Optic Neuritis Research Articles

Reversible visual recovery in Optic Neuritis patient with No Light Perception VA

Abstract
 Introduction : Optic neuritis is characterized by an inflammation and demyelination of the optic nerve and is traditionally treated with corticosteroids. The findings of the ONTT, reported in more than 50 publications during the past 20 years, have numerous implications
 Case Illustration : A 48-year-old woman presented to the ophthalmology outpatient clinic with a 12-day history of blurred vision. The best-corrected visual acuities and Ishihara colour vision was unilaterally reduced with a non light perception on left eye. There were also relative afferent pupillary defect with slow respons of pupillary reflect. Slit-lamp examination was otherwise normal. Funduscopy was Papillary edema on left eye, with all quadrant blurry edge. Optic neuritis (ON) was presumed as she had suffered with this previously. She was hospitalized and undergone (optic neuritis treatment trial) ONTT . Thereafter, a trial of pulsed methylprednisolone was commenced, resulting in near complete resolution of the Visual acuity that become 6/40 UCVA from NLP.
 Discussion : The original ONTT, along with three follow-up studies at five-year intervals, generated more than three dozen papers in scientific journals. Collectively they show what happens to an individual over the course of 15 years following about of optic neuritis.
 Conclusion : Prior to the ONTT well-established guidelines for treating optic neuritis did not exist, this case were demonstrates the importance of keep starting ONTT with a point valuable to patient with NLP.

Orbital Tumor Misdiagnosed as Optic Neuritis in Rheumatoid Arthritis Patient: A Case Report

Abstract
 Introduction : Unilateral blurred vision and optic nerve swelling are the two most common signs of optic neuritis (ON). However, ON is rarely reported in patients with rheumatoid arthritis (RA), thus necessitating further diagnostic workup.
 Case Illustration : A 42-year-old woman visited our hospital with strabismus and progressive loss of vision in her left eye since three months ago. She had been treated with pulse dose steroid for suspected ON. She had been diagnosed with RA over five years and treated with immunomodulatory medication. Left eye examination revealed best corrected visual acuity of 20/50, proptosis, esotropia with painless limitation in temporal movement. Afferent pupillary defect (RAPD) and optic nerve swelling were present. OCT assessment revealed reduction in RNFL thickness. Further magnetic resonance imaging (MRI) investigation revealed a 2.35 x 2.6 x 1.8 cm mass in the left intraconal retrobulbar, in proximity to the medial optic nerve, which suggests a case of orbital Schwanoma. Excisional biopsy was performed and revealed a benign tumor.
 Discussion : Intraconal tumor may falsely be diagnosed as an optic neuritis in autoimmune patient. Neuro- ophthalmologic symptoms of systemic inflammatory in the optic nerve includes unilateral acute visual loss and pain in eye movement. Signs of ON includes RAPD, disc edema, and corresponding visual field defect. MRI of the optic nerve can assist diagnostic workup.
 Conclusion : The exact diagnosis of rheumatoid arthritis-associated ocular manifestations is challenging. Imaging using MRI might be needed to provide clarity in the diagnosis.

Searching The New Culprit of Optic Neuritis: The Role of Vitamin D

Abstract
 Introduction : Low vitamin D status has long been associated in multiple sclerosis patient with higher risk of disease progression and frequent relapses. Vitamin D plays an important role in modulating the immune process responsible for demyelination. To this date, the study on vitamin D deficiency in optic neuritis case is still rare.
 Case Illustration : Female, 20 years old, presenting with bilateral sudden visual loss since 1 week prior. The patient looked relatively pale and complained of accompanying headache and myalgia. Visual acuity in both eyes were light perception with dilated pupils and posterior segment showing optic disc swelling. CBC, infection and autoimmune screening came within normal limit and brain MRI further confirmed the presence of optic neuritis. The patient was given high dose intravenous steroid but showed no improvement. Later, she was screened for nutrient deficiency and found sign of low serum 25-hydroxyvitamin D. After one week of oral supplementation and neuroprotective agents, her vision drastically improved to 6/6 in both eyes.
 Discussion : Vitamin D deficiency nowadays is getting more prevalent in high risk individuals with low dietary intake and sunlight exposure. Recent study found that mean serum vitamin D level were significantly lower in autoimmune optic neuritis (ON) and were associated with ON attack severity. Vitamin D sufficiency is associated with better inflammatory outcome and long term neurodegenerative measures in demyelinating diseases, as shown by improvement in RNFL thickness after attack.
 Conclusion : Screening for vitamin D deficiency is essential to consider in managing patient with atypical optic neuritis.

Neuromyelitis Optica Spectrum Disorder (NMOSD) or Chronic-Relapsing Inflammatory Optic Neuropathy (CRION)?: A Challenge in Diagnosis and Treatment

Abstract
 Introduction : Neuromyelitis optica spectrum disorder (NMOSD) is a chronic inflammatory, immune-mediated central nervous system disease targeting the AQP4-water channel membrane protein of the optic nerve and the spinal cord. This case report aims to present a case of seroconverting NMOSD which was initially diagnosed as chronic relapsing inflammatory optic neuropathy (CRION).
 Case Illustration : 15-year-old female with recurrent, steroid-dependent optic neuritis of the LE. During the first attack, brain MRI showed optic nerve inflammation with normal brain findings. Initial test for AQP4-IgG, MOG-Ab and ANA profile were negative. The diagnosis of CRION was made. Treatment with corticosteroid and immunosuppressant agent was maintained for one year. After treatment cessation, however, LE optic neuritis recurred with bilateral lower limb weakness. Spinal MRI showed signs of longitudinal extensive transverse myelitis. Re-testing for AQP4-IgG showed positive result. Diagnosis of NMOSD was made, and the patient was treated with high-dose intravenous corticosteroid followed by oral dose, and immunosuppressant. Final visual acuity of the LE was 1.0, with minimal residual visual field defect and good motor function.
 Discussion : During the initial attack, the patient did not meet the 2015 NMOSD international consensus diagnostic criteria. The AQP4-IgG was negative and no other corresponding signs and symptoms. The criteria was met one year after: optic neuritis, LETM and positive seroconversion of AQP4-IgG.
 Conclusion : Diagnosing NMOSD is at times challenging especially during initial attack. Retesting of the AQP4- IgG is at times necessary in seronegative patients, especially in children showing ADEM-like event with optic neuritis and LETM to achieve diagnosis and proper treatment strategy.

OCULAR INVOLVEMENT IN SNAKEBITE PATIENT: A CASE TO REMEMBER

Introduction : Snake bite cases in Indonesia are not rare with mild to catastrophic consequences on the bitten subject due to its hemolytic and neurotoxic effects. This case represents a rare ocular neurotoxic effect of snake’s venom that cause binocular diplopia and ptosis in a child.
 Case Illustration : A 9 year-old boy was consulted to ophthalmologist with chief complaint of binocular diplopia. He was hospitalized after being bitten by a snake on his right hand and had an episode of delirious state. Ophthalmologic examination revealed ptosis and exotropia of his left eye in primary position. Further examination of anterior and posterior segments showed good results. His visual acuity was unaffected. Intravenous antivenom was administered and the bite wound was taken care. The boy was discharged from the hospital with a good general condition but still an opthalmoplegia. After a week of follow up the diplopia diminished and his ocular motility was recovered.
 Discussion : Ocular manifestations in snake bite case might be missed because of delirious state of the patients. Snake venom can cause ptosis and ophthalmoplegia (neurotoxic) along with uveitis, optic neuritis, central retinal artery occlusion or vitreous and retina hemorrhage due to its hemolytic effect. Time interval between the bite and treatment is one of crucial factor that indicate the patient’s prognosis.
 Conclusion : Thorough examination of anterior to posterior ocular segments is needed in every snake bitten patients to rule out any further ocular complication. An early administration of intravenous antivenom is still a mandatory.

Neuro-ophthalmic complications of modern anti-cancer drugs.

Targeted cancer therapies have been responsible for a dramatic shift in treatment strategies for cancer, and the number of drugs, classes, and indications are continually growing. Neuro-ophthalmic complications of these medications are an uncommon but important subset of adverse events which profoundly impact vision. This review aims to collate studies and reports of known neuro-ophthalmic complications of targeted therapies and describe their management. The anti-cancer drugs included in the review were any drugs targeting specific molecules involved in the cancer disease process. PubMed, EMBASE, and Web of Science were searched using the generic names of each drug and keywords of neuro-ophthalmic conditions. The prescribing information published by the US Food and Drug Administration (FDA) for each drug was also reviewed. Several classes of targeted anti-cancer drugs were found to cause neuro-ophthalmic adverse effects. Immune checkpoint inhibitors are responsible for a raft of immune-related adverse events such as optic neuritis, ischemic optic neuropathy, PRES, and myasthenia gravis. Therapies with anti-VEGF activity can provoke posterior reversible leukoencephalopathy, which commonly presents with visual loss and can be fatal if not treated promptly. Inhibitors of BCR-ABL1, VEGF, ALK, and proteasomes have all been linked to optic nerve disorders which can have debilitating consequences for vision. The neuro-ophthalmic complications of modern anti-cancer drugs can limit or necessitate the withdrawal of these life-prolonging medications. Ophthalmologists should be alert for neuro-ophthalmic complications in these medications to facilitate prompt diagnosis and treatment and reduce the risk of severe and permanent consequences.

Neuromyelitis Optica with Negative AQP-4 Antibody

Abstract
 Introduction : Neuromyelitis Optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease of the central nervous system that causes demyelination of the optic nerve and spinal cord. Approximately 75% of cases have antibodies against aquaporin 4 (AQP-4 Antibody).
 Case Illustration : A 42 years old male presented with progressing blurred vision in his right eye for 3 weeks. He described there was a black spot in his central vision which gradually enlarged to his peripheral vision, headache for 4 months, and transient tingling sensation and weakness on the left arm for 1 week. Positive RAPD in the right eye and signs of optic neuritis on funduscopy in Opthalmology examination. Campimetry examination revealed generalized depression in the right eye. The visual evoked potential (VEP) was consistent with right optic nerve demyelination. Spine MRI showed extensive transverse myelitis lesions >3 spinal cord hyperintensity on the level of C2-C6. However, the serum AQP-4 antibody was negative. The patient was treated with corticosteroid, and neuroprotector and was also being treated alongside the neurology department.
 Discussion : NMO is a rare autoimmune disease. NMO is characterized by segmented demyelination and inflammation of the spinal cord and the optic nerves which gives many ocular signs and symptoms like optic neuritis. A negative test result does not rule out the diagnosis of NMO. Brain and spine MRI plays an important role in diagnosing NMO. Treatment of NMO consists of corticosteroid and plasmapheresis or intravenous immunoglobulin.
 Conclusion : This case describes a diagnosis of NMO with a Negative AQP-4 antibody.

OCULAR INVOLVEMENT IN SNAKEBITE PATIENT: A CASE TO REMEMBER

Introduction : Snake bite cases in Indonesia are not rare with mild to catastrophic consequences on the bitten subject due to its hemolytic and neurotoxic effects. This case represents a rare ocular neurotoxic effect of snake’s venom that cause binocular diplopia and ptosis in a child.
 Case Illustration : A 9 year-old boy was consulted to ophthalmologist with chief complaint of binocular diplopia. He was hospitalized after being bitten by a snake on his right hand and had an episode of delirious state. Ophthalmologic examination revealed ptosis and exotropia of his left eye in primary position. Further examination of anterior and posterior segments showed good results. His visual acuity was unaffected. Intravenous antivenom was administered and the bite wound was taken care. The boy was discharged from the hospital with a good general condition but still an opthalmoplegia. After a week of follow up the diplopia diminished and his ocular motility was recovered.
 Discussion : Ocular manifestations in snake bite case might be missed because of delirious state of the patients. Snake venom can cause ptosis and ophthalmoplegia (neurotoxic) along with uveitis, optic neuritis, central retinal artery occlusion or vitreous and retina hemorrhage due to its hemolytic effect. Time interval between the bite and treatment is one of crucial factor that indicate the patient’s prognosis.
 Conclusion : Thorough examination of anterior to posterior ocular segments is needed in every snake bitten patients to rule out any further ocular complication. An early administration of intravenous antivenom is still a mandatory.

Swollen Disc in Children : Diagnostic Approach

Swollen disc is a clinical sign of eye disease, neurological or systemic. It happened because of the increase in intracranial. Some of the conditions that cause swollen disc are optic neuritis, optic neuropathy, retinal vein occlusion, papilloedema, or pseudo-disc swelling (e.g drusen). In children, the incidence of swollen discs differs from that in adults. Diagnosis can be made using OCT (Optical Coherence Tomography), ocular ultrasound, VEP (Visual Evoked Potential) and MRI (Magnetic Resonance Imaging) of the orbit and head

Assessment of the Quality, Accountability, and Readability of Online Patient Education Materials for Optic Neuritis

ABSTRACT Most cases of optic neuritis (ON) occur in women and in patients between the ages of 15 and 45 years, which represents a key demographic of individuals who seek health information using the internet. As clinical providers strive to ensure patients have accessible information to understand their condition, assessing the standard of online resources is essential. To assess the quality, content, accountability, and readability of online information for optic neuritis. This cross-sectional study analyzed 11 freely available medical sites with information on optic neuritis and used PubMed as a gold standard for comparison. Twelve questions were composed to include the information most relevant to patients, and each website was independently examined by four neuro-ophthalmologists. Readability was analyzed using an online readability tool. Journal of the American Medical Association (JAMA) benchmarks, four criteria designed to assess the quality of health information further were used to evaluate the accountability of each website. Freely available online information. On average, websites scored 27.98 (SD ± 9.93, 95% CI 24.96–31.00) of 48 potential points (58.3%) for the twelve questions. There were significant differences in the comprehensiveness and accuracy of content across websites (p < .001). The mean reading grade level of websites was 11.90 (SD ± 2.52, 95% CI 8.83–15.25). Zero websites achieved all four JAMA benchmarks. Interobserver reliability was robust between three of four neuro-ophthalmologist (NO) reviewers (ρ = 0.77 between NO3 and NO2, ρ = 0.91 between NO3 and NO1, ρ = 0.74 between NO2 and NO1; all p < .05). The quality of freely available online information detailing optic neuritis varies by source, with significant room for improvement. The material presented is difficult to interpret and exceeds the recommended reading level for health information. Most websites reviewed did not provide comprehensive information regarding non-therapeutic aspects of the disease. Ophthalmology organizations should be encouraged to create content that is more accessible to the general public.

Relationship between pattern reversal visual evoked potential P100 wave latency and dysfunctional HDL in patients with multiple sclerosis subjected to an optic neuritis attack: A case-control study.

Optic neuritis frequently occurs during the clinical course of multiple sclerosis (MS). In this condition, demyelination of the optic nerve occurs, which electrophysiologically causes a delay in P100 wave latency. Sensitive cholesterol homeostasis is critical for the formation of the myelin sheath and for myelin to become functionally mature. High-density lipoprotein (HDL) becomes dysfunctional under oxidative stress and plays an important role in the pathogenesis of MS. In this study, HDL levels of MS patients suffering from optic neuritis were compared with those of healthy individuals, and the relationship between pattern reversal visual evoked potential (PRVEP) P100 wave latency and HDL levels in patients with optic neuritis attacks was analyzed. PRVEP studies were performed in patients with MS who had an episode of optic neuritis, and P100 wave latencies were measured. Peripheral blood samples were collected from healthy participants and patients. Lipid levels and myeloperoxidase (MPO) and paraoxonase (PON) activities were measured, and the MPO/PON ratio was then calculated. The lipid profiles and dysfunctional HDL levels in the healthy and patient groups were compared. Finally, the relationship between these parameters and the PRVEP-P100 wave latency was examined. Total cholesterol and low-density lipoprotein (LDL) levels were significantly higher in the patient group (P = .044; P = .038, respectively). There was no statistically significant difference in HDL levels between groups (P = .659). The distribution of MPO values was similar between groups (P = .452). PON values were significantly lower, whereas the MPO/PON ratios were significantly higher in the patient group than in the control group (P = .025; P = .028, respectively). A statistically significant positive correlation was found between the elevated MPO/PON ratio, representing dysfunctional HDL, and both the mean and maximum PRVEP-P100 wave latencies (P < .001, R = 0.690; P < .001, R = 0.815, respectively). A dysfunctional form of HDL may lead to poor deactivation of remyelination-limiting factors and may ultimately be associated with poor outcomes in optic neuritis.

Effectiveness of immunotherapies in relapsing myelin oligodendrocyte glycoprotein antibody-associated disease

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can cause optic neuritis, transverse myelitis, or acute disseminated encephalomyelitis (ADEM). Immunotherapy is often used for relapsing disease, but there is variability in treatment decisions. Objective: The objective was to determine the annualized relapse rates (ARRs) and incidence rate ratios (IRRs) compared to pre-treatment and relapse-freedom probabilities among patients receiving steroids, B-cell depletion (BCD), intravenous immunoglobulin (IVIG), and mycophenolate mofetil (MMF). Methods: Retrospective cohort study of patients with relapsing MOGAD treated at Mass General Brigham. ARRs and IRRs compared to pre-treatment, and relapse-freedom probability and odds ratio for relapse-freedom compared to prednisone were calculated. Results: A total of 88 patients met the inclusion criteria. The ARR on IVIG was 0.13 (95% confidence interval (CI) = 0.06–0.27) and the relapse-freedom probability after at least 6 months of therapy was 72%. The ARR on BCD was 0.51 (95% CI = 0.34–0.77), and the relapse-freedom probability was 33%. The ARR on MMF was 0.32 (95% CI = 0.19–0.53) and the relapse-freedom probability was 49%. In pediatric-onset disease, MMF had the lowest ARRs (0.15, 95% CI = 0.07–0.33). Conclusion: IVIG had the lowest ARRs and IRRs compared to pre-treatment and the highest relapse-freedom odds ratio compared to prednisone, while BCD had the lowest. In pediatric-onset MOGAD, MMF had the lowest ARRs.

Genetic susceptibility and causal pathway analysis of eye disorders coexisting in multiple sclerosis.

The comorbidity of optic neuritis with multiple sclerosis has been well recognized. However, the causal association between multiple sclerosis and optic neuritis, as well as other eye disorders, remains incompletely understood. To address these gaps, we investigated the genetically relationship between multiple sclerosis and eye disorders, and explored potential drugs. In order to elucidate the genetic susceptibility and causal links between multiple sclerosis and eye disorders, we performed two-sample Mendelian randomization analyses to examine the causality between multiple sclerosis and eye disorders. Additionally, causal single-nucleotide polymorphisms were annotated and searched for expression quantitative trait loci data. Pathway enrichment analysis was performed to identify the possible mechanisms responsible for the eye disorders coexisting with multiple sclerosis. Potential therapeutic chemicals were also explored using the Cytoscape. Mendelian randomization analysis revealed that multiple sclerosis increased the incidence of optic neuritis while reducing the likelihood of concurrent of cataract and macular degeneration. Gene Ontology enrichment analysis implicated that lymphocyte proliferation, activation and antigen processing as potential contributors to the pathogenesis of eye disorders coexisting with multiple sclerosis. Furthermore, pharmaceutical agents traditionally employed for allograft rejection exhibited promising therapeutic potential for the eye disorders coexisting with multiple sclerosis. Multiple sclerosis genetically contributes to the development of optic neuritis while mitigating the concurrent occurrence of cataract and macular degeneration. Further research is needed to validate these findings and explore additional mechanisms underlying the comorbidity of multiple sclerosis and eye disorders.

New insights in the pathogenic mechanism of multiple sclerosis: Is Epstein-Barr virus associated with optic nerve involvement?

There are no reports in the literature studying the possible relationship between Epstein-Barr virus (EBV) and optic nerve involvement in multiple sclerosis (MS). The aim of our study was to analyze the association between EBV antibodies titres and optical coherence tomography (OCT) and OCT angiography (OCTA) quantitative parameters. We conducted a retrospective study. The study included 98 eyes of 49 patients with MS. Years of MS duration, relapse count, history of optic neuritis (ON), and immunoglobulin (Ig) G antibodies to the EBV viral capsid antigen (VCA) were recorded from each patient. Also, OCT analysis (including retinal nerve fibre layer (RNFL) thickness and ganglion cell-inner plexiform layer (GCIPL) thickness) and OCTA analysis (including perfusion density (PD) and flux index (FI) of the radial peripapillary capillary plexus) were performed in each participant. No significant associations were observed between anti-EBV antibody levels and OCT or OCTA parameters (p > 0,05). Correlation analysis between OCT and OCTA measurements showed a significant positive correlation between RNFL thickness and GCIPL thickness with peripapillary PD and FI (p < 0,035). Subgroup analysis revealed a significant diminution of RNFL thickness, GCIPL thickness and peripapillary PD and FI (p < 0,05) in the ON group. We were unable to demonstrate a significant association between anti-EBV VCA IgG antibody titres and OCT or OCTA parameters. Nonetheless, further longitudinal studies are needed to explore the possible association of EBV with optic nerve involvement in MS.

Subnormal visual acuity after compliant amblyopia therapy: residual/refractory amblyopia or co-existing pathology? - a retrospective analysis

ABSTRACT Purpose: To assess the prevalence of alternate etiology/co-existing pathology among patients with amblyopia, and to characterize factors contributing to over-diagnosis of amblyopia. Methods: We retrospectively reviewed records of children (from 1 January 2016 to 31 December 2019) who were initially diagnosed as “amblyopia” but later an alternate diagnosis for subnormal vision was established. Patients who had a best corrected visual acuity (BCVA) of ≤20/32 (0.2 logMAR) after compliant amblyopia therapy were divided into 2 groups: those with refractory amblyopia (BCVA improvement from baseline <1 logMAR line) and residual amblyopia (BCVA improvement from baseline >1 logMAR line). Data was collected for presence/absence of amblyogenic risk factors, history, ocular examination, and investigations leading to the final alternate diagnosis. We analyzed the factors that contributed to the initial over-diagnosis of amblyopia using the diagnostic error evaluation and research (DEER) taxonomy tool. Results: During the study period, 508 children with an initial diagnosis of amblyopia met the study criteria. Among these 508 children, 466 were diagnosed to have amblyopia alone, while 26 children (5.1%, median age: 7 years, 17 boys: 9 girls) were revised to have an alternate diagnosis/co-existing pathology. These 26 patients comprised of 2 groups: children referred to us as amblyopia but rediagnosed to have an alternate diagnosis; and a second subset, initially diagnosed by us to have amblyopia, but later found to have alternate diagnosis/co-existing pathology. Subclinical optic neuritis (50%, 13 children), and occult macular dystrophy (OMD) (38.4%, 10 children) were the most frequent alternative diagnoses. Children with ametropic amblyopia (8/26, 30.7%) were most frequently misdiagnosed. Risk factors that led to an initial diagnosis of amblyopia were: high refractive error and heterotropia in 7 patients each (26.9%), anisometropia in 12 (46.1%), and prior pediatric cataract surgery in 4(15.3%). No improvement in BCVA in 21/26 (80.7%) children led to suspicion of co-existing etiology. Other clues were optic disc pallor (11), subnormal color vision (7), history of parental consanguinity in 7, and preceding febrile illness/rhinitis in 1 child. The DEER taxonomy tool suggested that the most common reasons for diagnostic errors were over-emphasis on amblyopia. Conclusion: Our study suggests that 5% of children diagnosed with amblyopia might have co-existing/alternate etiology. Most common co-existing etiologies were subclinical optic neuropathy, and OMD. No improvement in BCVA, subtle history and examination findings prompted further workup. Not considering co-existing etiologies was the most common reason for an initial overdiagnosis of amblyopia.

The Risk of Optic Neuritis following mRNA Coronavirus Disease 2019 Vaccination Compared to Coronavirus Disease 2019 Infection and Other Vaccinations

Purposeto determine the risk of optic neuritis (ON) after mRNA COVID-19 vaccine administration. DesignU.S. National aggregate database retrospective cohort study SubjectsPatients were placed into cohorts based on mRNA COVID-19 vaccination status (no vaccine and positive history of COVID-19 infection, one vaccine, or two vaccines received) from December 2020-June 2022. Two control cohorts were created with patients vaccinated against influenza or tetanus diphtheria and pertussis (Tdap) from June 2018-December 2019. Patients with any history of ON or significant risk factors for ON development including infectious, inflammatory, and neoplastic diseases were excluded. MethodsA large de-identified database was queried for the Common Procedural Technology (CPT) codes for immunization encounters specific to first dose and second dose of mRNA COVID-19 vaccine, influenza, or Tdap. Cohorts were 1:1 propensity score matched on age, sex, race, and ethnicity. The risk of ON development following vaccination was calculated and compared for all five cohorts with 95% confidence intervals (CIs) reported. Main outcome measuresrisk ratio (RR) of optic neuritis 21 days after vaccination (or COVID-19 infection) and incidence of ON per 100,000 individuals. ResultsAfter matching, the first dose COVID-19 and influenza vaccine cohorts (n=1,678,598, mean age [standard deviation] at vaccination of 45.5[23.3] and 43.2[25.5], 55% female) had a 0.44 risk reduction of developing ON (95% CI 0.28-0.80). The first dose COVID-19 and Tdap (n=797,538, mean age 38.9[20.0], 54.2% female) cohort had 10 and 16 patients develop ON (RR 0.63, 0.28-1.38). Comparison of COVID-19 vaccinated patients (n=3,698,848, 48.2[21.5] years, 54.7% female) to unvaccinated and COVID-19 infected patients (n=3,698,848, 49.6[22.0] years, 55.2% female) showed 49 and 506 patients developing ON respectively (RR 0.10, 0.07-0.13). The incidence per 100,000 for ON was 1 in the first dose COVID-19 vaccine cohort, 2 in the influenza and Tdap cohorts, and 14 in the COVID-19 infected and unvaccinated cohort. ConclusionRisk of ON following mRNA COVID-19 vaccination is rare and is comparable to Tdap vaccination, decreased compared to influenza vaccination, and decreased compared to COVID-19 infection in the absence of vaccination.

CNS Demyelination Syndromes Following COVID-19 Vaccination: A Case Series

ABSTRACT Background and Objectives: Although immunization against coronavirus disease 2019 (COVID-19) is ongoing, adverse reactions to these vaccinations have been observed in isolated cases. We aimed to report different neurological complications developed after COVID-19 vaccination. Materials and Methods: In our case series study, we report all cases of CNS demyelination following COVID-19 immunization. Clinical evaluation, brain MRI, and CSF analysis for oligoclonal bands and IgG index were performed for all patients. Other investigations were performed for selected patients, including spine MRI, EEG, VEP, and aquaporin-4. Results: Eighteen patients (eight males and ten females) with no history of COVID-19 infection had neurological manifestations (vertigo, ataxia, recurrent attacks of loss of consciousness, optic neuritis, and myelitis) starting within 14 days after Pfizer (n = 12) and AstraZeneca (n = 6) vaccination. MRI was obtained during the acute stage of the disease. The most common presenting symptoms were optic neuritis and hemiparesis. Sixteen patients had altered signal intensity and multiple variable-sized, round to ill-defined oval lesions suggestive of MS. Two showed findings compatible with transverse myelitis Conclusion: This study identified CNS demyelination complications after COVID-19 vaccination. The COVID-19 vaccination could result in CNS complications, possibly connected to a post-vaccination inflammatory process. We recommend continuous post-marketing monitoring for adverse reactions in individuals who received the vaccines to establish a connection and guarantee the long-term safety of COVID-19 vaccines.

A unique case of disseminated granulomatous disease associated with optic neuritis

A unique case of disseminated granulomatous disease associated with optic neuritis

Optic Neuritis after Infliximab Treatment in a Patient with Ulcerative Colitis

Optic neuritis is a rare extraintestinal manifestation of inflammatory bowel disease. Antitumor necrosis factor alpha (anti-TNF-α) agents are essential treatment options in inflammatory bowel diseases. However, anti-TNF-α agents can implicate optic neuritis and other demyelinating diseases as complications of these agents. We report a patient of infliximab-induced optic neuritis in a patient with ulcerative colitis who was treated with high-dose steroids after discontinuation of infliximab.

Advances in the long-term treatment of neuromyelitis optica spectrum disorder.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune neuroinflammatory disorder with a prevalence of 1-5/100,000 globally, characterized by attacks of the central nervous system including but not limited to optic neuritis, transverse myelitis and brainstem lesions, including area postrema lesions. These autoimmune attacks can lead to irreversible damage if left untreated, therefore strategies have been developed to prevent relapses. Initial off-label treatments have achieved variable levels of success in relapse prevention, but improved relapse prevention and quality of life remain a goal in the field. A better understanding of the underlying pathophysiology of NMOSD over the last 10years has led to newer, more specific approaches in treatment, culminating in the first FDA approved treatments in the disease. In this review, we will discuss the seminal trials of PREVENT or Eculizumab in the treatment of aquaporin-4 (AQP4)-IgG positive NMOSD, N-Momentum or Inebilizumab in the study of NMOSD (both AQP4-IgG positive and negative) and SAkura Sky and SAkuraStar which studied satralizumab in AQP4-IgG seropositive and seronegative NMOSD patients. We will also discuss the extension trials of each of these medications and what lead to their approval in AQP4-IgG seropositive NMOSD patients. We will then examine treatments in the pipeline for adult and pediatric NMOSD patients and conclude with discussions on treatment considerations in pregnant patients and how to approach treatment of NMOSD patients during COVID.