Acute retinal pigment epitheliitis during treatment of hyperprolactinaemia.

BACKGROUND: Acute retinal pigment epitheliitis (ARPE) is a rare, idiopathic and self-limiting disease. The article aims to present ARPE in a patient using D2 dopamine receptor agonists for the treatment of hyperprolactinemia. CASE PRESENTATION: A 28-year-old female during hyperprolactinaemia treatment suffered from a dyschromatopsia and a central visual field defect in the left eye. She noticed a deterioration of vision and discontinued the cabergoline administration. The woman had not been diagnosed with other chronic conditions and exhibited no symptoms of infection. Upon admission, the patient was subjected to a test for COVID-19, which was negative. The ophthalmological examination revealed a decrease in visual acuity to distance in the left eye, which amounted to 18/20 on the Snellen chart. A central scotoma was noted on the Amsler chart and a loss of pigment epithelium was visible on the fundus of the left eye. Fluorescein angiography showed a discrete window defect in the left one, with no signs of leakage. Optical coherence tomography (OCT) scans of the maculae revealed a characteristic change in the photoreceptor layer and retinal pigment epithelium (RPE) in the fovea in the left eye. The electrophysiological tests revealed decreased function of cells in macular region. A magnetic resonance imaging (MRI) of the head and orbits demonstrated an asymmetric pituitary gland without chiasm compression and discrete signal enhancement from the left optic nerve. The patient underwent observation during hospitalisation. She reported improved colour vision and a decreased scotoma in the centre of her visual field. In regular outpatient follow-ups, successive improvements in visual acuity, as well as a decreased RPE damage and outer photoreceptor layer loss during an OCT test were observed. CONCLUSIONS: A case of ARPE is reported in a patient taking medications for hyperprolactinemia. The role of dopamine receptor antagonists in the photoreceptor function and causation of ARPE needs further evaluation.

Case series of neuroretinitis in Korea.

BACKGROUND: To present the clinical characteristics of neuroretinitis in Korea. METHODS: Twelve patients with neuroretinitis between January 2009 and September 2020 were retrospectively reviewed. Neuroretinitis was diagnosed based on fundus findings, optical coherence tomography, and fluorescein angiography. The serological findings of each patient were reviewed. RESULTS: Fifteen eyes of 12 patients (9 male and 3 female), with a mean age of 46.0 ± 10.7 years were included. Of the nine patients who underwent serological testing for Toxocara antibodies, six (66.6%) were positive. One patient had high titers of Toxoplasma immunoglobulins M and G. One patient diagnosed with dengue fever was suspected to have neuroretinitis in both eyes. There were no related abnormalities in the serological findings in four patients (33.3%) out of 12 patients. There were no suspected cases of cat-scratch disease. The six patients who were positive for Toxocara antibodies were older (mean age: 54.5 ± 9.1 years) than the others (mean age: 37.5 ± 4.4 years, p = 0.004). The four patients without any abnormal serological findings were relatively younger (mean age: 35.7 ± 3.0 years) than the other 8 patients (mean age: 51.1 ± 10.1 years, p = 0.008). CONCLUSIONS: Two-thirds of neuroretinitis patients were seropositive for Toxocara in the current cohort from Koreans. Causative factors in cases of neuroretinitis may vary according to age and region.

Varicella Zoster Associated Vasculopathy and Retinitis with Natalizumab Use in Multiple Sclerosis.

Natalizumab (Tysabri®, NTZ) is a monoclonal autoantibody approved for treatment of relapsing-remitting multiple sclerosis. NTZ inhibits leukocyte migration across the blood-brain barrier, preventing autoreactive cells from inciting an inflammatory immune response. This immunosuppression is highly efficacious in attenuating the risk of relapse of disease, but has been associated with opportunistic central nervous system (CNS) infections, most notably progressive multifocal leukoencephalopathy. Varicella-zoster and herpes simplex viruses have also been associated with NTZ, inciting a spectrum of disease, including encephalitis, meningitis, and acute retinal necrosis. While rare, these infections can result in devastating outcomes even when promptly identified and treated.   We present a case of combined CNS varicella zoster vasculitis and acute retinal necrosis in a 57-year-old woman maintained on monthly Natalizumab therapy, who presented with headache and visual field deficits.

Retinitis after haematopoietic stem cell transplantation with multiple intraocular viral infections (cytomegalovirus, Epstein‒Barr virus and herpes simplex virus)- a case report.

BACKGROUND: To report a case of retinitis with multiple intraocular viral infections after second haematopoietic stem cell transplantation. CASE PRESENTATION: A 39-year-old female patient developed retinitis after a second haematopoietic stem cell transplant. Right eye was tested for three viral infections- cytomegalovirus, Epstein‒Barr virus and herpes simplex virus, while left was infected with cytomegalovirus. The patient was subsequently treated with vitreous cavity ganciclovir injections, and 1 week later both eyes tested negative for aqueous humour viruses. DISCUSSION AND CONCLUSION: CMV, EBV and HSV belong to the herpes virus family. They are all commonly observed in the body and represent opportunity infectious viruses. The retinitis they cause have different characteristics. But simultaneous infection of the eye by multiple viruses is quite rare. In this case, three viruses were detected in the patient's eye, but whether the retina was caused by all three viruses at the same time could not be determined. A satisfactory outcome was achieved after treatment with vitreous cavity ganciclovir injection.

Enhanced systematic delivery of fluconazole-loaded biotin-glutathione functionalized chitosan-g-proline carrier into the infected retinitis treatment.

BACKGROUND: The polymer-based facile and effective drug carrier approach was developed to treat superficial fungal infected retinopathy infections. METHODS: Here, biotin-glutathione (B-GHS) functionalized with chitosan grafted proline (CS-g-P) moieties were fabricated with the loading of fluconazole (FLZ) for the treatment of retinopathy. FT-IR and XRD techniques were used to characterize chemical structural and phase changes of the prepared carriers The SEM results show that the sphere morphology with interconnection particle nature. RESULTS: The particle diameter was found as ~ 6.5 and ~ 8.6 nm for CS-g-P/B-GHS and FLZ-loaded CS-g-P/B-GHS carriers, respectively. The negative surface charge was found as the values of CS-g-P/B-GHS and FLZ-loaded CS-g-P/B-GHS, such as -20.7 mV and - 32.2 mV, from zeta potential analysis. The in-vitro FLZ releases from the CS-g-P/B-GHS were investigated at pH 7.4 (PBS) as the tear fluid environment, and it was observed at 85.02% of FLZ release in 8 h reaction time. The sustained release was observed, leading to the necessity for prolonged therapeutic effects. The antifungal effect of the carrier was studied by the minimum inhibitory concentration (MIC) and the percentage inhibition of viable fungal count against Candida albicans, and it observed 81.02% of the zone of inhibition by the FLZ carrier. CONCLUSION: FLZ-loaded CS-g-P/B-GHS carrier could inhibit the biofilm formation in a concentration-dependent inhibition. Hence, A novel FLZ/B-GHS-CS-g-P carrier is a hopeful approach for effectively treating superficial fungal contaminations of the retina region.

Enlarging aneurysm with paracentral acute middle maculopathy in idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) - a case report.

INTRODUCTION: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare clinical entity affecting young healthy individuals. Treatment primarily involves pan retinal photocoagulation (PRP) to capillary non perfusion areas. Intravitreal anti-VEGF or steroids are given in the presence of macula edema. Oral steroids do not alter the course of the disease. Arterial occlusions have been reported in IRVAN. METHODS: Retrospective case review. RESULT: A twenty seven year old male presented to us with mild blurring of vision for one week. His BCVA was OU 20/20. Anterior segment examination was normal. Fundus examination showed bilateral disc aneurysm with OS arterial aneurysm along the inferior arcade. Fundhus fluorescein angiography and OCT angiography were confirmatory of the disc and retinal aneurysm. Capillary non perfusion (CNP) areas were noted in the periphery. Two days later he presented with paracentral scotoma in his left eye which was confirmed by Amsler chart. Fundus, OCT and OCTA were confirmatory of Paracentral Acute Middle Maculopathy (PAMM). The retinal aneurysm had increased in size from 333 micron diameter to 566 micron diameter. Panretinal photocoagulation to the CNP areas was done and intravitreal antiVEGF was given. At 6 months follow up, retinal aneurysm had disappeared. DISCUSSION: Our case describes a unique event with sudden increase in size of the aneurysm leading to acute blockage in the deep capillary plexus thus being the first report of PAMM in IRVAN. The patient was treated with PRP and intravitreal anti-VEGF for the enlarging aneurysm which reduced in size within a week.

Ocular syphilis masquerading as CMV retinitis in a transplanted patient.

BACKGROUND: Syphilis has historically been referred to as "the great imitator", for the extent of disease manifestations secondary to infection. Ocular manifestations include a wide range of intra-ocular inflammation. METHODS: In this study, we report the case of a 52 years-old male patient with syphilitic hemorrhagic necrotizing retinitis. RESULTS: The patient presented to the emergency room for rapid and progressive vision loss and ocular redness lasting three weeks and was under immunosuppressive treatment. The diagnosis was syphilitic hemorrhagic necrotizing retinitis mimicking the typical clinical picture of retinitis caused by Cytomegalovirus infection in immunocompromised patients. CONCLUSIONS: The presented case highlights the need to consider ocular syphilis as a great masquerader even in the presence of atypical presentations such as hemorrhagic retinitis. Syphilis should be tested for treponemal and non-treponemal tests, and it should be ruled out as an etiological agent in every case of new-onset intra-ocular inflammation.

Short peptides derived from pigment epithelium-derived factor attenuate retinal ischemia reperfusion injury through inhibition of apoptosis and inflammatory response in rats.

Pigment epithelium-derived factor (PEDF) is widely recognized as a neuroprotective factor expressed in the retina and has shown therapeutic potential in several retinal diseases. Our study aimed to identify the neuroprotective fragment in PEDF and investigate its protective activity in retinas under ischemia-reperfusion (IR) condition. We synthesized a series of shorter synthetic peptides, 6-mer (Ser93-Gln98) and its d-form variant (6 dS) derived from the 44-mer (Val78-Thr121; a PEDF neurotrophic fragment), to determine their cytoprotective activity in IR injury, which was induced in rat retinas by injection of saline into the anterior chamber to increase the intraocular pressure (IOP) followed by reperfusion. We found the cytoprotective effect of 6-mer on glutamate-treated Neuro-2a cells and tert-butyl hydroperoxide (tBHP)-treated 661W cells were 2.6-fold and 1.5-fold higher than the 44-mer, respectively. The cytoprotective effect was blocked by a chemical inhibitor atglistatin and blocking antibody targeting PEDF receptor (PEDF-R). IR induced several impairments in retina, including cell apoptosis, activation of microglia/macroglia, degeneration of retinal capillaries, reduction in electroretinography (ERG) amplitudes, and retinal atrophy. Such IR injuries were ameliorated by treatment with 6-mer and 6 dS eye drops. Also, the neuroprotective activity of 6-mer and 6 dS in ischemic retinas were dramatically reversed by atglistatin preconditioning. Taken together, our data demonstrate smallest neuroprotective fragment of PEDF has potential to treat retinal degeneration-related diseases.

Fundus imaging features of congenital rubella retinopathy.

PURPOSE: To evaluate the clinical characteristics of congenital rubella retinopathy (CRR) with modern fundus imaging. METHODS: Single-center case series. Eleven patients (2005-2020) at the Emory Eye Center with known or presumed CRR. Trained image readers reviewed fundus imaging (color fundus photography, widefield pseudocolor imaging, near-infrared reflectance imaging, autofluorescence imaging, and spectral-domain optical coherence tomography) for pre-specified features suggestive of CRR. RESULTS: Eleven patients with confirmed (63.6%) or presumed (36.3%) CRR were identified. All were female with median (range) age of 53 (35-67) years. Six (54.5%) were born during the 1964-1965 United States rubella epidemic. All had congenital hearing loss. Two (18.2%) had a congenital heart defect. Eleven (50.0%) eyes had salt-and-pepper retinal pigmentary changes. Twenty-two eyes (100.0%) had irregularly distributed regions of speckled hypoautofluorescence. One eye (4.5%) had a presumed macular neovascularization. CONCLUSION: Modern fundus imaging demonstrates characteristic features of CRR, even when pigmentary changes are not readily apparent on examination. Widefield autofluorescence findings of irregularly distributed speckled hypoautofluorescence are particularly revealing. This series of newly diagnosed adults with CRR may represent the milder end of the phenotypic spectrum of this condition, highlighting imaging findings that may aid in diagnostically challenging cases of CRR.

CMV neuroretinitis in a post-transplant patient.

This report presents an atypical case of neuroretinitis in a post-transplant patient. A man in his 40s presented to a provincial ophthalmology service with unilateral left visual loss. He was immunosuppressed following orthotopic liver transplantation for end-stage liver cirrhosis secondary to primary sclerosing cholangitis. He had received his third Pfizer-BioNTech COVID-19 booster vaccine 34 days prior to symptom onset. His presenting left visual acuity was 6/36. His left optic nerve head was grossly swollen with peripapillary haemorrhage, intraretinal and subretinal fluid extending to involve the fovea. His serological and radiological investigations were all negative except for serum IgG and IgM positivity to cytomegalovirus (CMV). Following the commencement of antiviral treatment and systemic steroids, his neuroretinitis improved with visual recovery to 6/4.5. This report describes an atypical presentation of neuroretinitis in an immunocompromised patient without AIDS but with evidence of CMV exposure.

Incidental multiple myeloma in a patient with neuroretinitis: a case report.

BACKGROUND: Neuroretinitis is classically defined as a clinical triad of unilateral, painless vision loss, accompanied by optic disc edema and characteristic macular star formation. The causes of neuroretinitis can be categorized as infectious, non-infectious, and idiopathic, therefore differential diagnosis and careful evaluation are required, owing to the various etiologies and masqueraders. CASE PRESENTATION: A 54-year-old woman presented to the clinic with blurred vision in both eyes. A complete ophthalmic examination revealed optic disc edema with blurred margins and macular exudates, intraretinal edema in the temporal peripapillary area, and subretinal fluid with neurosensory retinal detachment in the macular area. Systemic laboratory investigations showed no signs of infection or inflammation. However, bone marrow suppression was suspected based on the results of the complete blood count test, and the patient was diagnosed with multiple myeloma. CONCLUSION: Although neuroretinitis is rarely accompanied by hematological malignancy, it is important to be mindful of the latter because ophthalmic manifestations are a common feature of hematological malignancies and lesions occur in nearly every ocular structure.

Pediatric Posterior Infectious Uveitis.

PURPOSE: To describe the most important cause of infectious posterior uveitis in pediatric patients. METHODS: Review of the literature. RESULTS: The most important causes of infectious uveitis in pediatric patients are: cat-scratch disease, toxocariasis, tuberculosis, viral diseases and toxoplasmosis. Ocular manifestations include retinitis, neuroretinitis, choroidal granulomas, peripheral granulomas and posterior pole granulomas. CONCLUSION: Infectious posterior uveitis is a challenging subject and should be considered in the differential diagnosis of any posterior uveitis in children. Infectious uveitis must be excluded before initiating immunosuppressive therapy.

Recurrent Idiopathic Neuroretinitis: Anti-MOG Positive?

Neuroretinitis, originally described by Leber at the turn of the 20th century, has long perplexed ophthalmologists due to its multiple recognized causes and often atypical presentation. Optic disk edema and macular star in the affected eye are well-agreed upon findings and are due to increased permeability of blood vessels near the optic disk and in the retina at large. It also is universally painless and presents with a relative afferent pupillary defect (RAPD) in the affected eye or eyes. However, depending on the infectious agent, an underlying autoimmune condition, or undefined idiopathic cause, there can be various degrees of vision loss, visual field loss, progression or recurrence, as well as involvement of the other eye. We present this case of presumed sequential idiopathic neuroretinitis with severe vision and visual field loss with a low-positive anti-MOG test in the border county of El Paso.

CD8+ T cells reduce neuroretina inflammation in mouse by regulating autoreactive Th1 and Th17 cells through IFN-γ.

Various regulatory CD8+ T-cell subsets have been proposed for immune tolerance and have been implicated in controlling autoimmune diseases. However, their phenotypic identities and suppression mechanisms are not yet understood. This study found that coculture of T-cell receptor (TCR)- or interferon (IFN)-ß-activated CD8+ T cells significantly suppressed the cytokine production of Th1 and Th17 cells. By experimenting with the experimental autoimmune uveitis (EAU), we found that adoptive transfer of TCR or IFN-ß-activated CD8+ T cells significantly lessened disease development in an IFN-γ-dependent manner with a decreased uveitogenic Th1 and Th17 response. Interestingly, after adoptive transfer into the EAU mice, the IFN-γ+ CD8+ T cells were recruited more efficiently into the secondary lymphoid organs during the disease-priming phase. This recruitment depends on the IFN-γ-inducible chemokine receptor CXCR3; knocking out CXCR3 abolishes the protective effect of CD8+ T cells in EAU. In conclusion, we identified the critical role of IFN-γ for CD8+ T cells to inhibit Th1 and Th17 responses and ameliorate EAU. CXCR3 is necessary to recruit IFN-γ+ CD8+ T cells to the secondary lymphoid organ for the regulation of autoreactive Th1 and Th17 cells.

A Case Series of Cat-Scratch Disease with Ocular Manifestations: Clinical Findings and Treatment Approach.

Objectives: To present the clinical and demographic characteristics, imaging findings, diagnosis and treatment approach in cases of cat scratch disease (CSD) with ocular involvement. Materials and Methods: The records of 19 patients followed-up and treated between 2010 and 2020, including detailed ophthalmological examinations, imaging findings, and treatment approach, were evaluated retrospectively. Results: Twenty-three eyes of 19 patients, 7 female (37%) and 12 male (63%), were included in the study. The mean age was 34.1±13.3 (range: 11-56) years, and the mean follow-up duration was 12.6±18.0 (range: 1-81) months. Unilateral involvement was observed in 15 cases (79%). Cat contact was reported in 14 cases (74%). In 6 cases (32%), flu-like symptoms were present before the ocular complaints. The mean visual acuity (VA) at presentation was 0.42±0.36 (range: 0.001-1.0). Anterior uveitis was observed in 3 eyes (13%). Posterior segment findings included neuroretinitis in 14 (61%), superficial retinal infiltrate(s) in 8 (35%), papillitis in 3 (13%), branch retinal artery occlusion in 2 (8%), and cilioretinal artery occlusion in 1 (4%) of the eyes. All cases were positive for Bartonella henselae immunglobulin (Ig)M and/or IgG. Systemic antibiotic therapy was administered to all patients. Intravenous pulse or oral corticosteroids were given, especially in cases with optic disc involvement. The mean final VA was 0.80±0.25 (range: 0.01-1.0). Conclusion: CSD may present with different ocular involvement patterns. Apart from the classical neuroretinitis and macular star appearance, patients may present with isolated optic disc edema, branch retinal artery occlusion, and retinal infiltrations. In such patients, cat contact history and Bartonella serology should be evaluated to differentiate CSD.