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Background: Ischemic optic neuropathy (ION) is exceedingly rare in children on dialysis, resulting from poor perfusion of the optic nerve, and presents as sudden acute painless vision loss. Case–diagnosis/treatment: We report the case of a 3-year-old male with stage 5 chronic kidney disease (CKD 5) due to focal segmental glomerulosclerosis (FSGS) status post-bilateral nephrectomy on chronic hemodialysis who had acute loss of vision several hours after a hemodialysis session. Earlier that day, he had a drop in blood pressure intra-dialysis to 89/67 mmHg, with at home blood pressures ranging 90/60 to 150/100 mmHg. The patient was treated with tight blood pressure control to maintain blood flow and prevent blood pressure lability, received high-dose corticosteroids with a corticosteroid taper, and placed on high-dose erythropoietin for neuroprotective effect. He regained partial vision beginning approximately 1 month after presentation. Conclusions: The exact cause of our patient's simultaneous bilateral anterior and posterior ION, confirmed via MRI and fundoscopic examination, is unclear; however, is likely secondary to a combination of fluctuating blood pressure, anemia, anephric status, and hemodialysis. This highlights the need for close blood pressure monitoring, management of anemia, and more diligent ophthalmologic screening in pediatric patients on chronic hemodialysis. [ABSTRACT FROM AUTHOR]

Simple Summary: This study investigates the adverse effects of selective intra-arterial chemotherapy (SIAC) on the eyes and optic nerves of retinoblastoma patients using magnetic resonance imaging (MRI). We aim to understand the post-SIAC changes in orbital and ocular structures and evaluate their impact on eye and optic nerve growth. Experienced radiologists analyzed MR images of retinoblastoma eyes treated with SIAC, comparing them to eyes treated with other eye-saving methods and healthy eyes. Results reveal common adverse effects like inflammation and vascular changes, along with significant ocular growth arrest and optic nerve atrophy in eyes treated with SIAC, especially in children treated ≤ 12 months of age. This study underscores the importance of careful consideration when utilizing SIAC, particularly in young patients, due to its potential negative effects on eye and optic nerve development. This retrospective multicenter study examines therapy-induced orbital and ocular MRI findings in retinoblastoma patients following selective intra-arterial chemotherapy (SIAC) and quantifies the impact of SIAC on ocular and optic nerve growth. Patients were selected based on medical chart review, with inclusion criteria requiring the availability of posttreatment MR imaging encompassing T2-weighted and T1-weighted images (pre- and post-intravenous gadolinium administration). Qualitative features and quantitative measurements were independently scored by experienced radiologists, with deep learning segmentation aiding total eye volume assessment. Eyes were categorized into three groups: eyes receiving SIAC (Rb-SIAC), eyes treated with other eye-saving methods (Rb-control), and healthy eyes. The most prevalent adverse effects post-SIAC were inflammatory and vascular features, with therapy-induced contrast enhancement observed in the intraorbital optic nerve segment in 6% of patients. Quantitative analysis revealed significant growth arrest in Rb-SIAC eyes, particularly when treatment commenced ≤ 12 months of age. Optic nerve atrophy was a significant complication in Rb-SIAC eyes. In conclusion, this study highlights the vascular and inflammatory adverse effects observed post-SIAC in retinoblastoma patients and demonstrates a negative impact on eye and optic nerve growth, particularly in children treated ≤ 12 months of age, providing crucial insights for clinical management and future research. [ABSTRACT FROM AUTHOR]

Perioperative visual loss (POVL) is a rare but severe complication following non-ophthalmological surgery under general anesthesia. A POVL can be caused by lesions in any part of the optical system. The predominant causes include corneal injuries and particularly ischemic damage. The symptoms of POVL substantially vary ranging from reduced vision to complete blindness. The risks involve factors related to the surgery as well as patient-specific factors. In general, the prognosis in cases of mechanical damage is better than for ischemic lesions. The treatment measures depend on the underlying pathomechanism and due to the limited evidence only a few treatment options are available. Therefore, preventive measures and meticulous documentation play a crucial role. [ABSTRACT FROM AUTHOR]

Objective: Optic neuritis (ON) is a condition that causes vision loss usually in one eye, often due to multiple sclerosis (MS). In this study, we aim to examine the clinical course, diagnostic tests, and treatment outcomes of patients presenting with acute or subacute ON. Method: In this retrospective study, we examined the medical records of pediatric patients aged 3-18 years who were evaluated for acute ON in our neurology department between January 2015 and January 2021. Results: Our study population of 18 participants consisted of female (55.6%), and male (44.4%) patients with an overall mean age of 13.8±2.3 years at admission. During the follow-up period, patients received the diagnosis of isolated ON (n=10), MS (n=7), and acute disseminated encephalomyelitis (n=1). The most common complaints at initial presentations were blurred vision and visual loss. ON was unilateral in 83.7% and bilateral in 16.7% of the patients. Color vision was initially impaired in 11 of 18 patients. Cranial magnetic resonance imaging (MRI), orbital MRI and spinal MRI revealed demyelinating lesions at different rates. Conclusion: It is crucial to consider ON as one of the potential causes of vision loss in patients. The possibility of other demyelinating diseases, including MS, which can be present or may develop in patients with ON either during the initial presentation or follow-up should be kept in mind. [ABSTRACT FROM AUTHOR]

Objectives: To review the current literature related to the correlation between translaminar pressure difference (TLPD) and glaucoma. Materials and Methods: In this article, we conducted a literature review using MEDLINE via PubMed, Cochrane Eyes and Vision, and Google Scholar from 01/01/2010 to 31/12/2022. Search terms included "glaucoma", "intraocular pressure", "translaminar cribrosa pressure gradient/difference", "intracranial pressure", and "cerebrospinal fluid pressure". Of 471 results, 8 articles were selected for the meta-analysis. Results: Our meta-analysis demonstrated significantly higher intraocular pressure, lower cerebrospinal fluid pressure (CSFp), and greater TLPD in high-tension and normal-tension glaucoma groups compared to healthy groups. Conclusion: The differences in CSFp and TLPD between glaucoma and healthy people detected in current studies suggests a potential relationship between TLPD and glaucoma. [ABSTRACT FROM AUTHOR]

Strachan's syndrome comprises a triad of optic, auditory and painful sensory peripheral neuropathy. It has been recognised since the late 19th century and is presumed to result from nutritional deficiency. Patients present acute or subacutely after a period of systemic illness, weight loss or, most commonly, dietary restriction, especially veganism, which can cause riboflavin (vitamin B2) and vitamin B12 deficiencies. The syndrome is more common in people who are black British and often of Jamaican descent. We describe the clinical phenotype using a typical case example, review other endemic nutritional peripheral neuropathies and discuss the potential benefit of riboflavin as a treatment. [ABSTRACT FROM AUTHOR]

Glaucoma is a chronic, progressive eye disease affecting the optic nerve, which may cause visual damage and blindness. In this study, we present a machine-learning investigation to classify patients with glaucoma (case group) with respect to normal participants (control group). We examined 172 eyes at the Ophthalmology Clinic of the "Elpis" General Hospital of Athens between October 2022 and September 2023. In addition, we investigated the glaucoma classification in terms of the following: (a) eye selection and (b) gender. Our methodology was based on the features extracted via two diagnostic optical systems: (i) conventional optical coherence tomography (OCT) and (ii) a modern RETeval portable device. The machine-learning approach comprised three different classifiers: the Bayesian, the Probabilistic Neural Network (PNN), and Support Vectors Machines (SVMs). For all cases examined, classification accuracy was found to be significantly higher when using the RETeval device with respect to the OCT system, as follows: 14.7% for all participants, 13.4% and 29.3% for eye selection (right and left, respectively), and 25.6% and 22.6% for gender (male and female, respectively). The most efficient classifier was found to be the SVM compared to the PNN and Bayesian classifiers. In summary, all aforementioned comparisons demonstrate that the RETeval device has the advantage over the OCT system for the classification of glaucoma patients by using the machine-learning approach. [ABSTRACT FROM AUTHOR]

Since its introduction, optical coherence tomography (OCT) has revolutionized the field of ophthalmology and has now become an indispensable, noninvasive tool in daily practice. Most ophthalmologists are familiar with its use in the assessment and monitoring of retinal and optic nerve diseases. However, it also has important applications in the assessment of anterior segment structures, including the cornea, conjunctiva, sclera, anterior chamber, and iris, and has the potential to transform the clinical examination of these structures. In this review, we aim to provide a comprehensive overview of the potential clinical utility of anterior segment OCT (AS-OCT) for a wide range of anterior segment pathologies, such as conjunctival neoplasia, pterygium, scleritis, keratoconus, corneal dystrophies, and infectious/noninfectious keratitis. In addition, the clinical applications of AS-OCT (including epithelial mapping) in preoperative planning and postoperative monitoring for corneal and refractive surgeries are discussed. [ABSTRACT FROM AUTHOR]

Purpose: To investigate how the anatomical, vascular and functional structure of the optic nerve is affected in patients with retinitis pigmentosa (RP) and to compare the data obtained with healthy volunteers. Materials and Methods: This cross-sectional, comparative clinical study included 25 eyes of 25 RP cases and 25 eyes of 25 healthy volunteers. After the routine ophthalmological examination, optical coherence tomography angiography (OCTA) and pattern electroretinography (pERG) examinations were performed. Results: In the optic nerve head retinal nerve fiber layer (RNFL) thickness analysis, the thickness of lower temporal quadrant and mean RNFL thickness values were found to be significantly lower in the patient group when compared to the control group (respectively; p=0.001; p=0.003). In pERG analysis, it was found that there was a prolongation in implicit time and a decrease in wave amplitudes were detected in P50 and N95 waves in patients with RP (p<0.05 for all values).Radial peripapillary capillary (RPC) mesh vascular density (VD) was significantly lower in all quadrants in the RP group (p<0.001). A correlation was found between best corrected visual acuity (BCVA) and upper nasal quadrant RNFL thickness. Conclusion: In RP patients, the vascular changes can cause optic nerve damage and decrease in BCVA. To identify changes in both vascular and nerve fiber structures around the disc using OCTA may contribute to the prediction of the prognosis and follow-up of the disease. The limited evaluable wave formation in the pERG test suggests that pERG does not provide clear results for functional evaluation in RP. [ABSTRACT FROM AUTHOR]

Glaucoma represents a group of neurodegenerative diseases characterized by optic nerve damage and the slowly progressive death of retinal ganglion cells. Glaucoma is considered the second leading cause of irreversible blindness worldwide. Pharmaceutical treatment of glaucoma is critical because of the properties of the ocular barrier that limit the penetration of drugs, resulting in lower systemic bioavailability. This behavior causes the need of frequent drug administration, which leads to deposition of concentrated solutions on the eye, causing toxic effects and cellular damage to the eye. To overcome these drawbacks, novel drug-delivery systems, such as liposomes, can play an important role in improving the therapeutic efficacy of antiglaucomatous drugs. In this work, liposomes were synthesized to improve various aspects, such as ocular barrier penetration, bioavailability, sustained release of the drug, targeting of the tissue, and reduction in intraocular pressure. Citicoline (CDP-choline; cytidine 5′-diphosphocholine) is an important intermediate in the biosynthesis of cell membrane phospholipids, with neuroprotective and neuroenhancement properties, and it was used in the treatment on retinal function and neural conduction in the visual pathways of glaucoma patients. In this study, citicoline was loaded into the 1,2-dioleoyl-sn-glycerol-3-phosphocholine and cholesterol liposomal carrier to enhance its therapeutic effect. The citicoline encapsulation efficiency, drug release, and size analysis of the different liposome systems were investigated using dynamic light scattering, nuclear magnetic resonance, infrared spectroscopy, and ToF-SIMS experiments. [ABSTRACT FROM AUTHOR]

Systemic vascular occlusive disease associated with neurofibromatosis type 1 (NF1) has been reported in the aortic, cerebral, renal, celiac, and mesenteric vessels and is referred to as NF1 vasculopathy. Although retinal vascular involvement in patients with NF1 usually manifests as retinal capillary hemangiomatosis, a few cases of NF1 with retinal vascular occlusive disease have also been described. Here, we report a 2-year-old girl with NF1 who presented with branch retinal vein occlusion and peripheral retinal ischemia secondary to NF1. This case demonstrates that NF1-related retinal occlusive vasculopathy may occur in very young patients and that detailed fundus examination with fluorescein angiography is necessary in all patients with NF1. [ABSTRACT FROM AUTHOR]

Background: Individuals with thiamine-responsive megaloblastic anemia (TRMA) mainly manifest macrocytic anemia, sensorineural deafness, ocular complications, and nonautoimmune diabetes. Macrocytic anemia and diabetes may be responsive to high-dosage thiamine treatment, in contrast to sensorineural deafness. Little is known about the efficacy of thiamine treatment on ocular manifestations. Cases presentation: Our objective is to report data from four Italian TRMA patients: in Cases 1, 2 and 3, the diagnosis of TRMA was made at 9, 14 and 27 months. In 3 out of 4 subjects, thiamine therapy allowed both normalization of hyperglycemia, with consequent insulin suspension, and macrocytic anemia. In all Cases, thiamine therapy did not resolve the clinical manifestation of deafness. In Cases 2 and 3, follow-up showed no blindness, unlike Case 4, in which treatment was started for megaloblastic anemia at age 7 but was increased to high doses only at age 25, when the genetic diagnosis of TRMA was performed. Conclusions: Early institution of high-dose thiamine supplementation seems to prevent the development of retinal changes and optic atrophy in TRMA patients. The spectrum of clinical manifestations is broad, and it is important to describe known Cases to gain a better understanding of this rare disease. [ABSTRACT FROM AUTHOR]

Glaucoma causes the degeneration of the retinal ganglion cells (RGCs) and their axons, inducing a tissue reshaping that affects both the retina and the optic nerve head. Glaucoma care especially focuses on reducing intraocular pressure, a significant risk factor for progressive damage to the optic nerve. The use of natural treatments, such as herbs, vitamins, and minerals, is becoming increasingly popular today. While plants are a rich source of novel biologically active compounds, only a small percentage of them have been phytochemically examined and evaluated for their medicinal potential. It is necessary for eye care professionals to inform their glaucoma patients about the therapy, protection, and efficacy of commonly used herbal medicines, considering the widespread use of herbal medicines. The purpose of this review is to examine evidence related to the most widely used herbal medicines for the management and treatment of glaucoma, to better understand the potential benefits of these natural compounds as supplementary therapy. [ABSTRACT FROM AUTHOR]

Objective • To explore the incidence and associated factors of glaucoma optic nerve damage (GOND) among patients with Posner-Schlossman syndrome. Methods • We retrieved relative studies till July 2022 using databases including PubMed, CNKI, VIP, and Wan-Fang. The retrieval terms include “Posner-Schlossman syndrome”, “Glaucomatocyclitic Crisis”, and “visual field”. The Chinese retrieval terms were the corresponding Chinese terminologies for the English terms mentioned above. The outcomes were the incidence of GOND among PSS patients, the male proportion, patient age, the proportion of patients with single eye affected, disease duration, and intraocular pressure during the episode in patients with or without GOND. Review manager 5.3 was used for the analysis. Results • In total, 19 studies were included in our analysis. The pooled incidence of GOND among PSS patients was 0.26 (95% CI = 0.16-0.43). Age [MD = 11.3(5.86, 16.73); P < .0001], disease duration [MD = 4.27 (3.38, 5.16), P < .00001], and single or double eye affected [RR = 0.69 (0.49, 0.98), P=.04] were significantly associated with the development of GOND. Whereas, gender [RR = 1.09 (0.91, 1.29), P = .35] and intraocular pressure at episodes [MD = 2.66 (-0.38, 5.7), P = .09] were not significantly associated with GOND development. Conclusion • A fraction of PSS patients ultimately develop GOND so physicians should not be highly optimistic about the prognosis of PSS patients and timely and effective treatment is very important. Patients of higher age, those with double eyes affected, and suffering from a long disease duration might be at a greater risk of developing GOND. [ABSTRACT FROM AUTHOR]

Background • The optic nerve fiber layer, composed of ganglion cell axons within the ganglion cell layer, undergoes thickness changes due to diabetic retinopathy. However, the relationship between intraocular pressure (IOP) and optic fiber layer thickness remains unclear. Objective • To investigate the correlation between 24-hour intraocular pressure and optic nerve fiber layer thickness in patients with early diabetic retinopathy. Methods • This retrospective study collected 353 patients with early diabetic retinopathy from January 2019 to December 2021. They were categorized into the retinopathy group (n = 153) and the control group (n = 200). 24-hour IOP and optic fiber layer thickness were assessed, and the correlation between them was analyzed. Results • The observation group exhibited significantly higher 24-hour IOP compared to the control group (16.64 ± 2.58 vs. 15.63 ± 2.52 mmHg, P < .001). Notably, the thickness of upper, lower, nasal, temporal, and average optic nerve fiber layers in the observation group decreased significantly (P<.001). Pearson linear correlation revealed significant negative associations between 24-hour IOP and upper, nasal, temporal, and mean optic nerve fiber layer thickness (R2 = -0.277, -0.399, -0.344, and -0.489, P<.05). The upper, lower, nasal, temporal, and mean optic fiber thickness demonstrated diagnostic value for nonearly diabetic retinopathy in type 2 diabetes patients (P < .05), with mean optic fiber thickness displaying the highest diagnostic potential (area under the curve: 0.843, 95% Confidence Interval: 0.803-0.884, P<.001). Conclusions • Thinning of the optic nerve fiber layer in early diabetic retinopathy patients holds predictive value for the condition and exhibits a negative correlation with 24-hour intraocular pressure. [ABSTRACT FROM AUTHOR]

Renal coloboma syndrome (RCS) and dominant optic atrophy are mainly caused by heterozygous mutations in PAX2 and OPA1, respectively. We describe a patient with digenic mutations in PAX2 and OPA1. A female infant was born without perinatal abnormalities. Magnetic resonance imaging at 4 months of age showed bilateral microphthalmia and optic nerve hypoplasia. Appropriate body size was present at 2 years of age, and mental development was favorable. Color fundus photography revealed severe retinal atrophy in both eyes. Electroretinography showed slight responses in the right eye, but no responses in the left eye, suggesting a high risk of blindness. Urinalysis results were normal, creatinine-based estimated glomerular filtration rate was 63.5 mL/min/1.73 m2, and ultrasonography showed bilateral hypoplastic kidneys. Whole exome sequencing revealed de novo frameshift mutations in PAX2 and OPA1. Both variants were classified as pathogenic (PVS1, PS2, PM2) based on the guidelines from the American College of Medical Genetics and Genomics (ACMG). Genetic testing for ocular diseases should be considered for patients with suspected RCS and a high risk of total blindness. [ABSTRACT FROM AUTHOR]

A 45-year-old woman presented with sudden complete vision loss in her left eye and retroorbital pain worsened by eye movements. A previous milder episode of vision loss had occurred in the same eye 1 year before, with complete recovery after high-dose intravenous methylprednisolone. She had no light perception in the left eye with a swollen optic disc, but with a normal right optic disc. There were no systemic manifestations or infections. MR scan of the brain showed extensive enlargement and enhancement of the left optic nerve and optic chiasm. After excluding infections and autoimmune markers, a left optic nerve biopsy confirmed non-caseating granulomas, leading to a diagnosis of neurosarcoidosis. [ABSTRACT FROM AUTHOR]

This article discusses the challenges and opportunities in the diagnosis and management of glaucoma, a sight-threatening eye condition. It highlights the high rates of undiagnosed glaucoma in Australia, even among those who have seen an optometrist or ophthalmologist. The article emphasizes the importance of early detection and suitable management to minimize the disability-adjusted life years (DALYs) associated with vision loss. It also explores the role of new diagnostic techniques, collaborative care models, and emerging technologies like artificial intelligence (AI) in improving glaucoma diagnosis and management. The article concludes by emphasizing the need for intra- and inter-professional collaboration and a deeper understanding of disease mechanisms to improve patient outcomes and develop novel therapies. [Extracted from the article]

The article presents a case study of an elderly couple who experienced acute vision loss following accidental ingestion of closantel, a veterinary drug. Despite limited literature on closantel toxicity in humans, the study emphasizes the importance of early treatment with systemic corticosteroids and plasmapheresis to reverse toxic effects, highlighting the need for public awareness and appropriate drug labeling to prevent accidental toxicity.

Background: In multiple sclerosis (MS), optical coherence tomography (OCT) has become a sensitive tool for evaluating retinal and optic nerve diseases. Optical coherence tomography angiography (OCT-A) is a novel technique that can assess the density of retinal vascular networks. Vascular changes caused by MS play a chief role in the development and progression of the MS lesions giving the idea and goal of this study which aimed to assess vascular anomalies in MS using OCT and OCT-A, determine their relationship to clinical characteristics, and determine if vasculopathy may play a role in MS causation. This research also looked at whether the retinal vasculature can be used as a biomarker for neurodegeneration and disease progression. A total of 30 MS patients with and without history of optic neuritis (ON), as well as 10 healthy volunteers, were included in the study. OCT-A and spectral domain. (SD-OCT) were performed on all of them. The data included clinical history, the Expanded Disability Status Scale (EDSS), illness duration, visual function assessment, and investigations. Results: When comparing MS patients to controls, OCT-A revealed a drop in the arterial density in the eyes of MS patients. When compared to control eyes, all SD-OCT and OCT-A parameters indicated a statistically significant drop in MS eyes (with and without ON). Results showed a direct association (p = 0.001) between vascular density metrics and SD-OCT parameters (p ≤ 0.001). Conclusions: Results showed a reduction in retinal vascular density (VD) in MS patients. The clinical link between VD and SD-OCT characteristics is highlighted, implying that OCT-A may be a useful marker of illness and impairment in MS. [ABSTRACT FROM AUTHOR]

Purpose: SARS‐CoV‐2 viral infection affects multiple systems including the respiratory, gastrointestinal, neurological, cardiac, and ophthalmic systems. We report a case of myelin oligodendrocyte glycoprotein (MOG) related optic neuritis in a SARS‐CoV‐2 (COVID‐19) patient. Methods: Case report. Results: A 36‐year‐old Malay gentleman with underlying hypertension presented with the first episode of bilateral progressively worsening blurred vision for 1 week associated with retrobulbar pain. There were no other neurological symptoms. He had fever a week before the eye symptoms and tested positive for COVID‐19. He received COVID‐19 booster vaccine a month before the disease onset. On examination, his vision was hand motion on right eye and 6/18 on left eye. Relative afferent pupillary defect (RAPD) was positive on right eye with abnormal optic nerve function tests. Anterior segments were unremarkable. Fundus examination showed bilateral optic disc swelling. MRI revealed multifocal hyperintense subcortical white matter lesions. Optic nerves appeared normal with no enhancement seen. Blood investigation showed a positive serum MOG antibody. Intravenous methylprednisolone was commenced followed by oral prednisolone after which his vision and ocular symptoms markedly improved. The oral prednisolone was tapered alongside addition of azathioprine. At 1 month, the disease was stable with no recurrence. Conclusion: While optic neuritis has been associated with both COVID‐19 infection and vaccination, MOG IgG antibody‐mediated optic neuritis is also a possible manifestation. This type of optic neuritis associated with COVID‐19 infection does not show a similar pattern of frequent recurrences as seen in non‐COVID‐19 related optic neuritis. [ABSTRACT FROM AUTHOR]

Acute optic neuritis (AON) is a common cause of sudden visual loss in young patients. Because of the risk of demyelinating disease, patients affected by unilateral or bilateral optic neuritis should be evaluated and treated accordingly. Despite advancements in imaging of the brain and retina, misdiagnosis of AON is not uncommon. Indeed, some acute disorders of the retina have the potential to mimic AON and their prompt diagnosis may avoid unnecessary neurologic investigation, psychological stress to the patient, and delays in treatment. This review describes uncommon retinal disorders presenting with sudden-onset visual loss and absent or subtle funduscopic manifestation that can mimic AON. Multimodal retinal imaging is essential in detecting these conditions and in their differential diagnosis. It behooves neurologists and general ophthalmologists to be aware of these entities and be familiar with multimodal imaging of the retina. [ABSTRACT FROM AUTHOR]

Primary optic nerve sheath meningioma is generally a benign tumor. In rare instances, however, the growth rate and intraocular and intracranial extensions can be highly aggressive, especially in children, leading to poor prognosis. Here, we reported a case of a 24-year-old woman who presented with left eye swelling for 3 years. This was associated with blurred vision, retrobulbar pain, and redness. On examination, the left eye was severely proptosed with complete ophthalmoplegia. Magnetic resonance imaging showed an extensive tumor occupying the whole left orbital cavity with a disfigured eyeball. However, no intracranial extension was observed. Interestingly, complete surgical excision was feasible via transconjunctival anterior orbitotomy without bone removal. The histopathological examination confirmed the diagnosis of optic nerve sheath meningioma. Adjunct radiotherapy was given. On a follow-up after 2 years, left enophthalmos with esotropia was observed. [ABSTRACT FROM AUTHOR]

Retinal imaging has greatly expanded our understanding of various pathological conditions. This article presents a summary of the key points covered during the 2022 Ophthalmologica Lecture held at the Euretina Congress in Hamburg. The first part of the article focuses on the use of optical coherence tomography angiography to examine and comprehend the choroid in age-related macular degeneration (AMD). Subsequently, we delve into the discussion of the "postreceptor neuronal loss" theory in AMD, which was studied using en face structural optical coherence tomography (OCT). Following that, we explore pertinent findings obtained through cross-sectional OCT in retinal and optic nerve diseases, such as AMD, diabetic macular edema, pathologic myopia, central serous chorioretinopathy, and Leber's hereditary optic neuropathy. [ABSTRACT FROM AUTHOR]

The retinal ganglion cells (RGCs) are the sole output neurons that connect information from the retina to the brain. Optic neuropathies such as glaucoma, trauma, inflammation, ischemia and hereditary optic neuropathy can cause RGC loss and axon damage, and lead to partial or total loss of vision, which is an irreversible process in mammals. The accurate diagnoses of optic neuropathies are crucial for timely treatments to prevent irrevocable RGCs loss. After severe ON damage in optic neuropathies, promoting RGC axon regeneration is vital for restoring vision. Clearance of neuronal debris, decreased intrinsic growth capacity, and the presence of inhibitory factors have been shown to contribute to the failure of post‐traumatic CNS regeneration. Here, we review the current understanding of manifestations and treatments of various common optic neuropathies. We also summarise the current known mechanisms of RGC survival and axon regeneration in mammals, including specific intrinsic signalling pathways, key transcription factors, reprogramming genes, inflammation‐related regeneration factors, stem cell therapy, and combination therapies. Significant differences in RGC subtypes in survival and regenerative capacity after injury have also been found. Finally, we highlight the developmental states and non‐mammalian species that are capable of regenerating RGC axons after injury, and cellular state reprogramming for neural repair. [ABSTRACT FROM AUTHOR]

We report two cases of optic nerve pathology after the administration of the Pfizer-BioNTech and AstraZeneca-Oxford COVID-19 vaccines, respectively, and describe the implications for management of post-vaccination central nervous system (CNS) inflammation. A 69-year-old woman presented with bilateral optic nerve head oedema, 16 days after the second dose of the Pfizer-BioNTech vaccine. She was diagnosed with post-vaccination CNS inflammatory syndrome and was treated for five days with intravenous methylprednisolone at a dose of 1 gram per day. Her optic disc swelling improved, and her vision stabilised. A 32-year-old woman presented six days after her first dose of the AstraZeneca-Oxford vaccine with two days of sudden onset of progressive blurring of vision in her left eye. Posterior segment examination revealed left optic disc swelling, and an MRI of the brain, orbit, and cervical spine was significant for left optic nerve enhancement. The patient was diagnosed with a unilateral post-vaccination optic neuritis. She was treated with a three-day course of intravenous methylprednisolone followed by oral prednisone. Her optic disc swelling and visual field improved, and she recovered 6/6 vision. Clinicians and patients should be aware of the potential for post-vaccination CNS inflammatory syndromes associated with COVID-19 vaccine administration. Neuroimaging and cerebrospinal fluid analysis may aid in the diagnosis of the cause of vision loss. Further studies are needed to evaluate the spectrum and frequency of optic nerve involvement associated with COVID-19 vaccination. [ABSTRACT FROM AUTHOR]

BACKGROUND: The aim of this case report is to present the successful management of both diplopia and amblyopia in a specific clinical situation, demonstrating neuroplasticity of the visual system in an adult patient. Causes of diplopia include eye pathologies in monocular diplopia and ischemic ocular motor nerve palsies, sudden life-threatening and chronic conditions in central nervous system in binocular diplopia. Strabismic amblyopia and nonarteritic anterior ischemic optic neuropathy are quite often ophthalmic conditions, first one is caused by suppression during developmental period and the latter one by ischemia of the optic nerve in adults. Coexistence of aforementioned conditions may cause unusual clinical situation in which ability of nervous system to functional reorganization could be demonstrated. CASE PRESENTATION: In our adult patient, diplopia was incited by the loss of suppression of the strabismic amblyopic eye, which was the consequence of a sudden decrease of the visual acuity in the previously better eye in the course of nonarteritic anterior ischemic optic neuropathy. This led to impairment in daily activities. RESULTS: Visual training rehabilitation improved distance and near visual acuity in the amblyopic eye over three months, and prescribing two pairs of glasses with prisms enabled the patient to return to daily activities. CONCLUSION: The discussed patient lost the suppression of the strabismic amblyopic eye. Management of amblyopia is usually undertaken in children, however considering neuroplasticity we successfully attempted to improve visual functioning of our patient, despite lower intensity of neuroplasticity functions in an adult brain. [ABSTRACT FROM AUTHOR]

The article focuses on the use of gene therapy for Leber's hereditary optic neuropathy (LHON), a mitochondrial genetic disorder, which has shown promising benefits in preserving retinal ganglion cells (RGCs) and improving vision in patients through the use of nuclear deoxyribonucleic acid transported into mitochondria. It mentions the REFLECT trial, a Phase III study, demonstrated a statistically significant increase in visual acuity in treated eyes compared to placebo eyes.

Purpose: Diagnosis of congenital optic nerve hypoplasia (CONH) can be challenging in children or uncooperative individuals. Misdiagnosis can lead to inappropriate treatment; thus, it is important to identify an objective and reliable measurement. The purpose of this study was to evaluate whether Cirrus spectral domain optical coherence tomography (SD‐OCT) is a valid test for diagnosing CONH by comparing it to the disc–macula distance to disc diameter (DM:DD) ratio. Methods: A total of 93 participants (64 controls and 29 CONH) underwent comprehensive eye examinations, fundus photography and Cirrus SD‐OCT. Receiver operating characteristic (ROC) curves for the DM:DD ratio and OCT disc area were constructed for CONH and control eyes. Results: Mean (±SD) OCT disc area was 1.46 (±0.42) mm2 and 1.89 (±0.38) mm2 for CONH and control eyes, respectively (p < 0.0001). The area under the curve for the DM:DD ratio was 0.97 (95% confidence interval: 0.91–0.99) and 0.79 for OCT disc area (95% confidence interval: 0.70–0.86), which were significantly different (p = 0.0005). The optimal cut‐off value for OCT disc area was 1.66 mm2 (76% sensitivity, 70% specificity), while the optimal cut‐off for DM:DD ratio was 3.10 (85% sensitivity and 95% specificity). The Cirrus SD‐OCT showed a tendency to overestimate disc size, especially in cases with no light perception (NLP) or segmental CONH. Conclusions: Although the DM:DD ratio is superior to OCT in diagnosing CONH with a higher sensitivity and specificity, the ratio is subject to inter‐examiner variability and can be challenging to obtain. We found the Cirrus SD‐OCT to be a valid objective test for diagnosing CONH. Caution is advised when using SD‐OCT in segmental CONH or in an eye with NLP. We suggest 1.66 mm2 as the optimal cut‐off value for Cirrus SD‐OCT disc area to differentiate a hypoplastic from a normal optic disc. [ABSTRACT FROM AUTHOR]

Deep learning (DL) represents a paradigm-shifting, burgeoning field of research with emerging clinical applications in optometry. Unlike traditional programming, which relies on human-set specific rules, DL works by exposing the algorithm to a large amount of annotated data and allowing the software to develop its own set of rules (i.e. learn) by adjusting the parameters inside the model (network) during a training process in order to complete the task on its own. One major limitation of traditional programming is that, with complex tasks, it may require an extensive set of rules to accurately complete the assignment. Additionally, traditional programming can be susceptible to human bias from programmer experience. With the dramatic increase in the amount and the complexity of clinical data, DL has been utilised to automate data analysis and thus to assist clinicians in patient management. This review will present the latest advances in DL, for managing posterior eye diseases as well as DL-based solutions for patients with vision loss. [ABSTRACT FROM AUTHOR]

Glaucoma is a chronic eye disease that may lead to permanent vision loss if it is not diagnosed and treated at an early stage. The disease originates from an irregular behavior in the drainage flow of the eye that eventually leads to an increase in intraocular pressure, which in the severe stage of the disease deteriorates the optic nerve head and leads to vision loss. Medical follow-ups to observe the retinal area are needed periodically by ophthalmologists, who require an extensive degree of skill and experience to interpret the results appropriately. To improve on this issue, algorithms based on deep learning techniques have been designed to screen and diagnose glaucoma based on retinal fundus image input and to analyze images of the optic nerve and retinal structures. Therefore, the objective of this paper is to provide a systematic analysis of 52 state-of-the-art relevant studies on the screening and diagnosis of glaucoma, which include a particular dataset used in the development of the algorithms, performance metrics, and modalities employed in each article. Furthermore, this review analyzes and evaluates the used methods and compares their strengths and weaknesses in an organized manner. It also explored a wide range of diagnostic procedures, such as image pre-processing, localization, classification, and segmentation. In conclusion, automated glaucoma diagnosis has shown considerable promise when deep learning algorithms are applied. Such algorithms could increase the accuracy and efficiency of glaucoma diagnosis in a better and faster manner. [ABSTRACT FROM AUTHOR]

Although optic disc cupping is mostly seen in glaucoma patients, it can occur in non-glaucomatous optic neuropathies (NGON). The characteristics of NGON are cupping toxic optic neuropathies, optic neuritis, compressive ischemia, and hereditary nature. The basic components of optic disc cupping are prelaminar and laminar. Prelaminar thinning, which seemed to be non-specific, occurs in all types of retinal ganglion cell axon loss; such as compressive, ischemic, and inflammatory events; glaucoma; and aging. This form of cupping is usually shallow, with less excavation of the optic disc. Laminar type of cupping which is a clinically profound type of cupping, may damage peripapillary scleral and lamina cribrosa. Sometimes experienced clinicians cannot clearly distinguish glaucomatous from non-glaucomatous cupping. The non-glaucomatous optic neuropathy has more neuroretinal rim pallor with less excavation of the disc than in glaucoma. It also involves central visual acuity and color vision in primary levels with visual field defects aligned vertically and respecting the midline. Evaluation of the patient's medical records, disease presentation, ocular function, and examination are also crucial. Secondary examinations, including visual field examination and optical coherence tomography (OCT) or neuroimaging, facilitate the disease's differentiation. This review presents the methods of examining a patient with an increased cup-to-disc ratio. [ABSTRACT FROM AUTHOR]

Background: Multiple sclerosis (MS) is a common neurological disease affecting the optic nerve, directly or indirectly, through transsynaptic axonal degeneration along the visual pathway. New ophthalmological tools, arguably the most important being optical coherence tomography (OCT), could prove paramount in redefining MS diagnoses and shaping their follow-up protocols, even when the optic nerve is not involved. Methods: A prospective clinical study was conducted. In total, 158 eyes from patients previously diagnosed with relapsing remitting MS (RRMS)—with or without optic neuritis (ON), clinically isolated syndrome (CIS) with or without ON, and healthy controls were included. Each patient underwent an ophthalmologic exam and OCT evaluation for both eyes (a posterior pole analysis (PPA) and the optic nerve head radial circle protocol (ONH-RC)). Results: The macular retinal thickness (the 4 × 4, respectively, 2 × 2 grid) and thickness of the peripapillary retinal nerve fiber layer (pRNFL) were investigated. Various layers of the retina were also compared. Our study observed significant pRNFL thinning in the RRMS eyes compared to the control group, the pRNFL atrophy being more severe in the RRMS-ON eyes than the RRMS-NON eyes. In the ON group, the macular analysis showed statistically significant changes in the RRMS-ON eyes when compared only to the CIS-ON eyes, regarding decreases in the inner plexiform layer (IPL) thickness and inner nuclear layer (INL) on the central 2 × 2 macular grid. The neurodegenerative process affected both the inner retina and pRNFL, with clinical damage appearing for the latter in the following order: CIS-NON, CIS-ON, RRMS-NON, and RRMS-ON. In the presence of optic neuritis, SMRR patients presented an increase in their outer retina thickness compared to CIS patients. Conclusions: To differentiate the MS patients from the CIS patients, in the absence of optic neuritis, OCT Posterior Pole Analysis could be a useful tool when using a central 2 × 2 sectors macular grid. Retinal changes in MS seem to start from the fovea and spread to the posterior pole. Finally, MS could lead to alterations in both the inner and outer retina, along with pRNFL. [ABSTRACT FROM AUTHOR]

This study aims to determine the relationship between intracranial pressure (ICP) and intraocular pressure (IOP) in patients with normal-tension glaucoma (NTG) who were already on anti-glaucoma treatment using an estimated ICP (estICP) and translaminar pressure difference (estTPD) formula. A cross-sectional comparative study consisted of 66 subjects (66 eyes) who were divided into NTG (n=33) and normal (n=33) group was conducted from 1st November 2017 until 31st May 2020 at a tertiary hospital in Malaysia. After obtaining consent from subjects, ocular and systemic data including IOP, visual field testing, axial length, central corneal thickness (CCT), peripapillary and macular retinal nerve fibre layer evaluation as well as blood pressure (BP) and body mass index (BMI) were collected. The estICP (mm Hg) was calculated as 0.44 x BMI (kg/m2) + 0.16 x diastolic blood pressure (mmHg) - 0.18 x age (years) - 1.91. The estTPD was derived from this calculated value, where estTPD (mm Hg) = IOP -- estICP. Analysis showed there was no significant difference in estimated ICP between NTG and normal subjects [mean difference (95% CI): 0.37 (-1.39, 2.12), p=0.679]. The difference in estTPD between NTG and normal subjects were found to be statistically insignificant too [mean difference (95% CI): -1.24 (-2.95, 0.47), p=0.149]. The variables significant in multivariate model included best corrected visual acuity (p=0.028), retinal nerve fiber layer (RNFL) (p=0.003), average macular (p=0.002) and estTPD (p=0.008). The EstTPD was found to be protective towards NTG, which the unit increased in estTPD will decreased the odds of having NTG by 26.5% [Adj. OR (95% CI): 0.735 (0.586, 0.922), p=0.008]. In conclusion, ICP was correlated in increased in IOP. A higher TPD may be associated with a lower chance of developing NTG. [ABSTRACT FROM AUTHOR]

A 60-year-old white woman presented to the emergency department with painless decrease of visual acuity in the left eye (LE). The diagnosis of a non-arteritic anterior ischemic optic neuropathy in the LE was established based on the clinical picture and the results of static perimetry, fluorescein angiography, visual evoked potential, and magnetic resonance imaging (MRI) of the brain and orbit. Six months later, the patient reported visual impairment in the right eye (RE). Best corrected visual acuity (BCVA) in the RE was 5/10. Gadolinium-enhanced MRI showing inflammation of both optic nerves and the optic chiasm in correlation with positivity for immunglobulin G antibody against aquaporin-4 led to the diagnosis of late-onset neuromyelitis optica spectrum disorder. High-dose intravenous methylprednisolone therapy followed by oral tapering was administered and oral azathioprine was started to reduce the risk of further relapse. At discharge, BCVA was 5/5 in the RE. The patient remains under the care of neurology and ophthalmology clinics, with no recurrences for two years. The possibility of neuromyelitis optica spectrum disorder with optic neuritis in older patients is important in the differential diagnosis of ischemic optic neuropathy. [ABSTRACT FROM AUTHOR]

Simple Summary: Patients with multiple myeloma (MM) who become refractory to three or more lines of therapy (RRMM patients) have few valid therapeutic alternatives. Among them, drugs directed against the BCMA antigen expressed in plasma cells are very appealing. Belantamab-mafodotin (belamaf) is the first antibody-drug conjugate against BCMA ready for clinical use. In this paper, we report the Spanish experience of belamaf monotherapy in 156 patients with RRMM. The overall response rate was 41.8%, with 39.8% of patients achieving a partial response or better. Median progression-free survival was 3.61 months, but interestingly, it increased to 14.47 months in patients achieving a minimal response or better. Treatment was well tolerated, ocular events being the most reported toxicity (87.9%; grade ≥ 3, 33.7%), but only two patients discontinued treatment due to side effects. Overall, our results confirm the safety and efficacy of belamaf in this poor prognosis subset of patients. Belantamab-mafodotin (belamaf) is a novel antibody-drug conjugate targeting B-cell maturation antigen that showed anti-myeloma activity in patients with relapsed and refractory multiple myeloma (RRMM). We performed an observational, retrospective, and multicenter study aimed to assess the efficacy and safety of single-agent belamaf in 156 Spanish patients with RRMM. The median number of prior therapy lines was 5 (range, 1–10), and 88% of patients were triple-class refractory. Median follow-up was 10.9 months (range, 1–28.6). The overall response rate was 41.8% (≥CR 13.5%, VGPR 9%, PR 17.3%, MR 2%). The median progression-free survival was 3.61 months (95% CI, 2.1–5.1) and 14.47 months (95% CI, 7.91–21.04) in patients achieving at least MR (p < 0.001). Median overall survival in the entire cohort and in patients with MR or better was 11.05 months (95% CI, 8.7–13.3) and 23.35 (NA-NA) months, respectively (p < 0.001). Corneal events (87.9%; grade ≥ 3, 33.7%) were the most commonly adverse events, while thrombocytopenia and infections occurred in 15.4% and 15% of patients, respectively. Two (1.3%) patients discontinued treatment permanently due to ocular toxicity. Belamaf showed a noticeably anti-myeloma activity in this real-life series of patients, particularly among those achieving MR or better. The safety profile was manageable and consistent with prior studies. [ABSTRACT FROM AUTHOR]

Objective: Neurofibromatosis type 1 (NF1) is the most common hereditary neurocutaneous syndrome. The most crucial morbidity of NF1 is tumors that may develop. Cases with café-au-lait macules (CALMs) which is the first clinical finding of NF1, due to the anxiety of its associated morbidity, are referred to the pediatric oncology clinic. In this study, we aimed to examine the characteristics of the patients who applied to our outpatient clinic with CALMs. Patients and Methods: The data of 157 pediatric patients who applied to our institution with the diagnosis of CALMs between June 2010 and November 2020 were analyzed retrospectively. Results: There were 157 pediatric cases referred to us for CALMs. According to the National Institutes of Health (NIH) diagnostic criteria, 109 (69.4%) cases were diagnosed with NF1. The diagnosis of 22 cases with NF1 were supported by genetic examination. Optic glioma was detected in 39 (24.8%) cases. In 15 (38.4%) of cases with optic glioma, visual functions were also affected. Second diagnostic criterion did not develop during the follow-up period, except for macules, in 48 cases (30.5%). Conclusion: In cases with multiple CALMs, the probability of NF1 diagnosis is high, and close and regular follow-up is of great importance in catching the development of the second clinical criterion and minimizing its morbidity. [ABSTRACT FROM AUTHOR]

Introduction: Children with optic nerve hypoplasia (ONH) frequently have co-occurring developmental disorders, and children with both visual impairments and autism spectrum disorder (ASD) have complex educational needs. Research Question: Do the individualized education programs (IEPs) of children with visual impairment and ASD comport with national educational guidelines? Methods: Participants were 13 children enrolled in a larger longitudinal study of ONH. Children were assessed for ASD, verbal intellectual functioning, and level of visual impairment. An IEP coding manual was developed by the authors and used to compare the IEP goals, services, and accommodations to national guidelines for the education of children with visual impairment and children with ASD. Results: Key findings indicated that needs in the domains of academics, assistive technology, and orientation and mobility were addressed, but other crucial areas of development such as social skills, social-emotional and behavioral needs, and skills for independent living were commonly overlooked. Discussion: It is recommended that IEP teams pay greater attention to the complex range of needs of children with visual impairments and that national guidelines for the education of children with visual impairment and ASD be incorporated into federal special education law. Implications for Practitioners: National guidelines for the educational needs of children with visual impairments and with ASD provide important guidance for IEP teams when developing goals, services, and supports, and efforts should be made to ensure educators and parents are aware of them. Children who have both visual impairments and ASD have complex educational needs that are often not fully addressed in IEPs. In particular, social-emotional, behavioral, and developmental needs may be overlooked by IEP teams, and consistent inclusion of school psychologists or other mental health professionals on IEP teams may lead to greater attention to these areas of need. [ABSTRACT FROM AUTHOR]

We present the largest study of the frequency and nature of visual complications in a cohort of 350 patients consecutively diagnosed with giant cell arteritis (GCA). Methods All individuals were assessed using structured forms and diagnosed using imaging or biopsy. A binary logistic regression model was used to analyse data for predicting visual loss. Results Visual symptoms occurred in 101 (28.9%) patients, with visual loss in one or both eyes in 48 (13.7%) patients. Four patients had binocular visual loss. Anterior ischaemic optic neuropathy (N=31), retinal artery obstruction (N=8) and occipital stroke (N=2) were the main causes of visual loss. Of the 47 individuals who had repeat visual acuity testing at 7 days, three individuals had improvement to 6/9 or better. After introducing the fast-track pathway, the frequency of visual loss decreased from 18.7% to 11.5%. Age at diagnosis (odds ratio (OR) 1.12) and headache (OR 0.22) were significant determinants of visual loss in a multivariate model. Jaw claudication trended to significance (OR 1.96, p=0.054). Conclusions We recorded a visual loss frequency of 13.7% in the largest cohort of patients with GCA examined from a single centre. Although improvement in vision was rare, a dedicated fasttrack pathway reduced visual loss. Headache could result in earlier diagnosis and protect against visual loss. [ABSTRACT FROM AUTHOR]

The study aims to assess the thickness of the retinal outer nuclear layer (ONL), ellipsoid zone (EZ) and photoreceptor outer segment (POS) band in various macular regions and its associations with axial length and other parameters. Participants of the Beijing Eye Study 2011 underwent a series of examinations including spectral-domain optical coherence tomography of the macula. The current study included 2213 participants without retinal or optic nerve diseases (age: 61.7 ± 8.4 years; range 50–93 years); axial length: 23.15 ± 0.95 mm; range 18.96–29.15 mm). The ONL (fovea: 98.9 ± 8.8 µm), EZ (fovea: 24.1 ± 0.5 µm) and POS band (fovea: 24.3 ± 3.5 µm) were the thickest (P < 0.001) in the fovea (defined as the thinnest central point), followed by the temporal inner, nasal inner, inferior inner, superior inner, inferior outer, temporal outer, nasal outer, and superior outer region. In multivariable analysis, a thicker retinal ONL was associated (correlation coefficient r: 0.40) with shorter axial length (beta: − 0.14; P < 0.001) and shorter disc–fovea distance (beta: − 0.10; P = 0.001), after adjusting for younger age (beta: − 0.26; P < 0.001), male sex (beta: 0.24; P < 0.001), lower serum cholesterol concentration (beta: − 0.05; P = 0.04), and thicker subfoveal choroidal thickness (beta: 0.08; P < 0.001). The POS thickness increased with shorter axial length (beta: − 0.06; P < 0.001) and shorter optic disc–fovea distance (beta: − 0.05; P = 0.03), after adjusting for younger age (beta: − 0.34; P < 0.001), male sex (beta: 0.15; P < 0.001), and thicker subfoveal choroidal thickness (beta: 0.24; P < 0.001). As a conclusion, the photoreceptor ONL, EZ and POS band vary in thickness between different macular regions and differ in their correlations with axial length, disc–fovea distance, age, sex, and subfoveal choroidal thickness. The ONL thickness decrease with longer axial length and longer disc–fovea distance may point to an axial elongation-associated retinal stretching in the macula. [ABSTRACT FROM AUTHOR]

Congenital Stationary Night Blindness (CSNB) constitutes a group of non-progressive retinal disorders characterized by disturbances in scotopic vision and/or by a delay in adaptation to darkness, as well as by low visual acuity, myopia, nystagmus, and strabismus. Color vision and fundus appearance tend to be normal. To date, several CACNA1F gene variants have been linked to a CSNB phenotype but only few reports have focused on the optic nerve in this disease. Twelve patients underwent standard ophthalmological and genetic evaluation including spectral domain optical coherence tomography (SD-OCT), full-field electroretinography (ffERG), kinetic perimetry, fundus photography, magnetic resonance imaging (MRI), and next-generation sequencing (NGS). Bilateral thinning of the peripapillary nerve fiber layer (pRNFL) and the ganglion cell complex (GCC) supported involvement of the optic nerves. MRI, when available, was assessed for gross intracranial optic pathway abnormalities. All patients were shown to carry pathogenic variants in the CACNA1F gene, and all showed signs of optic nerve involvement. All patients showed a certain degree of myopic refractive error. Low average pRNFL thickness was evident in all patients. In three of them, pRNFL thickness was evaluated longitudinally and was proven to be stable over time. MRI imaging was unremarkable in all cases. Our data support the hypothesis that CACNA1F could be related to early-onset or congenital optic nerve involvement without any signs of a progressive optic neuropathy. Even though additional data from larger cohorts and longer follow-up periods are needed to further support and confirm our findings, there is a clear significance to our findings in the preparation for future CACNA1F gene therapy trials. [ABSTRACT FROM AUTHOR]

Objectives: We investigated choroid plexus (CP) volume in patients presenting with optic neuritis (ON) as a clinically isolated syndrome (CIS), compared to a cohort with established relapsing–remitting multiple sclerosis (RRMS) and healthy controls (HCs). Methods: Three-dimensional (3D) T1, T2-FLAIR and diffusion-weighted sequences were acquired from 44 ON CIS patients at baseline, 1, 3, 6 and 12 months after the onset of ON. Fifty RRMS patients and 50 HCs were also included for comparison. Results: CP volumes was larger in both ON CIS and RRMS groups compared to HCs, but not significantly different between ON CIS and RRMS patients (analysis of covariance (ANCOVA) adjusted for multiple comparisons). Twenty-three ON CIS patients who converted to clinically definite MS (MS) demonstrated CP volume similar to RRMS patients, but significantly larger compared to HCs. In this sub-group, CP volume was not associated with the severity of optic nerve inflammation or long-term axonal loss, not with brain lesion load. A transient increase of CP volume was observed following an occurrence of new MS lesions on brain magnetic resonance imaging (MRI). Interpretation: Enlarged CP can be observed very early in a disease. It transiently reacts to acute inflammation, but not associated with the degree of tissue destruction. [ABSTRACT FROM AUTHOR]

The article presents a review and analysis of scientific works on the problem of chronic relapsing inflammatory optic neuropathy, its diagnostic criteria and treatment methods. Chronic relapsing inflammatory optic neuropathy, first described in 2003, is an autoimmune inflammatory disease of the optic nerve of unknown etiology, accompanied by frequent painful episodes of optic neuritis, with a clear response to steroid therapy and relapses after its withdrawal. An analysis of foreign works published in the E-Library, CrossRef, PubMed, Web of Science, Scopus databases was carried out in order to systematize data on the etiology, pathogenesis, clinical manifestations, diagnostic criteria, differential diagnosis and treatment of this pathological condition. It has been established that chronic recurrent inflammatory optic neuropathy is a rare, recurrent, corticosteroid-dependent optic neuropathy in which there is no other neurological deficit, no etiological factor is identified and it is a diagnosis of exclusion. It is important to identify such patients because the appointment of appropriate immunosuppressive therapy causes remission of the disease. [ABSTRACT FROM AUTHOR]

Simple Summary: Retinopathy and optic neuropathy are well-known, severe ocular complications in patients undergoing radiation therapy for brain, head, and neck cancer. However, little is known about the prevalence and dose–response relationship of retinopathy and optic neuropathy in these patients. More knowledge about the prevalence and dose–response relationship may contribute to developing a high-precision radiation therapy approach. Background: Patients with brain, head, and neck tumors experience a decline in their quality of life due to radiation retinopathy and optic neuropathy. Little is known about the dose–response relationship and patient characteristics. We aimed to systematically review the prevalence of radiation retinopathy and optic neuropathy. Method: The primary outcome was the pooled prevalence of radiation retinopathy and optic neuropathy. The secondary outcome included the effect of the total radiation dose prescribed for the tumor according to the patient's characteristics. Furthermore, we aimed to evaluate the radiation dose parameters for organs at risk of radiation retinopathy and optic neuropathy. Results: The pooled prevalence was 3.8%. No retinopathy was reported for the tumor's prescribed dose of <50 Gy. Optic neuropathy was more prevalent for a prescribed dose of >50 Gy than <50 Gy. We observed a higher prevalence rate for retinopathy (6.0%) than optic neuropathy (2.0%). Insufficient data on the dose for organs at risk were reported. Conclusion: The prevalence of radiation retinopathy was higher compared to optic neuropathy. This review emphasizes the need for future studies considering retinopathy and optic neuropathy as primary objective parameters. [ABSTRACT FROM AUTHOR]

Introduction: This retrospective study aims to describe the pattern of visual field defects among patients presenting with neuro-ophthalmological diseases in a tertiary hospital of Kathmandu. Materials and Methods: Medical records of patients undergoing Goldmann visual fields (GVF) for neuroophthalmological diseases from July 2019 to June 2021 were retrospectively reviewed. Patient demographics, laterality of the disease, clinical diagnosis and pattern of visual field defect were evaluated and entered and analyzed using SPSS version 20 using descriptive univariate and bivariate analysis. Results: A total of 216 subjects (378 eyes) with the mean age 34.52±14.80 years were included in the study. The visual field defects were divided 4 categories: optic nerve disorders (76.4%, n=165), chiasmal disorders (17.6%, n=38), retrochiasmal disorders (2.8%, n=6) and miscellaneous (3.2%, n=7). Papilledema was the most common diagnosis in optic nerve disorders with enlarged blind spot (79.1%) and constriction of visual fields (18.3%) as pattern of visual field defects followed by optic neuritis. Common lesions affecting the chiasma in our study were pituitary lesions where 30% showed constriction of peripheral fields, 57.5% showed temporal field defects (mostly superotemporal) in both eyes and craniopharyngioma on whom 50% had the classical temporal field involvement (bitemporal-20%, inferotemporal-20% and superotemporal-10%) Retrochiasmal lesions were less common but all showed a bilateral and homonymous pattern of visual field defect. Conclusion: Optic nerve disorders were the most common group of conditions undergoing visual fields. Understanding the varied patterns of visual field defects can aid in early diagnosis and guide timely treatment of common neuroophthalmological diseases. [ABSTRACT FROM AUTHOR]

Optic nerve damage is a common cause of blindness. Optic nerve injury is often accompanied by fundus vascular disease, retinal ganglion cell apoptosis, and changes in retinal thickness. These changes can cause alterations in protein expression within neurons in the retina. Proteomics analysis offers conclusive evidence to decode a biological system. Furthermore, animal models of optic nerve injury made it possible to gain insight into pathological mechanisms, therapeutic targets, and effective treatment of such injuries. Proteomics takes the proteome as the research object and studies protein changes in cells and tissues. At present, a variety of proteomic analysis methods have been widely used in the research of optic nerve injury diseases. This review summarizes the application of proteomic research in optic nerve injury diseases and animal models of optic nerve injury. Additionally, differentially expressed proteins are summarized and analyzed. Various optic nerve injuries, including those associated with different etiologies, are discussed along with their potential therapeutic targets and future directions. [ABSTRACT FROM AUTHOR]

Objective: Unusual or unexpected effect of treatment Background: Tocilizumab is a therapeutic biologic antagonist of the interleukin-6 (IL-6) receptor that has been approved to treat some autoimmune and chronic inflammatory diseases. This report is of a patient with a history of Graves disease and severe corticosteroid-unresponsive thyroid eye disease that included edema of the optic nerve and choroid, which was successfully treated with tocilizumab. Case Report: A 46-year-old woman with a 4-year history of Graves disease presented with acutely progressive bilateral severe optic nerve compression, severe bilateral optic nerve edema, bilateral restriction of eye movement, and bilateral choroidal folds. The patient was managed with an initial high dose of systemic steroid without any success. She then underwent bilateral orbital wall decompression without any noticeable amelioration. She subsequently received 4 doses of a tocilizumab (8 mg/kg) infusion. This resulted in a considerable decrease in inflammatory signs and improvement in optic nerve function, thereby improving her quality of life. The patient did not have any adverse reactions to the tocilizumab. Conclusions: The findings from this case support recent case reports and clinical trials indicating that tocilizumab may be effective in corticosteroid-resistant thyroid eye disease associated with autoimmune hyperthyroidism. [ABSTRACT FROM AUTHOR]

Simple Summary: Nowadays, there is no univocal therapeutical care for children with optic pathway gliomas (OPG): different chemotherapy regimens are proposed, but no one has clearly proved its superiority over the others on the PFS (Progression free survival). The efficacy of bevacizumab, an anti-VEGF monoclonal antibody, used in combination with Irinotecan, has been raised by several recent publications. However, Irinotecan has demonstrated side effects, especially digestive. Our main goal is to understand if bevacizumab could be efficacious used as a single agent against OPG. This is a retrospective study conducted on patients with OPG, aged less than 19 years, treated with bevacizumab as a single agent, since 2010 at IHOPe (Institute of Pediatric Hematology and Oncology). Efficacy of the treatment was evaluated on the tumor response rate on MRI with a centralized review basing upon RAPNO criteria and with visual assessment basing upon a 0.2 log change in the logMAR scale. Thirty-one patients with OPG have been included. From a radiological point of view, best anytime responses were: 1 major response, 6 partial responses, 7 minor responses and 14 stable diseases; achieving disease control in 28 (96%) out of 29 patients. Ophthalmological response was evaluated in 25 patients and disease control was achieved in 22 (88%) out of 25, with 14 steady states and 8 significant improvements. Among patients treated with chemotherapy after the bevacizumab course, nine relapsed and have been retreated with objective responses. Bevacizumab used as single agent seems effective in children and adolescents with OPG. Our work paves the way for a phase II study in which bevacizumab alone could be used as frontline therapy. [ABSTRACT FROM AUTHOR]