the Humphrey Field Analyzer 10-2 program and the retinal morphology evaluated by optical coherence tomography did not show any abnormal changes before and after surgery. In two eyes, the postoperative refractive error was more myopic than the attempted refractive error. In conclusion, PPV with IOL implantation can be performed safely in RP patients, and the long-term visual acuity can be maintained.

Efficacy and safety of transdermal electrical stimulation in patients with nonarteritic anterior ischemic optic neuropathy.

BACKGROUND: No effective treatment for NAION with strong evidence has been established till date. The aim of this investigator-led, prospective, non-randomized, open-label, uncontrolled multi-center exploratory clinical trial is to evaluate the efficacy and safety of transdermal electrical stimulation (TdES) using skin electrodes in patients with NAION. METHODS: Five patients with monocular NAION underwent TdES (10-ms biphasic pulses, 1.0 mA, 20 Hz, 30 min) of the affected eye six times at 2-week intervals. The primary endpoint was the logarithm of the mini-mum angle of resolution (logMAR) visual acuity at 12 weeks compared with 0 weeks. The secondary endpoints were changes in the best-corrected logMAR visual acuity, Early Treatment of Diabetic Retinopathy Study (ETDRS) visual acuity, and mean deviation (MD) of the Humphrey field analyzer (HFA) 10-2 and HFA Esterman test scores. Additionally, the safety of TdES was evaluated. RESULTS: LogMAR visual acuity improved by ≥ 0.1 in two eyes, and ETDRS visual acu-ity improved by ≥ 5 characters in one eye. The mean change in logMAR visual acuity from week 0 showed an increasing trend. The mean MD of HFA 10-2 showed no obvious change, while HFA Esterman score improved in four eyes. All patients completed the study according to the protocol, and no treatment-related adverse events were observed. CONCLUSIONS: TdES treatment may have improved visual acuity and visual field in some patients. Further sham-controlled study in larger cohort is needed on its effectiveness. TRIAL REGISTRATION: UMIN, UMIN000036220. Registered 15 March, 2019, https://center6.umin.ac.jp/cgi-open-bin/ctr/ctr_view.cgi?recptno=R000041261 .

Visual Evoked Potentials for the Detection of Diabetic Retinal Neuropathy.

Visual evoked potentials (VEP) are visually evoked signals that extract electroencephalographic activity in the visual cortex that can detect retinal ganglion cells, optic nerves, chiasmal and retrochiasmal dysfunction, including optic radiations, and the occipital cortex. Because diabetes causes diabetic retinopathy due to microangiopathy and neuropathy due to metabolic abnormalities and intraneural blood flow disorders, assessment of diabetic visual pathway impairment using VEP has been attempted. In this review, evidence on the attempts to assess the visual pathway dysfunction due to abnormal blood glucose levels using VEP is presented. Previous studies have provided significant evidence that VEP can functionally detect antecedent neuropathy before fundus examination. The detailed correlations between VEP waveforms and disease duration, HbA1c, glycemic control, and short-term increases and decreases in blood glucose levels are evaluated. VEP may be useful for predicting postoperative prognosis and evaluating visual function before surgery for diabetic retinopathy. Further controlled studies with larger cohorts are needed to establish a more detailed relationship between diabetes mellitus and VEP.

Estimation of Visual Function Using Deep Learning From Ultra-Widefield Fundus Images of Eyes With Retinitis Pigmentosa.

Importance: There is no widespread effective treatment to halt the progression of retinitis pigmentosa. Consequently, adequate assessment and estimation of residual visual function are important clinically. Objective: To examine whether deep learning can accurately estimate the visual function of patients with retinitis pigmentosa by using ultra-widefield fundus images obtained on concurrent visits. Design, Setting, and Participants: Data for this multicenter, retrospective, cross-sectional study were collected between January 1, 2012, and December 31, 2018. This study included 695 consecutive patients with retinitis pigmentosa who were examined at 5 institutions. Each of the 3 types of input images-ultra-widefield pseudocolor images, ultra-widefield fundus autofluorescence images, and both ultra-widefield pseudocolor and fundus autofluorescence images-was paired with 1 of the 31 types of ensemble models constructed from 5 deep learning models (Visual Geometry Group-16, Residual Network-50, InceptionV3, DenseNet121, and EfficientNetB0). We used 848, 212, and 214 images for the training, validation, and testing data, respectively. All data from 1 institution were used for the independent testing data. Data analysis was performed from June 7, 2021, to December 5, 2022. Main Outcomes and Measures: The mean deviation on the Humphrey field analyzer, central retinal sensitivity, and best-corrected visual acuity were estimated. The image type-ensemble model combination that yielded the smallest mean absolute error was defined as the model with the best estimation accuracy. After removal of the bias of including both eyes with the generalized linear mixed model, correlations between the actual values of the testing data and the estimated values by the best accuracy model were examined by calculating standardized regression coefficients and P values. Results: The study included 1274 eyes of 695 patients. A total of 385 patients were female (55.4%), and the mean (SD) age was 53.9 (17.2) years. Among the 3 types of images, the model using ultra-widefield fundus autofluorescence images alone provided the best estimation accuracy for mean deviation, central sensitivity, and visual acuity. Standardized regression coefficients were 0.684 (95% CI, 0.567-0.802) for the mean deviation estimation, 0.697 (95% CI, 0.590-0.804) for the central sensitivity estimation, and 0.309 (95% CI, 0.187-0.430) for the visual acuity estimation (all P < .001). Conclusions and Relevance: Results of this study suggest that the visual function estimation in patients with retinitis pigmentosa from ultra-widefield fundus autofluorescence images using deep learning might help assess disease progression objectively. Findings also suggest that deep learning models might monitor the progression of retinitis pigmentosa efficiently during follow-up.

Exploratory clinical trial to evaluate the efficacy and safety of transdermal electrical stimulation in patients with central retinal artery occlusion.

PURPOSE: To evaluate the efficacy and safety of transdermal electrical stimulation (TdES) using skin electrodes in patients with central retinal artery occlusion (CRAO). METHODS: Five eyes of five patients with CRAO underwent TdES (10-ms biphasic pulses, 20 Hz, 30 min) six times at 2-week intervals. Only the affected eye was stimulated with 1.0-mA pulses in all patients. The primary endpoint was the best-corrected logMAR visual acuity. The secondary endpoints were changes in the best-corrected logMAR visual acuity, Early Treatment of Diabetic Retinopathy Study (ETDRS) visual acuity, mean deviation of the Humphrey field analyzer (HFA) 10-2, and HFA Esterman test score. We also evaluated its safety. RESULTS: The logMAR visual acuity at 12 weeks was improved by 0.1 or more in two patients and was maintained in two patients compared to the baseline. No obvious changes in the mean logMAR visual acuity, ETDRS visual acuity, mean deviation, and HFA Esterman score were observed at 12 weeks compared to the baseline. All five enrolled patients completed the study according to the protocol. No treatment-related adverse events were observed during this study. CONCLUSION: In this study, logMAR visual acuity was slightly improved in two patients, confirming the safety of TdES. Since CRAO has no established treatment method, further research into the effects of TdES treatment in CRAO patients may be beneficial.

Optical coherence tomography findings in three patients with Werner syndrome.

BACKGROUND: Werner syndrome is a rare, autosomal recessive disorder characterised by premature aging. It is a typical hereditary progeroid syndrome that can be difficult to diagnose owing to its rarity and the similarity of some of its symptoms, such as juvenile cataracts, to other common ophthalmologic conditions. Early onset of bilateral cataracts is currently used as the ophthalmological feature for Werner syndrome; however, ophthalmologists often find performing a detailed examination of the medical history and genetic testing for Werner syndrome at the time of an ophthalmologic consultation challenging. If a unique ocular finding was observed on ocular examinations in cases of juvenile bilateral cataracts, we could consider Werner syndrome as a differential diagnosis.  CASE PRESENTATION: We documented the cases of three patients with Werner syndrome in whom thinning of the retina in the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) were observed using optical coherence tomography (OCT). Visual field tests revealed the loss of visual field mainly owing to glaucoma. The thinnig of the choroidal thickness (CT) in three patients was also observed using enhanced depth imaging (EDI)-OCT. CONCLUSIONS: Three patients have thinning of the RNFL, GCC, and choroidal thickness and the loss of visual field. These findings suggest the need for including Werner syndrome in the differential diagnosis when patients presenting with juvenile cataracts of unknown cause also show abnormal retinal and choroidal thinning in the OCT images.

Genetic characterization of 1210 Japanese pedigrees with inherited retinal diseases by whole-exome sequencing.

Inherited retinal diseases (IRDs) comprise a phenotypically and genetically heterogeneous group of ocular disorders that cause visual loss via progressive retinal degeneration. Here, we report the genetic characterization of 1210 IRD pedigrees enrolled through the Japan Eye Genetic Consortium and analyzed by whole exome sequencing. The most common phenotype was retinitis pigmentosa (RP, 43%), followed by macular dystrophy/cone- or cone-rod dystrophy (MD/CORD, 13%). In total, 67 causal genes were identified in 37% (448/1210) of the pedigrees. The first and second most frequently mutated genes were EYS and RP1, associated primarily with autosomal recessive (ar) RP, and RP and arMD/CORD, respectively. Examinations of variant frequency in total and by phenotype showed high accountability of a frequent EYS missense variant (c.2528G>A). In addition to the two known EYS founder mutations (c.4957dupA and c.8805C>G) of arRP, we observed a frequent RP1 variant (c.5797C>T) in patients with arMD/CORD.

Progression Rate of Visual Function and Affecting Factors at Different Stages of Retinitis Pigmentosa.

We reviewed medical records of 121 patients/235 eyes of typical retinitis pigmentosa (RP) patients who could be followed up for at least 5 years with the aim of investigating the long-term course of visual function progression at each RP stage and appropriate assessment methods. Patients were classified into three groups: mild RP (baseline mean deviation (MD) ≥ -5), moderate RP (-25 < baseline MD < -5), and late RP (baseline MD ≤ -25). Linear mixed-effect models were used to follow MD, the average retinal sensitivity of the central four points of the Humphrey field analyzer 10-2 program (S4), and visual acuity (VA) with increasing time. The associations among factors (baseline MD group, sex, hereditary form) and the interaction between each factor and time were also investigated. The mean reduction of the MD, S4, and VA for all patients was -0.37 dB/year, -0.25 dB/year, and 0.018/year, respectively. The moderate RP group had a faster progression than other groups in MD (-0.43 dB/year, p < 0.05). The moderate (-0.31 dB/year, p = 0.01) and late RP groups (-0.25 dB/year, p < 0.01) had faster progression than the mild RP group in S4. The late RP group had faster progression in VA than the other groups (0.03/year, p < 0.05). Females had a slower progression of the S4 (-0.15 dB/year, p = 0.02) and VA (0.01/year, p < 0.001) than males. The autosomal dominant group had a slower progression than the sporadic group in MD (-0.22 dB/year, p = 0.02); the autosomal dominant and autosomal recessive groups had a slower VA decline than the sporadic group (0.01/year, p = 0.03; 0.01/year, p = 0.04). Because the progression rates of VA and visual field test differed as per the RP stage, S4 and VA can also be useful assessment methods depending on the stage. Inheritance form and sex may affect the progression rate.

Long-term follow-up of retinal morphology and physiology after 2000 mg sildenafil overdose as a means of attempted suicide: a case report.

BACKGROUND: Few case reports have described sildenafil overdose, particularly ingestion of > 1000 mg, and overdose-induced changes in visual function remain unclear. We report retinal morphology, retinal sensitivity, and findings of electrophysiological evaluation over long-term follow-up in a case of sildenafil overdose (2000 mg). CASE PRESENTATION: Our patient developed visual abnormalities in the paracentral visual field accompanied by photophobia, decreased contrast sensitivity, and difficulty distinguishing colors in both eyes, 8 hours after the sildenafil overdose. These symptoms did not improve throughout the course, and although abnormalities of retinal morphology and sensitivity, as well as the electroretinogram findings showed slight improvement, the patient did not recover completely at 6-month follow-up. CONCLUSIONS: We observed that high-dose sildenafil ingestion leads to retinal toxicity; the ocular abnormalities may persist for at least 6 months. Optical coherence tomography, Humphrey perimetry, microperimetry, and multifocal electroretinography are useful to quantitatively monitor temporal changes.

Evaluating the efficacy and safety of transdermal electrical stimulation on the visual functions of patients with retinitis pigmentosa: a clinical trial protocol for a prospective, multicentre, randomised, double-masked and sham-controlled design (ePICO trial).

INTRODUCTION: Previously, we conducted a clinical trial to evaluate the safety and efficacy of transdermal electrical stimulation (TdES) with skin electrodes to improve the visual functions in patients with retinitis pigmentosa (RP). No adverse events were related to the treatment during follow-up examinations, and TdES significantly improved the mean visual acuity and visual field sensitivity. METHODS AND ANALYSIS: We developed a study protocol for a prospective, multicentre, randomised, double-masked and sham-controlled clinical trial, planned to commence on June 2021. We intend to compare the maintenance or improvement in best-corrected visual acuity, and safety of TdES using skin electrodes between patients with RP and the sham group. The primary endpoint comprises the superiority of the logarithm of the minimum angle of resolution (logMAR) visual acuity change at week 24 from baseline in the treatment and sham groups. Secondary endpoints involve the comparison of the treatment and sham groups at week 24 for the logMAR visual acuity, early treatment diabetic retinopathy study visual acuity, the mean deviation value of Humphrey field analyser 10-2, monocular Humphrey Esterman visual field test score, ellipsoid zone length, central foveal thickness and 25-item National Eye Institute Visual Function Questionnaire score. We intend to enrol 50 patients from three Japanese institutions within 1 year and follow them up for 1 years. ETHICS AND DISSEMINATION: The protocol was approved by the institutional review board at the Chiba University Hospital and two other institutions, and was registered with the Japan Registry of Clinical Trials on 17 May 2021. The trial will be conducted in accordance with the principles of the Declaration of Helsinki, and is in accordance with Good Clinical Practice standards. The findings will be published in a peer-reviewed journal. TRIAL REGISTRATION NUMBER: JRCT2032210094.

The Impact of Cataract Surgery on Contrast Visual Acuity and Retinal Sensitivity in Patients with Retinitis Pigmentosa.

PURPOSE: To determine the effects of cataract surgery on contrast visual acuity and retinal sensitivity in patients with retinitis pigmentosa. METHODS: Retinal sensitivity and contrast visual acuity were determined by microperimetry (MAIA) and contrast sensitivity acuity tester (CAT-CP), respectively, before and after cataract surgery. The significance of the correlations between visual acuity, retinal sensitivity, contrast visual acuity, improvements after surgery, and macular structure before and after cataract surgery was determined. RESULTS: Retinal sensitivity and contrast visual acuity were significantly improved after cataract surgery. The correlations among postoperative visual acuity, postoperative retinal sensitivities, and preoperative ellipsoid zone length were significant. The postoperative retinal sensitivity of the central 10° and the ellipsoid zone length was particularly significantly correlated. Preoperative contrast visual acuity and the amount of improvement and preoperative retinal sensitivity and the amount of improvement were significantly negatively correlated. The contrast visual acuity under both the 100% and 10% photopic and mesopic conditions improved significantly after cataract surgery. CONCLUSIONS: Cataract surgery in retinitis pigmentosa patients with preserved ellipsoid zones significantly improved retinal sensitivity and contrast visual acuity. Cataract surgery can be expected to improve retinal sensitivity and contrast visual acuity under various conditions, even if preoperative visual parameters are low, as long as the ellipsoid zone is preserved.

Effects of Cataract Surgery on Vision-Related Quality of Life in Patients with Retinitis Pigmentosa and the Predictive Factors of Quality of Life Improvement.

PURPOSE: To determine the effects of cataract surgery and preoperative factors on the vision-related quality of life (QOL) in patients with retinitis pigmentosa (RP). MATERIALS AND METHODS: This was a prospective, interventional study of 54 patients diagnosed with RP. The 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25) was used to determine the QOL before and after the cataract surgery. The correlations between the scores of the questionnaire and the best-corrected visual acuity (BCVA), macular structure, and degree of improvement of the NEI VFQ-25 scores were also determined. RESULTS: Statistically significant improvements were observed in the BCVA and all of the NEI VFQ-25 subscale scores except for color vision. The improvement of general vision was the largest. The postoperative BCVA of the better-seeing eye was more strongly and significantly correlated with the postoperative NEI VFQ-25 scores than that of the worse-seeing eye. All of the postoperative NEI VFQ-25 scores were significantly correlated with the length of the ellipsoid zone (EZ) of the photoreceptors. No significant correlation was found between the preoperative general vision, near vision, mental health scores, and EZ length. All of the preoperative NEI VFQ-25 scores except the social function and mental health scores were negatively and significantly correlated with the degree of improvement of the NEI VFQ-25 score. The EZ length was significantly correlated with the degree of improvement of the NEI VFQ-25 scores of the general vision, distance vision, mental health, dependency, and composite 9 scores. CONCLUSIONS: Cataract surgery can significantly improve the NEI VFQ-25 scores in RP patients. The EZ length can be used to predict the postoperative VFQ scores. We conclude that the NEI VFQ-25 is a useful method to evaluate the impact of cataract surgery on the BCVA in patients with RP.

Refractive errors after sutureless intrascleral fixation of intraocular lens.

PURPOSE: To determine the difference between the attempted and the achieved postoperative refractive error after sutureless intrascleral fixation of the haptics of an implanted intraocular lens (IOL). PATIENTS AND METHODS: This was a retrospective study of the medical charts of patients who had undergone sutureless intrascleral fixation of an IOL. The IOLs were fixed by inserting the haptics into scleral tunnels with or without flanges. The differences between the attempted and achieved postoperative refractive error (spherical equivalent) were determined. The relationships between the surgical options and the differences of the refractive error and degree of astigmatism were also determined. RESULTS: Two hundred and twenty-three eyes were studied. There was a myopic shift of - 0.38 ± 1.13 diopters (D). The mean of the differences between the achieved postoperative and the attempted refractive error was 0.89 ± 0.79 D. The achieved refractive error was significantly correlated with the attempted refractive error (P < 0.001, R2 = 0.631). The surgical procedures selected were not significantly associated with the differences between the attempted and achieved refractive error. The size of sclerocorneal incision and presence of sutures were significantly associated with the final degree of astigmatism (P = 0.006 and 0.008, respectively). CONCLUSIONS: The postoperative refractive error was significantly correlated with the attempted refractive error after intrascleral fixation of an IOL. The wound construction was associated with the postoperative degree of astigmatism.

Characteristics and surgical outcomes of rhegmatogenous retinal detachments that develop after intravitreal injections.

PURPOSE: To determine the frequency and characteristics of rhegmatogenous retinal detachments (RRDs) that develop after an intravitreal injection of anti-vascular endothelial growth factor (VEGF) agent. STUDY DESIGN: A retrospective review of the medical charts. METHODS: The charts of patients who received intravitreal injections for age-related macular degeneration (AMD), diabetic macular edema (DME), retinal vein occlusion (RVO), or myopic choroidal neovascularization (mCNV) between 2013 and 2020 were reviewed. We included the RRD cases that developed within 90 days of the most recent intravitreal injection. The baseline characteristics and surgical outcomes were analyzed. RESULTS: A total of 3040 patients received 28,190 intravitreal injections. Seven eyes of 7 cases developed a RRD. There were 6 cases of AMD and one of DME, with an incidence of one in 4027 injections (0.025%). The retinal break was in the superior quadrants in 5 eyes (71%), and in the inferior quadrants in 2 eyes. All eyes had a posterior vitreous detachment. The average number of injections before the development of RRD was 14.1 (range: 2-39). Four eyes were treated by vitrectomy, and 3 by scleral buckling. The primary success rate was 86%, and the final reattachment rate was 100%. The best-corrected visual acuity was 0.41 ± 0.26 logarithm of minimal angle of resolution (logMAR) units before developing the RRD, 0.78 ± 0.78 logMAR units before the surgery for RRD, and 0.45 ± 0.47 logMAR units at the final visit. CONCLUSIONS: The incidence of RRD after an intravitreal injection is very low (0.025%), and it can be reattached with recovery of the visual acuity.

Comparisons of surgical outcomes after epiretinal membrane peeling in the myopic eyes with long to that with normal axial length.

PURPOSE: To compare the outcomes of vitrectomy with removal of an idiopathic epiretinal membrane (ERM) in the myopic eyes with long axial length (AL) to that in the eyes with normal AL. METHODS: This was a retrospective, observational, case-control study. Fifty-six eyes of 56 patients with an idiopathic ERM were studied. Twenty-eight of these eyes had an axial length longer than 26.0 mm (Group A), and the other 28 eyes had axial lengths < 26.0 mm (Group B). The age and visual acuity of the two groups were not significantly different. All subjects were treated by vitrectomy and peeling of the ERM with a 25- or 27-gauge system. The postoperative best-corrected visual acuity (BCVA) and optical coherence tomographic findings were determined at 3 and 6 months postoperatively. RESULTS: The mean BCVA improved from 0.35 ± 0.25 to 0.15 ± 0.25 logMAR units in the eyes with the long AL and from 0.35 ± 0.25 to 0.10 ± 0.21 logMAR units in the eyes with normal AL at 6 months postoperatively (both P < 0.001). The postoperative BCVA was not significantly different between the two groups at 6 months (P = 0.221). The central macula was thinner in the eyes of Group A than Group B (P < 0.001). The restorations of the ellipsoid zone and the external limiting membrane were observed in both groups, but the interdigitation zone was less frequently restored in Group A (P < 0.001). CONCLUSIONS: Vitrectomy with the removal of the epiretinal membrane is effective in attaining a good BCVA even in the myopic eyes with long AL.

Ocular syphilis with optic disc neovascularization treated with bevacizumab evaluated by OCT angiography and electroretinography.

We present our findings in an atypical case of ocular syphilis with optic disc neovascularization that was treated with intravitreal bevacizumab and followed by multimodal imaging and electroretinography. A 29-year-old man presented with a chief complaint of night blindness of one-year duration. Our initial examination showed that an optic disc neovascularization was present, and the optical coherence tomographic (OCT) images showed a reduction in the length of the ellipsoid zone of both eyes. Fluorescein angiography showed leakage from the optic disc neovascular tissue, and the presence of nonperfused areas in the peripheral retina. Blood test was strongly positive for syphilis. He was administered oral amoxicillin and prednisolone. He was also treated with an intravitreal injection of bevacizumab which led to a rapid suppression of the neovascularization. However, panretinal photocoagulation had to be performed because OCT angiography and fluorescein angiography detected residual neovascularization. Although these treatments suppressed the activity of the ocular syphilis, electrophysiological improvements were not seen even 1 year after the initial treatment. OCT angiography and electroretinogram are useful techniques for monitoring the effectiveness of the treatments.

Two cases with retinitis pigmentosa that developed severe retinal atrophy long after vitreo-retinal surgery.

PURPOSE: To report two cases with retinitis pigmentosa (RP) who underwent vitreo-retinal surgery and developed severe retinal atrophy. OBSERVATIONS: Case 1 was a 36-year old man who underwent 20-gauge pars plana vitrectomy (PPV) for a macular hole (MH), and Case 2 was a 71-year-old man who also underwent 20-gauge PPV for an epiretinal membrane (ERM). During 13 years follow-up period, severe retinal atrophy developed near the macula that progressed rapidly and the best-corrected visual acuity (BCVA) was reduced to 1.1 logarithm of the minimum angle of resolution (logMAR) units in the MH case and to no light perception in the ERM case. CONCLUSIONS AND IMPORTANCE: An unexpected severe retinal atrophy can develop long after vitreo-retinal surgery in RP patients.

Non-paraneoplastic autoimmune retinopathy that developed in fellow eye 10 years after onset in first eye: a case report.

BACKGROUND: Evidence-based criteria for the treatment of autoimmune retinopathy (AIR) have not been established. The pathology and clinical features of each antibody causing AIR, and its long-term course are still undetermined. We report our findings in a case of non-paraneoplastic AIR (npAIR) that developed in the fellow eye 10 years after the onset in the first eye. CASE PRESENTATION: Our patient had photophobia in both eyes and a rapidly progressing visual field defect in his right eye at the initial examination. He was diagnosed with non-paraneoplastic AIR based on the clinical findings and immunoblot analyses for anti-retinal antibodies, and he was treated with steroids. Ten years later, a visual field defect developed in the fellow eye, and a diagnosis of npAIR was made. Immunoblot analyses were positive for anti-α-enolase antibodies. He was treated with steroids, immunosuppressants, and plasma exchange. However, the response to the treatment was poor and both eyes eventually became blind. CONCLUSIONS: As best we know, this is the first case report of npAIR that developed in the fellow eye over 10 years after the development in the first eye. Long-term follow-up and a search for tumor lesions are necessary in cases of npAIR. Further understanding of the long-term course of AIR can contribute to an understanding of the pathology and treatment of npAIR.