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Purpose: To document vascular changes in eyes with post-fever retinitis (PFR) pre and post treatment demonstrated using optical coherence tomography angiography (OCTA)., Methods: This is a retrospective observational case series wherein patients with PFR were retrospectively evaluated for changes in the retinal vasculature during the course of disease using OCTA., Results: At presentation, OCTA revealed flow void areas in superficial and deep capillary plexus (SCP and DCP) corresponding to the areas of retinitis. Post treatment, OCTA showed a significant decrease in the flow void areas with the appearance of new capillary network in both SCP and DCP. The optical coherence tomography also demonstrated normalization of retinal architecture over time. It is speculated that the good visual outcome in PFR could be attributed to the normalization of retinal architecture and remodelling in retinal vasculature., Conclusion: OCTA being non-invasive can be used to understand and quantify the extent of vascular remodelling in PFR.

Purpose: To study the choroidal thickness (CT) and central macular thickness (CMT) in post-fever retinitis (PFR) and their correlation with visual acuity and treatment., Methods: A retrospective, observational study of patients presenting with PFR from 2013 to 2021 and with spectral domain optical coherence tomography (SD-OCT) (Heidelberg®, SpectralisTM, Heidelberg, Germany) images were included. The CT and CMT were measured at presentation and at the final visit. The CT was measured subfoveally and at points 2000 µm superior, inferior, medial, and lateral from the fovea using the caliper tool., Results: Seventy-nine eyes of 65 patients were included for this study. The mean age was 39.03 (±16.00) years with female preponderance of 53.84% (n = 35). Mean follow-up duration was 30 days. Mean CT at presentation and at follow-up was 254.12 µm and 241.51 µm, respectively. CT was decreased in majority of the eyes 67.1% (n = 53) from their baseline value. Mean CMTs at presentation and final visit were 454.8 µm and 223.7 µm, respectively. Best corrected visual acuity had a positive correlation with CMT (r = 0.340; P = 0.002) and negligible correlation with CT. A significant decrease in the mean CT was noted in patients who received doxycycline either alone or in combination with a steroid as compared to those who did not receive any treatment (P < 0.001). The significance of which is unknown presently., Conclusion: CMT has a greater role in determining the final visual outcome than CT. CT can be reduced post-treatment with no effect on vision., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Objective: We documented an older female with Coronavirus(CoV) Disease 2019 (COVID-19) and concomitant acquired monocular blindness. We examined this phenomenon in order to understand COVID-19 better., Methods: We observed an older female with COVID-19 and concomitant acquired monocular blindness. The following indicators were monitored during the course of the disease: ocular examinations, flash visual evoked potential examination, a blood test for COVID-19 IgM antibodies, as well as nasopharyngeal swab and tear sample tests for COVID-19 nucleic acid., Results: The patient's visual acuity for the left eye was NLP and the intraocular pressure was 51 mmHg. Keratic precipitates similar to mutton-fat were spread over the corneal endothelium of the left eye. The funduscopic examination of the patient's left eye revealed severe retinal arterial ischemia, and the color of the retina was off-white. Compared to the right eye, the flash visual evoked potential examination revealed a moderate decrease in P2 wave amplitude for the left eye. A blood test was positive for COVID-19 IgM antibodies, and a nasopharyngeal swab test taken for COVID-19 nucleic acid was positive on May 4, 2020. A sample of the patient's tears was taken, and the nucleic acid test for COVID-19 was still positive two weeks later., Conclusions: Our study was the first to find that acute viral retinitis could occur in patients with COVID-19 and severe blindness could be associated with SARS-CoV-2 infection. Therefore, physicians should consider the possibility of coronavirus infection in patients with an abnormal fundus or suddenly vision loss.

Purpose : To study treatment outcomes with and without oral corticosteroids in epidemic retinitis (ER). Method : A retrospective, observational study of 35 eyes of 29 patients diagnosed as ER. Days taken for resolution of macular edema and retinitis lesions were compared in patients treated with oral antibiotics (Group 1) and with corticosteroids-antibiotics combination (Group 2). Result : Eighteen eyes of 14 patients and 17 eyes of 15 patients formed Groups 1 and 2, respectively. At the presentation, mean best-corrected visual acuity (BCVA) was 40 and 44 letters and mean central macular thickness was 648 (±243) and 626 (±256) microns in Groups 1 and 2, respectively. Macular edema resolved in 30.83 and 31.94 days; retinitis lesions resolved in 36.71 and 41.41 days in Groups 1 and 2, respectively. BCVA improved to 74 and 77 letters in Groups 1 and 2, respectively. Conclusion : ER with macular edema can be well managed without corticosteroids.

N-Methyl-D-aspartate (NMDA)-induced neuronal cell death is involved in a large spectrum of diseases affecting the brain and the retina such as Alzheimer's disease and diabetic retinopathy. Associated neurological impairments may result from the inhibition of neuronal plasticity by Nogo-A. The objective of the current study was to determine the contribution of Nogo-A to NMDA excitotoxicity in the mouse retina. We observed that Nogo-A is upregulated in the mouse vitreous during NMDA-induced inflammation. Intraocular injection of a function-blocking antibody specific to Nogo-A (11C7) was carried out 2 days after NMDA-induced injury. This treatment significantly enhanced visual function recovery in injured animals. Strikingly, the expression of potent pro-inflammatory molecules was downregulated by 11C7, among which TNFα was the most durably decreased cytokine in microglia/macrophages. Additional analyses suggest that TNFα downregulation may stem from cofilin inactivation in microglia/macrophages. 11C7 also limited gliosis presumably via P.Stat3 downregulation. Diabetic retinopathy was associated with increased levels of Nogo-A in the eyes of donors. In summary, our results reveal that Nogo-A-targeting antibody can stimulate visual recovery after retinal injury and that Nogo-A is a potent modulator of excitotoxicity-induced neuroinflammation. These data may be used to design treatments against inflammatory eye diseases.

Angiotensin II and aldosterone are the main effectors of the renin-angiotensin aldosterone system (RAAS) and have a central role in hypertension as well as cardiovascular and renal disease. The localization of RAAS components within the retina has led to studies investigating the roles of angiotensin II, aldosterone and the counter regulatory arm of the pathway in vision-threatening retinopathies. This review will provide a brief overview of RAAS components as well as the vascular pathology that develops in the retinal diseases, retinopathy of prematurity, diabetic retinopathy and neovascular age-related macular degeneration. The review will discuss pre-clinical and clinical evidence that modulation of the RAAS alters the development of vasculopathy and inflammation in the aforementioned retinopathies, as well as the emerging role of aldosterone and the mineralocorticoid receptor in central serous chorioretinopathy., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Diabetic retinopathy (DR) and retinopathy of prematurity (ROP) present two examples of proliferative retinopathy, characterized by the same stages of progression; ischemia of the retinal vessels, leads to hypoxia and to correct the problem there is the setting up of uncontrolled angiogenesis, which subsequently causes blindness or even detachment of the retina. The difference is the following; that DR initiated by the metabolic complications that are due to hyperglycemia, and ROP is induced by overexposure of the neonatal retina to oxygen. In this review, we will demonstrate the physiopathological mechanism of the two forms of proliferative retinopathy DR and ROP, in particular the role of the CD40/CD40L axis and IL-1 on vascular complications and onset of inflammation of the retina, the implications of their effects on the onset of pathogenic angiogenesis, thus understanding the link between platelets and retinal ischemia. In addition, what are the therapeutic targets that could slow its progression?, (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

[Full article available at http://rimed.org/rimedicaljournal-2019-03.asp].

We have reported the gateway reflex, which describes specific neural activations that regulate immune cell gateways at specific blood vessels in the central nervous system (CNS). Four types of gateway reflexes exist, all of which induce alterations in endothelial cells at specific vessels of the blood-brain barrier followed by inflammation in the CNS in the presence of CNS-autoreactive T cells. Here we report a new gateway reflex that suppresses the development of retinal inflammation by using an autoreactive T cell-mediated ocular inflammation model. Exposure to photopic light down-regulated the adrenoceptor pathway to attenuate ocular inflammation by suppressing breaching of the blood-retina barrier. Mechanistic analysis showed that exposure to photopic light down-regulates the expression of α 1A -adrenoceptor (α 1A AR) due to high levels of norepinephrine and epinephrine, subsequently suppressing inflammation. Surgical ablation of the superior cervical ganglion (SCG) did not negate the protective effect of photopic light, suggesting the involvement of retinal noradrenergic neurons rather than sympathetic neurons from the SCG. Blockade of α 1A AR signaling under mesopic light recapitulated the protective effect of photopic light. Thus, targeting regional adrenoceptor signaling might represent a novel therapeutic strategy for autoimmune diseases including those that affect organs separated by barriers such as the CNS and eyes.

Autophagy is a lysosomal degradation process that maintains cellular homeostasis by removing dysfunctional organelles and unfolded proteins. Increasing evidence has shown that autophagy proteins are involved in retinal physiology and pathology and that defective autophagy contributes to retinal degeneration. In retinal diseases, autophagy plays a dual role: promoting retinal cell survival and death. Autophagy at a normal level helps retinal cells defend themselves against harmful stress; however, excessive autophagy results in retinal deterioration. Both synergistic and antagonistic roles of autophagy and apoptosis in the retina have been reported in the literature. In this review, we summarize the roles of autophagy in the development of the retina and retinal diseases. This review highlights the importance of autophagy in retinal diseases, and targeting autophagy may provide a new therapeutic approach for retinal diseases., (© 2018 S. Karger AG, Basel.)

Changes in permeability of retinal blood vessels contribute to macular edema and the pathophysiology of numerous ocular diseases, including diabetic retinopathy, retinal vein occlusions, and macular degeneration. Vascular endothelial growth factor (VEGF) induces retinal permeability and macular thickening in these diseases. However, inflammatory agents, such as tumor necrosis factor-α (TNF-α), also may drive vascular permeability, specifically in patients unresponsive to anti-VEGF therapy. Recent evidence suggests VEGF and TNF-α induce permeability through distinct mechanisms; however, both require the activation of atypical protein kinase C (aPKC). We provide evidence, using genetic mouse models and therapeutic intervention with small molecules, that inhibition of aPKC prevented or reduced vascular permeability in animal models of retinal inflammation. Expression of a kinase-dead aPKC transgene, driven by a vascular and hematopoietic restricted promoter, reduced retinal vascular permeability in an ischemia-reperfusion model of retinal injury. This effect was recapitulated with a small-molecule inhibitor of aPKC. Expression of the kinase-dead aPKC transgene dramatically reduced the expression of inflammatory factors and blocked the attraction of inflammatory monocytes and granulocytes after ischemic injury. Coinjection of VEGF with TNF-α was sufficient to induce permeability, edema, and retinal inflammation, and treatment with an aPKC inhibitor prevented VEGF/TNF-α-induced permeability. These data suggest that aPKC contributes to inflammation-driven retinal vascular pathology and may be an attractive target for therapeutic intervention., (Copyright © 2018 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.)

Chikungunya is a systemic viral disease transmitted to humans by infected mosquitoes in endemic areas of Africa, Asia and more recently in the Americas. Chikungunya infection produces a sudden onset of fever, joint pains and erythematous skin eruption. A plethora of ocular manifestations have been described ranging from a non-specific conjunctivitis to exudative retinal detachment. Ocular chikungunya seems to respond well to corticosteroid therapy, and outcomes are usually better if treated early. Our patient acquired this infection on a travel to Mexico jungle. This was confirmed by ReverseTranscriptase-PCR test once she returned to the UK. The peculiarity of the case is the inordinate delay of almost a year in the onset of eye symptoms, from acquiring the viral infection. The ocular inflammation responded to systemic corticosteroid therapy with a favourable visual outcome. She developed a recurrence many months later which again responded well to a course of oral steroids., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Severe macular oedema causing marked loss of vision is seen in cases of retinitis developing postviral fever. The use of antivascular endothelial growth factor agents for macular oedema and submacular fluid secondary to viral retinitis has not been studied or well established in the past. We report a case series of two patients of postviral retinitis with severe macular oedema resistant to steroid therapy, treated with intravitreal bevacizumab. The patients showed significant symptomatic improvement in the visual acuity. The retinitis lesions resolved slowly and macular oedema regressed. Bevacizumab appears to be a safe and useful agent to manage macular oedema subsequent to postviral retinitis. An early resolution of macular oedema helps in the preservation of visual acuity which left untreated can cause severe visual loss., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Diffuse unilateral subacute neuroretinitis (DUSN) is caused by a subretinal live and mobile nematode. Acute phase: Patients usually present with severe pain, decreased vision, vitritis/papillitis, and tracks of grayish-white lesions-and a live nematode. Late phase: Arterial narrowing, optic atrophy, diffuse disruption of the retinal pigment epithelium (RPE), with severe visual loss.

Purpose: To accurately evaluate the autoimmune inflammation, we aim to develop three quantitative measurements to monitor the inflammatory changes in the retina: retinal-choroidal thickness, major retinal vessel diameter, and electroretinography amplitudes., Methods: During a 21-day experimental period, eyes were examined by confocal scanning laser ophthalmoscopy, optical coherence tomography, fundus fluorescein angiography, and electroretinography in living mice, which were then subsequently killed for histologic assessments., Results: On day 21 postimmunization, inflammation was observed both in vivo and in vitro. Fold change of retinal-choroidal thickness and major retinal vessel diameter in experimental autoimmune uveoretinitis mice were significantly greater than controls (P < 0.001). Both scotopic and photopic electroretinography amplitudes were significantly attenuated when compared with control mice (P < 0.01). Our results showed that these three quantifiable indicators provided an objective and accurate evaluation of autoimmune inflammation, which are in good correlations with the reported clinical and histopathologic scoring systems (P < 0.05)., Conclusions: These three indicators will be useful to detect the small but significant differences in the severity of experimental autoimmune uveoretinitis for future longitudinally therapeutic studies.

Purpose: To describe the clinical characteristics, macular structure and function, and to document sequential changes over 5 years in a 10-year-old boy with bilateral primary foveomacular retinitis., Methods: A 10-year-old boy presented with sudden onset scotoma in both eyes, experienced after getting up from bed on a non-eclipse day. He persistently denied direct sun-gazing. He neither had any significant systemic illness, nor was using any medications. In addition to a detailed examination at presentation that included fundus fluorescein angiogram (FFA), electroretinogram (ERG), pattern ERG and electrooculogram (EOG), he was examined periodically for 5 years with Humphrey visual field (HVF), spectral domain optical coherence tomogram (SDOCT), Amsler grid charting and multifocal ERG. The macular structure and functions were analyzed over the years and correlated with the symptoms., Results: All findings were bilaterally symmetrical at each visit. At presentation, his corrected visual acuity was 20/25 with subfoveal yellow dot similar to solar retinopathy, central scotoma with reduced foveal threshold in HVF 24-2, micropsia in Amsler grid, missing of two plates on Ishihara color vision chart, transfoveal full thickness hyper-reflective band on SD OCT, unremarkable FFA and normal foveal peak in mfERG. The flash ERG and EOG were unremarkable. A month later, his VA improved to 20/20, he had relative scotoma in Amsler grid, no scotoma in HVF (10-2), restoration of the inner segment of the photoreceptors with sharp defect involving ellipsoid and photoreceptor interdigitation zone in SDOCT and blunting of foveal peaks in mfERG. Three months later, his corrected VA was 20/20 with relative scotoma in Amsler grid, normal color vision, no scotoma in HVF 10-2 and unchanged SDOCT findings. In subsequent examinations at 6, 9, 14, 29, 39 and 60 months, he was symptomless with VA 20/20, unremarkable fundus, normal Amsler grid and HVF (normal foveal threshold), unchanged SDOCT findings and the reduced foveal peaks on mfERG in both eyes got normalized only at 60 months., Conclusion: Presented here is a case of bilaterally symmetrical idiopathic foveomacular retinitis that had a clinical appearance similar to solar retinopathy. The fundus changes persisted for 4 weeks, the symptoms and changes in Amsler grid lasted for 3 months, and the foveal threshold in visual fields normalized within 3 months. Maximum change in the SDOCT defect occurred within a month, and the extrafoveal defect in the ellipsoid and photoreceptor interdigitation line persisted despite resolution of symptoms and resolution of the visual field defect and normal distance vision. Probably, the foveal lesion detected on SDOCT was too small to cause a reduction in the distance visual acuity or show up in the visual field and mfERG later.

Purpose: To evaluate the features of acute retinal pigment epitheliitis (ARPE) at onset and in the course of recovery by serial spectral-domain optical coherence tomography (SD OCT) and the correlation to visual acuity (VA)., Design: Retrospective cohort study., Participants: Consecutive patients with ARPE., Methods: A review of medical records was performed., Main Outcome Measures: Integrity of SD OCT retinal bands at onset and in the course of disease, time required to achieve each retinal band restoration, corresponding VA change, and final VA., Results: Four patients were included. Initial SD OCT showed a dome-shaped hyper-reflective lesion at the photoreceptor outer segment layer disrupting the ellipsoid zone (EZ) and interdigitation zone (IZ) (100%). In the early phase, there was also upward displacement of the external limiting membrane (ELM) and mild transient thickening of the retinal pigment epithelium (RPE)/Bruch's complex (Bc). Acute retinal pigment epitheliitis resolved in a sequence of (1) a decrease in height of SD OCT hyper-reflective lesion and the upwardly displaced ELM returning to its normal position with irregularity; (2) complete disappearance of the hyper-reflective lesion; (3) restoration of ELM; (4) restoration of EZ; and (5) restoration of IZ. The average time to restore ELM, EZ, and IZ was 4.3±5.2, 7.3±7.2, and 12.5±12.4 weeks, respectively, and the corresponding logarithm of the minimum angles of resolution (logMAR) VAs were 0.24±0.23, 0.09±0.07, and 0.05±0.06, respectively. Visual acuity improved when IZ was restored., Conclusions: Early SD OCT revealed an inflammatory lesion in the photoreceptor outer segment layer displacing ELM. The RPE was involved only mildly and transiently. Recovery occurred in a sequence of ELM, EZ, and IZ restoration, and VA improved when the IZ was restored. These features suggested that the IZ (i.e., the contact between photoreceptors and RPE) is the primary site of inflammation in ARPE., (Copyright © 2017 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Diabetic macular edema (DME) is caused by blood-retinal barrier breakdown associated with retinal vascular hyperpermeability and inflammation, and it is the major cause of visual dysfunction in diabetic retinopathy. Adrenomedullin (ADM) is an endogenous peptide first identified as a strong vasodilator. ADM is expressed in the eyes and is up-regulated in various eye diseases, although the pathophysiological significance is largely unknown. We investigated the effect of ADM on DME. In Kimba mice, which overexpress human vascular endothelial growth factor in their retinas, the capillary dropout, vascular leakage, and vascular fragility characteristic of diabetic retinopathy were observed. Intravitreal or systemic administration of ADM to Kimba mice ameliorated both the capillary dropout and vascular leakage. Evaluation of the transendothelial electrical resistance and fluorescein isothiocyanate-dextran permeability of an endothelial cell monolayer using TR-iBRB retinal capillary endothelial cells revealed that vascular endothelial growth factor enhanced vascular permeability but that co-administration of ADM suppressed the effect, in part by enhancing tight junction formation between endothelial cells. In addition, a comprehensive PCR array analysis showed that ADM administration suppressed various molecules related to inflammation and NF-κB signaling within retinas. From these results, we suggest that by exerting inhibitory effects on retinal inflammation, vascular permeability, and blood-retinal barrier breakdown, ADM could serve as a novel therapeutic agent for the treatment of DME., (Copyright © 2017 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.)

Purpose: To compare the efficacy of infliximab (IFX) on refractory uveoretinitis between Behçet disease (BD) patients with short duration (≤18 months, n = 6) versus long duration (>18 months, n = 7) of their ocular disease., Methods: Records of 13 BD patients treated with IFX for 24 months were retrospectively reviewed., Results: Frequency of ocular attacks decreased with IFX treatment in both groups, with no significant difference. Retinal and disc vascular leakage scores also decreased in both groups, however was significantly lower in the short-duration group. Percentage of eyes with a best-corrected visual acuity (BCVA) of 1.0 or better at year 2 was 100% in the short-duration and 21% in the long-duration group., Conclusions: Induction of early remission using IFX appeared to be more effective in reducing background vascular leakage and in maintaining good visual acuity in BD patients.

Chronic low grade inflammation is considered to contribute to the development of experimental diabetic retinopathy (DR). We recently demonstrated that lack of CD40 in mice ameliorates the upregulation of inflammatory molecules in the diabetic retina and prevented capillary degeneration, a hallmark of experimental diabetic retinopathy. Herein, we investigated the contribution of CD40 to diabetes-induced reductions in retinal function via the electroretinogram (ERG) to determine if inflammation plays a role in the development of ERG defects associated with diabetes. We demonstrate that diabetic CD40-/- mice are not protected from reduction to the ERG b-wave despite failing to upregulate inflammatory molecules in the retina. Our data therefore supports the hypothesis that retinal dysfunction found in diabetics occurs independent of the induction of inflammatory processes.

Background: Syphilis with ocular involvement has reemerged as a critical health problem. The aim of the present study was to explore the clinical manifestations and cerebrospinal fluid (CSF) status in ocular syphilis in human immunodeficiency virus (HIV)-negative patients., Methods: The clinical records of patients with ocular syphilis presenting to the Shanghai Xuhui Central Hospital in the period from January 2011 to December 2012 were retrospectively reviewed., Results: The median age of 25 HIV-negative patients with ocular syphilis was 53 years, 18 patients (72.0 %) were males and 7 (28.0 %) were females. None of them self-identified themselves as men who had sex with men (MSM). The ocular lesions included: uveitis (13 cases), optic neuropathy (6 cases), retinal vasculitis (5 cases), retinal detachment (3 cases), and neuroretinitis (4 cases). Serum toluidine red unheated serum test (TRUST) titer ranged from 1 to 512, with a median of 64. Overall, 18 (72.0 %) of the 25 patients had abnormal CSF results, 15 (60.0 %) CSF samples had elevated white blood cell counts, 13 (52.0 %) had elevated protein levels, and 9 (36.0 %) had reactive CSF Venereal Disease Research Laboratory (VDRL) test, respectively. Mann-Whitney U tests showed higher serum TRUST titer (>32) correlated with the abnormal CSF results., Conclusions: The demographic characteristics of patients with ocular syphilis in this study were different from previous reports. The study showed a high CSF abnormal rate in HIV-negative patients. The recommendation for CSF examination from all patients with ocular syphilis, including HIV-negative cases, is strongly supported by the present data.

We describe a rare case of Lyme disease complicated by unilateral neuroretinitis in the right eye. We report a case of a 27-year-old woman with blurred vision on her right eye. Because of the suspicion of optic neuritis (multiplex sclerosis) neurological examination was ordered. Surprisingly, computer tomography of the brain revealed incomplete empty sella, which generally results not monocular, but bilateral optic nerve swelling. Opthalmological examination (ophthalmoscopy and optical coherence tomography) indicated not only monocular optic nerve, but retinal oedema next to the temporal part of the right optic disk. Visual evoked potentials (VEP) demonstrated no P100 latency delay and mild differences between the amplitudes of the responses of the left and right eye. Optical coherence tomography (OCT) demonstrated the swelling of the optic nerve head and oedematous retina at the temporal part of the disk. Suspicion of an inflammatory cause of visual disturbance blood tests was ordered. Doxycycline treatment was ordered till the result of the blood test arrived. The Western blot and ELISA test were positive for Borrelia burgdorferi sensu lato. Following one week corticosteroide and ceftriaxone treatments, the patient displayed a clinical improvement. Unilateral neuroretinitis with optic disk swelling due to neuroborreliosis is a rare complication and in many cases it is difficult to distinguish between inflammatory and ischemic lesions. Further difficulty in the diagnosis can occur when intracranial alterations such as empty sella is demonstrated by CT examination.

A 20-year-old man applied with vision loss in the left eye. Right eye examination was unremarkable. Best-corrected visual acuity (BCVA) in the left eye was 20/200. Fundus examination revealed a few yellow spots within a round-shaped macular lesion. Autofluorescence imaging showed hyperautofluorescence in the lesion. Central amplitudes in multifocal electroretinogram (mfERG) were depressed. The patient reported a rhinopharyngitis 7-10 days before the visual loss. The patient was diagnosed as acute retinal pigment epithelitis. BCVA improved gradually up to 20/20 in 4 weeks. mfERG amplitudes returned to normal. A slight pigmentary distortion was the only residual fundus finding.

Purpose: To report novel spectral domain optical coherence tomography and electrophysiologic findings in diffuse unilateral subacute neuroretinitis., Methods: Six patients with a diagnosis of diffuse unilateral subacute neuroretinitis were retrospectively ascertained. All patients had received oral treatment with albendazole; resolution of the inflammatory lesions without subsequent relapse was noted. Spectral domain optical coherence tomography was performed using a Spectralis HRA OCT (Heidelberg Engineering). The inner and outer retinal volumes were calculated for the macular area. The contralateral eyes acted as controls. All six patients underwent standardized full-field electroretinography and pattern electroretinography. Some had multifocal electroretinography., Results: Inner retinal volume significantly differed between affected and control eyes (P < 0.02), but there was no significant difference in outer retinal volume. Electroretinography data showed a mixed pattern of inner and outer retinal dysfunction, with inner retinal dysfunction being greater; reduction in b:a ratio of the scotopic bright flash electroretinography was a consistent observation in those patients (5/6) with generalized retinal dysfunction. Two patients showed definite photoreceptor involvement, with probable involvement in a third. Of the four patients in whom serial data are available, there was definite evidence of progressive inner and outer retinal dysfunction in one patient, with inner retinal dysfunction being greater, and probably in a second patient., Conclusion: The data provide anatomical and functional evidence of both inner and outer retinal dysfunction in diffuse unilateral subacute neuroretinitis, even though the worm is usually assumed to be located in the subretinal space. The mechanism is unclear.

Objective: To determine the role of intravitreal triamcinolone acetonide (IVTA) as an adjuvant in the management of acute retinal necrosis (ARN)., Design: Interventional case series., Participants: Four eyes of 4 patients diagnosed with ARN were studied., Methods: This pilot case series included 4 patients who presented with clinical features suggestive of ARN. IVTA was injected 1 to 2 weeks after initiation of antiviral therapy and concurrent usage of oral corticosteroids. The disease course and clinical outcome were observed. The patients received additional treatment depending on the sequelae of ARN., Results: Signs of reduction in vitritis and resolution of retinitis were observed as early as 1 week after IVTA was administered. In all 4 patients, inflammation promptly resolved with no untoward effects like raised intraocular pressure or worsening of the retinitis. A final visual acuity of 20/40 or better was achieved in 3 of 4 patients after appropriate treatment of ARN-related complications., Conclusions: Intravitreal triamcinolone under antiviral cover could be a useful adjunct to systemic steroids in the treatment of ARN., (Copyright © 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Microglia and Müller cells are cell types that feature prominently in responses to disease and injury in the retina. However, their mutual interactions have not been investigated in detail. Here, we review evidence that indicate that these two cell populations exchange functionally significant signals under uninjured conditions and during retinal inflammation. Under normal conditions, Müller cells constitute a potential source of extracellular ATP that mediates the activity-dependent regulation of microglial dynamic process motility. Following microglial activation in inflammation, microglia can signal to Müller cells, influencing their morphological, molecular, and functional responses. Microglia-Müller cell interactions appear to be a mode of bi-directional communications that help shape the overall injury response in the retina.

Diffuse unilateral subacute neuroretinitis (DUSN) is a form of uveitis that can potentially lead to blindness. In Brazil and other parts of South America, diffuse unilateral subacute neuroretinitis is an important cause of posterior uveitis in children and healthy young adults. If diagnosed and treated in early stage, allows a resolution of symptoms with improvement of visual acuity. If the disease progresses to the late stage, can result in significant visual loss. In this study, through a literature review, we describe the main characteristics of this disease, including the following aspects: history, etiology, physiopathology, clinical features, diagnosis, differential diagnosis and treatment.

Purpose: To describe the determinant clinical signs to diagnose diffuse unilateral subacute neuroretinitis (DUSN) and the main features related to identification of the live worm., Design: Retrospective study performed at the Federal University of Rio Grande do Norte, Rio Grande do Norte, Brazil, between 2003 and 2008., Methods: Patients with early- or late-stage diffuse unilateral subacute neuroretinitis were included. All patients underwent complete ophthalmologic examination and had a minimum of 6 months of follow-up., Results: A total of 121 patients were included. Most patients were younger than 20 years (69.42%). Visual acuity was 20/400 or worse in 86 patients (71.7%). Nine patients (7.43%) presented in the early stage and 112 (92.57%) patients presented in the late stage. Subretinal tracks (91.7%), focal alterations of the retinal pigment epithelium (89.3%), small white spots (80.2%), and optic nerve atrophy (76.9%) were the most frequent clinical features. The subretinal worm was identified in 48 patients (39.66%), and laser treatment to destroy it was performed in all cases. The most common location of the nematode was the posterior pole (21 patients). It was observed that the younger the age, the higher the indices of larvae identification (P=.022). Multifocal yellow-white lesions and vitritis were correlated with identification of the worm (P=.001). Mean logarithm of the minimal angle of resolution visual acuity was 1.466 (20/600) and 1.281 (20/400) before and after laser treatment, respectively (P<.005)., Conclusions: Identification of clinical signs and diagnosis of diffuse unilateral subacute neuroretinitis in its early stage, followed by prompt location and destruction of the worm by photocoagulation, may improve the vision of affected patients., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: To describe the visual recovery after intravitreal injections of the antivascular endothelial growth factor, bevacizumab, in a case of vaso obliteration from idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN). The name IRVAN was given to the condition to highlight the key findings present in the disease. IRVAN is a severe, sight threatening condition that can lead to peripheral capillary non-perfusion and vision loss from the ischemic sequelae of vascular occlusion. Panretinal photocoagulation (PRP) is the current standard of care for IRVAN but visual outcome is poor if PRP is initiated after neovascularization develops. Intravitreal bevacizumab has success at treating neovascularization from other ischemic retinopathies and inflammatory retinal conditions that have similar characteristics to IRVAN., Case Report: This case report describes a patient with decreased vision in the OS. The patient presented with best-corrected visual acuity of 20/20 in the OD and count fingers at 4 ft in the OS. Evaluation revealed findings consistent with an advanced stage of IRVAN. Anterior and posterior neovascularization had developed from extensive capillary non-perfusion in both retinas. A dense vitreous hemorrhage blocked vision OS. Bilateral intravitreal injections of bevacizumab and extensive PRP were given in the area of retinal ischemia for treatment. After 4 months, the patient's vision had improved from count fingers in the OS to 20/40., Conclusions: IRVAN has favorable outcomes when treated with a combination of PRP and intravitreal injections of antivascular endothelial growth factor. This case demonstrates the effectiveness of this combination treatment in a case of IRVAN with both posterior and anterior neovascularization.

Anthocyanin-rich bilberry extract, a plant-derived antioxidant, has been utilized as a popular supplement for ocular health worldwide. However, it is unclear whether this extract has any biological effect on visual function, and the mechanism for such an effect is completely unknown. In this study, we generated a mouse model of endotoxin-induced uveitis (EIU) that shows retinal inflammation, as well as uveitis, by injecting lipopolysaccharide. We pretreated the mice with anthocyanin-rich bilberry extract and analyzed the effect on the retina. Anthocyanin-rich bilberry extract prevented the impairment of photoreceptor cell function, as measured by electroretinogram. At the cellular level, we found that the EIU-associated rhodopsin decreased and the shortening of outer segments in photoreceptor cells were suppressed in the bilberry-extract-treated animals. Moreover, the extract prevented both STAT3 activation, which induces inflammation-related rhodopsin decrease, and the increase in interleukin-6 expression, which activates STAT3. In addition to its anti-inflammatory effect, the anthocyanin-rich bilberry extract ameliorated the intracellular elevation of reactive oxygen species and activated NF-κB, a redox-sensitive transcription factor, in the inflamed retina. Our findings indicate that anthocyanin-rich bilberry extract has a protective effect on visual function during retinal inflammation.

Objective: To report spectral domain optical coherence tomography (SD-OCT) findings in 3 patients with acute retinal pigment epitheliitis (ARPE)., Design: Retrospective chart review., Methods: Charts of three young patients with ARPE were reviewed., Results: In acute stage, SD-OCT demonstrated abnormal hyperreflectivity involving the photoreceptor outer segment layer and hyporeflectivity involving the associated RPE layer in all cases. In chronic stage, SD-OCT showed decreased abnormal reflectivity. In one case with incomplete recovery of visual acuity, disruption of the photoreceptor inner and outer segment (IS/OS) junction was demonstrated in chronic phase., Conclusions: SD-OCT confirmed that the outer segments of foveal photoreceptors and the associated RPE layer were the primary affected sites with ARPE. Detection of integrity of the foveal IS/OS line in resolved ARPE could be helpful in prediction of visual prognosis., (Copyright © 2011 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe the characteristics and clinical course of patients with active posterior syphilitic uveitis evaluated between 1991 and 2009., Methods: Retrospective chart review., Results: Thirteen patients with active posterior syphilitic uveitis were identified at a single institution. All were men, and all with available data were men having sex with men (MSM). Ten of 12 (83%) with available data were HIV positive. Four (31%) had a history of syphilis. Clinical findings included infiltrative retinitis, necrotizing retinitis and optic neuritis. Two of 8 patients tested (25%) had a positive Venereal Disease Research Laboratory test in the cerebrospinal fluid. Treatments included intravenous penicillin, intramuscular penicillin and intramuscular ceftriaxone. All treated cases improved and, in some cases, inflammatory lesions completely resolved without scarring., Conclusions: In this series, syphilitic posterior uveitis presented only in men, and all with available data were MSM. The majority were concomitantly infected with HIV. Clinical presentations varied and all patients demonstrated either significant improvement or complete resolution of inflammation.

Purpose: To describe the macular findings on optical coherence tomography (OCT) in patients with cat-scratch disease (CSD) neuroretinitis., Methods: Medical records of all patients diagnosed with CSD neuroretinitis at the Tel Aviv Medical Center between April 2006 and May 2010 were retrospectively reviewed. All patients underwent Stratus OCT macular examination., Results: Eight eyes of seven patients with confirmed CSD neuroretinitis, (mean age 33 ± 9.9 years, range 6-48 years) were included in the study. All patients presented clinically with optic nerve swelling and macular edema or macular exudates. OCT demonstrated flattening of the foveal contour, thickening of the neurosensory retina, and accumulation of subretinal fluid (SRF) in all studied eyes. Retinal exudates appeared as multiple hyper-reflective foci in the outer plexiform layer. The average central macular thickness was 460 μm (range 170-906 μm) and the average maximal retinal thickness was 613 μm (range 387-1103 μm), at presentation. The macula appeared normal on repeated exams during follow-up., Conclusion: Similar OCT findings were demonstrated in patients with CSD neuroretinitis. SRF was found in all eyes, although was not visible on clinical examination or fluorescein angiography. OCT may be used as an adjunct imaging tool in the diagnosis and follow-up of patients with CSD neuroretinitis.

Purpose: We report our experience in treating 2 patients of idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN) syndrome with antitumor necrosis factor agent, infliximab, who showed a very favorable response to treatment., Methods: Two patients with clinical diagnosis of IRVAN syndrome were included in the study. The visual acuity was affected due to ocular inflammation and presence of macular edema due to exudation around the optic nerve., Results: The patients did not respond to initial treatment with oral steroids, and visual acuity continued to deteriorate due to macular exudation. Infliximab therapy resulted in prompt resolution of the inflammatory reaction and retinal exudation, with improvement in visual acuity, that was subsequently maintained with maintenance therapy. The intravenous infliximab infusions were scheduled at 0, 4, 8, and 12 weeks initially, and every 2 months thereafter. Retinal neovasculariztion in each patient was managed by pan retinal photocoagulation., Conclusion: Infliximab therapy may be useful in reducing inflammation and leakage from the optic nerve in patients with IRVAN syndrome. This may help preserve or improve visual acuity. However, further studies are required to evaluate the long-term benefits of this therapy.

Background: Live intraocular nematode is a rare occurrence. Nematode can migrate actively within the eye, creating visual symptoms and damaging ocular tissue., Case Presentation: A 26-year old man presented with painless reduced vision of the left eye for one week duration. It was associated with floaters. Visual acuity on the left eye was hand movement. Anterior segment examination was normal with normal intra-ocular pressure. Fundus examination showed a live nematode lying subretinally at the macular area with macular oedema and multifocal chorioretinal lesions at peripheral retina. There was no vitritis, vasculitis or any retinal hemorrhage. Systemic examination revealed normal findings and laboratory studies only showed leucocytosis with normal eosinophil count and negative serum toxocara antibody. The diagnosis of introcular nematode with diffuse unilateral subacute neuroretinitis was made. He was treated with oral anti-helminths and a course of oral steroid at a reducing dose. The nematode had died evidenced by its immobility during the treatment and finally disintegrated, leaving macular oedema with mottling appearance and mild hyperpigmentation. Multifocal chorioretinal lesions had also resolved. However despite treatment his visual acuity during follow-up had remained poor., Conclusions: Cases of intraocular nematode, though not commonly encountered, continue to present the ophthalmologist with the problem of diagnosis and management and hence poorer prognosis to the patient.

Purpose: To determine the specific location of the initial lesion in acute retinal pigment epitheliitis., Methods: Four patients diagnosed with acute retinal pigment epitheliitis were studied. Fundus photographs, fluorescein angiography and indocyanine green angiography, and spectral-domain optical coherence tomography findings were reviewed., Results: Four healthy young patients presented with acute onset of unilateral decreased vision. Ophthalmoscopy showed macular pigment mottling with surrounding yellow hypopigmented areas at the level of the retinal pigment epithelium (RPE). Fluorescein angiography revealed transmission hyperfluorescence. Early-phase and midphase indocyanine green angiography images showed a patchy macular hyperfluorescence. At late phase of indocyanine green angiography, a hyperfluorescent halo with a cockadelike appearance of the macular area was observed. Spectral-domain optical coherence tomography showed a disruption of the photoreceptors' inner segment and outer segment interface associated with a wider disruption of the RPE inner band. These disrupted lines were replaced by a dome-shaped highly reflective lesion involving the RPE inner layer, the photoreceptors' inner segment and outer segment layers, and, in two cases, the outer nuclear layer. With time, indocyanine green angiography showed resolution of the observed lesions. Spectral-domain optical coherence tomography showed restored and continuous inner segment and outer segment layers and RPE inner band., Conclusion: Spectral-domain optical coherence tomography findings suggest that the initial lesion in acute retinal pigment epitheliitis is located at the junction between the photoreceptor outer segments and the apical side of the RPE cells. Indocyanine green angiography and spectral-domain optical coherence tomography show that the RPE appears to be more widely involved than the neurosensory retina.

We investigated the case of a young man with blurred vision in his left eye. His visual acuity was slightly decreased, and ophthalmoscopy disclosed a gray-white lesion in the macula. He had no systemic or ocular history. On the visual field test, the threshold sensitivity was decreased in the corresponding region. Spectral domain optical coherence tomography (OCT) demonstrated a disruption in the photoreceptor inner and outer segment (IS/OS) junction and undulation of the retinal pigment epithelium (RPE) with backscattering. We re-examined the patient after two weeks and after three months without any treatment. Visual acuity and visual field results were gradually normalized, and OCT demonstrated the recovery of continuity in the photoreceptor IS/OS junction, as well as decreased RPE irregularity with minimal backscattering. We used spectral domain OCT instead of time domain OCT (OCT3) so that we could provide better image resolution of the acute retinal pigment epitheliitis (ARPE). Finally, we observed recovery of the functional and anatomical changes in the ARPE patient with a resolution of the condition within three months following the initial examination, using OCT and visual field tests.

Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star figure. The underlying pathophysiology involves increased permeability of disc vasculature, but the etiology is not fully defined. In some cases, NR is probably due to an infectious process involving the disc; in others, a postviral or autoimmune mechanism is more likely. Cases can be divided into those in which a specific infectious agent has been identified, those considered idiopathic, and those with recurrent attacks. Some reports have not distinguished among these subgroups, and it is unclear if their clinical features vary. We reviewed the literature and our own patients looking particularly at features that might better distinguish these subtypes. Features common to all 3 groups included age, absence of pain, and fundus appearance. Preceding systemic symptoms were more common in patients with cat scratch disease (CSD) and uncommon in those with recurrence. The pattern and magnitude of visual field loss differed, more commonly confined to the central field in CSD cases and more severe in recurrent cases. Recovery of visual acuity and field was less substantial in recurrent cases even after the initial episode. MRI was usually normal in all 3 groups. Enhancement confined to the optic disc was found in all 3 groups, but enhancement of the retrobulbar optic nerve was seen only in recurrent cases. Findings that are strongly suggestive of CSD include very young age, preceding systemic symptoms, and poor visual acuity but with a small or absent relative afferent pupil defect (RAPD). In contrast, the following are suggestive of idiopathic NR with a high risk of recurrence: absence of systemic symptoms, visual field defect outside the central field, preserved visual acuity with a large RAPD, and poor recovery of vision. Decisions regarding evaluation and treatment should be made with these features in mind.

Purpose: The effects of aging and light exposure on cone photoreceptor survival were compared between mouse retinas of neural retina leucine zipper knockout (Nrl(-/-)) mice and double-knockout mice lacking G-protein-coupled receptor kinase 1 (Nrl(-/-)Grk1(-/-))., Methods: Mice were reared in total darkness, ambient cyclic light, or constant light, and their retinas were evaluated from 1 to 9 months of age using immunohistochemistry, electroretinography, and fluorescein angiography. Retinal gene expression and statistically significant probe sets were categorized using analysis software. Select gene expression changes were confirmed with quantitative RT-PCR., Results: In contrast to retinas from Nrl(-/-), those from Nrl(-/-)Grk1(-/-) exhibit a progressive loss of the outer nuclear layer, retinal physiology deficits, and a higher rate of degeneration with increasing age that is independent of environmental light exposure. Changes in retinal neovascularization occur in the Nrl(-/-)Grk1(-/-) at 1 month, before the onset of significant cone functional deficits. Microarray analyses demonstrate statistically significant changes in transcript levels of more than 400 genes, of which the oncostatin M signaling pathway and the inflammatory disease response network were identified., Conclusions: These data demonstrate that the loss of functional Grk1 on the enhanced S-cone Nrl(-/-) background exacerbates age-related cone dystrophy in a light-independent manner, mediated partly through the inflammatory response pathway and neovascularization. According to these findings, Grk1 helps to maintain a healthy cone environment, and the Nrl(-/-)Grk1(-/-) mouse allows examination of the alternative roles of Grk1 in cone photoreceptor homeostasis.

Introduction: Ocular disorders are useful in the characterisation of the different types of spinocerebellar ataxias (SCA); pigmentary retinitis is an alteration that is specifically associated to SCA type 7 and is characterised by night blindness, sensitivity to glare and progressive narrowing of the visual field., Case Report: A 34-year-old woman with clinical symptoms of progressive ataxia and visual impairment secondary to pigmentary retinitis. The patient had a personal history with an autosomal dominant pattern of a similar disorder in her father and paternal grandmother. In the genetic study she presented a triplet expansion in the SCA type 2 gene. CONCLUSIONS; Although pigmentary retinitis belongs to the SCA type 7 phenotype, our patient presented this retinal disorder, as in other cases of SCA type 2. A genetic study for SCA type 2 must therefore be conducted in patients with a degenerative ataxic clinical picture and who present evidence of pigmentary retinitis.

Purpose: To evaluate the efficacy and safety of infliximab administration in refractory uveoretinitis in Behçet's disease (BD)., Methods: The subjects were 22 consecutive BD patients with refractory uveoretinitis treated with infliximab. Three patients dropped out from the therapy. The other 19 patients received the therapy for 6 months or longer. The efficacy was evaluated by the ocular attacks and best corrected visual acuity at remission in the 19 patients and the safety was evaluated in all 22 patients., Results: The incidence of ocular attacks in the 6 months before and after the infliximab therapy were 3.0+/-2.6 and 0.4+/-1.1, respectively (p<0.0005). Infliximab was very effective in 7 patients where no ocular attacks occurred throughout infliximab therapy, effective in 10 patients where ocular attacks was reduced, and non-effective in 2 patients. The vision was either improved or unchanged in all eyes at the 6th months therapy. Various adverse effects were seen in 14 patients, but non were serious., Conclusions: Infiximab therapy is effective in managing the refractory uveoretinitis in BD, but elucidation of the safety needs more patients and a longer follow-up period.

Cat-scratch disease is a clinical syndrome that usually presents as a self-limiting illness featuring regional lymphadenopathy, fever, and small skin lesions in association with a cat scratch or bite. It is caused by the Gram-negative bacillus Bartonella henselae, which commonly affects children and young adults. Ocular bartonellosis is the most common atypical manifestation of cat-scratch disease. It can present with a wide spectrum of ocular diseases including neuroretinitis, Parinaud's oculoglandular syndrome, and other forms of intra-ocular inflammation. This case report describes cat-scratch disease neuroretinitis in a 10-year-old girl who presented with typical signs, including optic disc swelling and a macular star, preceded by pyrexia of unknown origin and cervical lymphadenopathy.

A 52-year-old woman with clinical features of Leber idiopathic stellate neuroretinitis (LISN) underwent retinal fluorescein and indocyanine green angiography that revealed lipid-containing fluid leakage from a single arteriole in the superficial nerve fiber layer of the optic disc. The fluid expanded gradually into the upper half of the optic disc and the adjacent peripapillary retina. Optical coherence tomography (OCT) demonstrated fluid accumulation in two separate subretinal spaces and in the outer nuclear-plexiform layer, which extended from the optic disc margin to the fovea. These angiographic and OCT findings support the hypothesis that LISN develops from focally increased permeability of an optic disc surface arteriole from which lipid-rich fluid flows through the outer nuclear-plexiform layer space to pool in these retinal areas.