Unilateral Optic Neuritis Research Articles

Examining the relative contribution of slow-burning inflammation and chronic demyelination to axonal damage in chronic multiple sclerosis lesions

Background and ObjectivesSlow-burning inflammation at the edge, and chronic demyelination at the core, of established multiple sclerosis (MS) lesions are potential mediators of disease progression. However, their relative contribution to progressive axonal damage has not been explored. Therefore, in this study, we investigated the comparative contribution of slow-burning inflammation and chronic demyelination to axonal attrition within MS lesions by measuring progressive tissue rarefaction. In addition, we use the visual system as a model to investigate the effect of chronic demyelination on the acceleration of axonal death in a sub-group of patients with unilateral optic neuritis. MethodsPre- and post-gadolinium 3D-T1, 3D FLAIR, diffusion tensor images, Optical Coherence tomography and multifocal visual evoked potentials were acquired from 52 relapsing-remitting MS patients who completed at least 5 years follow-up.Lesion expansion was measured using custom software, and the rate of tissue rarefication inside lesion core was assessed by measuring increase of normalized mean diffusivity (nMD). Axonal loss was also examined in eyes with severe optic nerve demyelination. ResultsAmong the 361 lesions analyzed, 104 were expanding (a minimum of 4 % expansion per year) and 257 were stable. Expanding lesions showed a significantly higher rate of progressive tissue rarefication inside lesion (1.12 % per year) core compared to stable lesions (0.21 % per year, p = 0.01). The magnitude of nMD change was significantly correlated with the rate of lesion expansion (r = 0.4, p < 0.001). Analysis of retinal ganglion cells in eyes with severe optic nerve demyelination (Inter-eye latency delay of >10 ms) revealed a similar rate of axonal loss (0.19 %) to the degree of tissue rarefaction observed in stable lesions (0.21 %). DiscussionThe results of the study suggest that the slow-burning inflammation at the lesion's edge (as measured by lesion expansion), is likely to have a greater impact on tissue damage (as measured by nMD change), when compared to stable chronically demyelinated lesions. The similar modest degree of tissue damage was also observed in chronically demyelinated fibers of the optic nerve.

Leber’s Hereditary Optic Neuropathy Following Unilateral Syphilitic Optic Neuritis

This report describes a case of Leber’s hereditary optic neuropathy (LHON) following unilateral syphilitic optic neuritis. A 31-year-old man presented with a four-day history of decreased vision in his left eye. At the initial visit, the patient’s best corrected visual acuity (BCVA) was 1.2 and 0.02 in the right and left eye, respectively, along with a relative afferent pupillary defect in the left eye. Goldmann visual field testing revealed a central absolute scotoma and an enlarged blind spot in the left eye. Serological testing confirmed syphilis with rapid plasma reagin positivity. The patient was diagnosed with syphilitic optic neuritis and treated with intravenous penicillin G (24 million units daily) for two weeks. After two weeks of therapy, his left BCVA improved to 0.4 after two months. However, five months later, despite completing syphilis treatment, the patient developed acute painless visual loss in both eyes. His BCVA decreased to 0.8 and 0.05 in the right and left eyes, respectively. Goldmann visual field testing revealed a central absolute scotoma and an enlarged blind spot in both eyes. After two weeks, BCVA progressively declined to 0.4 in the right and 0.04 in the left eye. Furthermore, Goldmann visual field testing revealed an enlarged central absolute scotoma and blind spot in both eyes. Six months after the initial examination, his BCVA was counting fingers and 0.02 in the right and left eye, respectively. The optic discs appeared diffusely pale in both eyes. Genetic testing for LHON revealed a pathologic mtDNA 11778 point mutation. This case underscores the importance of considering the possibility of LHON if acute or subacute vision loss occurs subsequently or simultaneously in both eyes and remains unresponsive to treatment.

A Case of Unilateral Optic Neuritis in a 17-Year-Old Girl

A Case of Unilateral Optic Neuritis in a 17-Year-Old Girl

Disease Course of Clinically Isolated Optic Neuritis.

Optic neuritis is the most common optic neuropathy in young adults and a frequent manifestation of multiple sclerosis. Its clinical course is pertinent to the design of visual pathway neuroprotection trials. This is a secondary analysis of longitudinal data from the TONE trial, which included 103 patients from 12 German academic tertiary centers with acute unilateral optic neuritis as a clinically isolated syndrome and baseline high-contrast visual acuity <0.5 decimal. Patients were randomized to 1,000 mg methylprednisolone i.v./d plus either erythropoietin (33,000 IU/d) or placebo (saline solution) for 3 days. They were followed up at standardized intervals with a battery of tests including high-contrast visual acuity, low-contrast letter acuity, contrast sensitivity, visual fields, visual evoked potentials, and retinal optical coherence tomography. At 6 months, participants answered a standardized questionnaire on vision-related quality of life (NEI-VFQ 25). We describe the disease course with mixed-effects piecewise linear models and calculate structure-function correlations using Pearson r. Because erythropoietin had no effect on the visual system, we use pooled (treatment-agnostic) data. Patients experienced initial rapid and then decelerating improvements of visual function with thinning of inner and thickening of outer retinal layers. At 6 months, visual parameters were positively correlated with inner and negatively correlated with outer retinal thickness changes. Peripapillary retinal nerve fiber layer thinning predominantly occurred in sectors without previous swelling. At 6 months, macular ganglion cell and inner plexiform layer thinning was weakly correlated with the P100 peak time (r = -0.11) and moderately correlated with the amplitude of visual evoked potentials (r = 0.35). Only functional outcomes were at least moderately correlated with vision-related quality of life. The longitudinal data from this large study cohort may serve as a reference for the clinical course of acute optic neuritis. The pattern of correlation between visual evoked potentials and inner retinal thinning may argue that the latter is mostly due to ganglion cell loss, rather than dysfunction. Visual pathway neuroprotection trials with functional outcomes are needed to confirm that candidate drugs will benefit patients' vision-related quality of life. ClinicalTrials.gov, NCT01962571.

The MOG antibody non-P42 epitope is predictive of a relapsing course in MOG antibody-associated disease

BackgroundMyelin oligodendrocyte glycoprotein (MOG) IgG seropositivity is a prerequisite for MOG antibody-associated disease (MOGAD) diagnosis. While a significant proportion of patients experience a relapsing disease, there is currently no biomarker...

Unilateral Syphilitic Optic Neuritis without Uveitis

This report describes a case of case of unilateral syphilitic optic neuritis without uveitis. A 56-year-old man presented with complaints of four days history of decreasing vision in his left eye. At the initial visit, the patient’s best corrected visual acuity was 1.2 and 0.03 in the right and left eye, respectively. A relative afferent pupillary defect was observed in the left eye. Slit-lamp examination revealed no cells, flare or keratic precipitates in the anterior segment either eye. Fundoscopy revealed diffuse optic disc swelling with peripapillary hemorrhage in the left eye. Goldmann visual field testing identified central absolute scotoma and an enlarged blind spot in the left eye. On serological evaluation for syphilis, serum rapid plasma reagin (RPR) and treponema pallidum hemagglutination assay (TPHA) were positive (RPR 52.5, TPHA 740.0). In addition, IgM fluorescent Treponemal antibody absorbance (FTA-ABS) was positive. In contrast, cerebrospinal fluid showed negative RPR and positive TPHA (TPHA 11.8). Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis and increased protein content and glucose. Magnetic resonance imaging showed a swollen enhanced lesion in the left optic nerve. The patient was diagnosed with syphilitic optic neuritis and received the currently recommended treatment with intravenous penicillin G (PCG). Twenty-four million units per day of PCG were administered for 2 weeks. After 2 weeks of therapy, his left visual acuity improved to 0.3 in 2 months. However, the optic disc looked diffusely pale in the left eye. At 6-month visit, the patient was clinically stable. RPR turned out negative and there was a reduction in serological TPHA reactivity. We consider that it is essential to perform serological tests for syphilis in every patient with optic neuritis, which makes it possible to initially treat syphilitic optic neuritis with PCG.

Myelin oligodendrocyte glycoprotein (MOG) antibody positive disease presenting as acute disseminated encephalomyelitis and unilateral optic neuritis

The autoantibody against myelin oligodendrocyte glycoprotein (MOG-abs) is a recognized biomarker in acquired demyelinating syndromes. The diversity in clinical and neuroimaging phenotypes is still being understood in the paediatric population. The presenting clinical phenotype strongly depends on the age at onset. Acute disseminated encephalomyelitis (ADEM) and ADEM-like phenotypes are common in younger children, but optic neuritis (ON), often bilateral, is predominantly seen in females and older children and or adults.We present a 6.5-year-old boy with a rapid onset encephalopathy due to ADEM and a unilateral longitudinally affected optic neuritis detected on neuroimaging. His serum was positive for MOG-abs but negative for Aquaporin-4 antibodies. His cerebrospinal fluid was negative for oligoclonal bands. He gradually improved following treatment with intravenous high-dose methylprednisolone and five cycles of plasma exchange. Complete clinical recovery was achieved within twelve weeks of admission.Conclusion: Contrary to the usual older age of MOG-ON, and the bilateral involvement, this case illustrates a unilateral ON in a six-year-old boy occurring in conjunction with a MOG-ab positive encephalomyelitis.

Subclinical damage to the contralateral eye in unilateral optic neuritis: A longitudinal study.

Early detection of subclinical injuries can lead to a correct diagnosis and help control the advancement of the condition. This study aims to investigate the presence of subclinical damage and silent progression to the contralateral eye's visual function and structure in patients experiencing their first episode of unilateral optic neuritis (ON). Fifty patients with first-onset unilateral ON were enrolled in this study. Based on etiology, they were classified as having neuromyelitis optica spectrum disorder-related ON (NMOSD-ON), myelin oligodendrocyte glycoprotein antibody-associated ON (MOG-ON), idiopathic ON (IDON), or multiple sclerosis-related ON (MS-ON). These cases were followed up for one year to determine whether there was any silent progression of visual function and structure in the contralateral non-ON (NON) eye. A gender- and age-matched healthy control (HC) group was included to compare the differences in visual function and structure between the patients with NON eyes and the HC group. Within two weeks of onset, best-corrected visual acuity (BCVA; P=0.008), mean deviation (MD) of the visual field (VF) (P=0.001), and peripapillary retinal nerve fiber layer (pRNFL; P=0.019) thickness were significantly worse in the NMOSD-NON patients than those in the HC group, while there were no differences in the pRNFL and the ganglion cell-inner plexiform layer (GCIPL) thicknesses and quadrant thicknesses (P > 0.05) of the groups. IDON-NON only showed subclinical damage in VF (P=0.001) and temporal pRNFL (P=0.042), while the BCVA, VF, and optic nerve structure (pRNFL, GCIPL) of the MOG-NON patients showed no subclinical damage (P > 0.05). In addition, the one-year follow-up of each NON eye type showed that there was no silent progression in NMOSD-NON, MOG-NON, or IDON-NON. A pairwise comparison of the different types of NON eyes revealed no statistical differences (P > 0.05). Among the patients with unilateral ON, NMOSD-NON and IDON-NON resulted in subclinical damage to the visual function and structure of the contralateral eye within two weeks of onset, whereas MOG-NON did not show any subclinical damage to visual function or structure. Furthermore, these subclinical damages did not show any silent progression during the one-year follow-up period.

Multiple sclerosis: Unveiling current immunogenetic factors and their role in etiopathogenesis and clinical aspects

Multiple sclerosis (MS) is the most common cause of neurological deficits among the young population. While the prevalence of MS is increasing worldwide, the incidence rate of MS is also undergoing a similar trend in Lithuania. Globally, women are twice as likely to be affected by MS as men. Unilateral optic neuritis is the most common initial symptom of MS. The signs and symptoms of MS vary greatly from patient to patient and depend on the location and severity of the nerve fiber damage in the central nervous system. Most people with MS have a relapsing-remitting disease course or clinically isolated syndrome. They experience periods of new symptoms or relapses that develop over days or weeks and usually resolve partially or completely. These relapses are followed by quiet periods of disease remission that may last months or even years. Data accumulated over the years suggest a complex interplay between environment and immunogenetics (strong associations with a large number of immune and genetic markers), and an increasingly convincing role of an underlying degenerative process leading to demyelination (in both white and gray matter), axonal and neurosynaptic damage, and a persistent innate inflammatory response, with T-cell-mediated autoimmunity appearing to play a diminishing role as the MS develops and progresses. In the absence of clinically proven, accurate, and reliable biomarkers, the disease can take a progressive course in case of late treatment, signifying the critical need for early diagnosis. This article therefore discusses the etiopathogenesis and clinical aspects of MS.

Acute-Onset Retinal Conditions Mimicking Acute Optic Neuritis: Overview and Differential Diagnosis.

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.

Anti-MOG antibody optic neuritis in AML – A case report

We report a unique presentation of anti-myelin oligodendrocyte (MOG) optic neuritis in a patient with AML in remission. An elderly man presented with diminished vision in the right eye associated with headache and jaw pain. Systemic investigations revealed no leukemic recurrence. The presence of anti-MOG antibodies and optic nerve enhancement suggested of unilateral optic neuritis. Steroid therapy resulted in the resolution of disc edema. We faced a diagnostic dilemma in a patient with unilateral disc edema in the background of multiple systemic diseases. A methodical approach to investigations helped us in accurate diagnosis, institution of prompt therapy, and minimizing visual morbidity. We present this case to highlight the importance of differentiating leukemic optic nerve infiltration from other causes of unilateral disc edema, as their therapeutic approaches are different.

Isolated optic neuritis with positive glial fibrillary acidic protein antibody

Background and objectivesAutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) has been reported as a spectrum of autoimmune, inflammatory central nervous system disorders. Linear perivascular radial gadolinium enhancement patterns on brain magnetic resonance imaging (MRI) are a hallmark of these disorders. GFAP-A is associated with cerebrospinal fluid (CSF) GFAP antibody (GFAP-Ab), while the association with serum GFAP-Ab is less clear. This study aimed to observe the clinical characteristic and MRI changes of GFAP-Ab-positive optic neuritis (ON).MethodsWe performed a retrospective, observational case study at the department of neurology, Beijing Tongren Hospital, from December 2020 to December 2021. The serum of 43 patients and CSF samples of 38 patients with ON were tested for GFAP-Ab by cell-based indirect immune-fluorescence test.ResultsFour patients (9.3%) were detected GFAP-Ab positive, and in three out of the four patients, GFAP-Abs were detected only in serum. All of them demonstrated unilateral optic neuritis. Three patients (1, 2, and 4) experienced severe visual loss (best corrected visual acuity ≤ 0.1). Two patients (2 and 4) had experienced more than one episode of ON at the time of sampling. MRI showed optic nerve hyperintensity on T2 FLAIR images in all GFAP-Ab positive patients, and orbital section involvement was the most common. During follow-up (mean 4.5 ± 1 months), only Patient 1 had a recurrent ON, and no patient developed new other neurological events or systemic symptoms.ConclusionGFAP-Ab is rare in patients with ON and may manifest as isolated, relapsing ON. This supports the notion that the GFAP-A spectrum should comprise isolated ON.

Approach to Balancing Risk in Multiple Sclerosis Management: a Worldwide Practice Survey (P14-3.013)

Approach to Balancing Risk in Multiple Sclerosis Management: a Worldwide Practice Survey (P14-3.013)

Longitudinal Optical Coherence Tomography Measurement of Retinal Ganglion Cell and Nerve Fiber Layer to Assess Benign Course in Multiple Sclerosis.

A benign form of multiple sclerosis (BMS) is not easily diagnosed, but changes of the retinal ganglion cell layer-inner plexiform layer (GCL-IPL) and retinal nerve fiber layer (RNFL) may be sensitive to the disease. The aim of this study was to use optical coherence tomography (OCT) to investigate longitudinal changes of GCL-IPL and RNFL in BMS. Eighteen patients with BMS and 22 healthy control (HC) subjects were included, with a mean follow-up period of 32.1 months in BMS and 34.3 months in HC. Mean disease duration in BMS was 23.3 years, with 14 patients left untreated. Unilateral optic neuritis (ON) was found in eight patients. Non-ON eyes showed thinner GCL-IPL layer in the BMS group relative to HC (p < 0.001). The thinning rate of GCL-IPL in non-ON BMS, however, was -0.19 ± 0.15 µm/year vs. 0 ± 0.11 µm/year for HC (p = 0.573, age-adjusted). Thinning rate of RNFL in non-ON BMS was -0.2 ± 0.27 µm/year vs. -0.05 ± 0.3 µm/year for HC (p = 0.454, age adjusted). Conclusions: Thinning rate of the GCL-IPL and RNFL in BMS is similar to the healthy population but differs from the thinning rate in relapsing-remitting MS, presenting a non-invasive OCT-based criterion for assessing a benign course in multiple sclerosis.

Hyperacute optic neuritis in a patient with COVID-19 infection and vaccination: a case report

BackgroundAs scientific knowledge continues to grow regarding coronavirus disease 2019 (COVID-19) infection, several neuro-ophthalmological manifestations have emerged, including rare reports of optic neuritis. Optic neuritis is an inflammatory demyelinating condition of the optic nerve that typically presents as subacute, unilateral vision loss and pain on eye movement. Several cases of COVID-19 infection and COVID-19 vaccination related cases of optic neuritis have been reported. We present a case of hyperacute, unilateral optic neuritis after both recent COVID-19 infection and subsequent booster vaccination.Case presentationWithin two hours after receiving her COVID-19 booster vaccination, a 58-year-old female began experiencing bilateral eye pain, worsened by eye movements. The patient had previously contracted a mild COVID-19 infection three weeks prior to receiving her booster vaccination, confirmed by a rapid antigen test. The pain persisted in her right eye for a week at which time she presented to an ophthalmology clinic. She denied any changes to her visual acuity. Neuroimaging revealed right optic nerve enhancement, and the patient was admitted to the hospital for a course of intravenous steroids, which quickly resolved her eye pain.ConclusionTo our knowledge, this is the first reported case of COVID-19 related optic neuritis following both COVID-19 infection and vaccination. High clinical suspicion is needed to make the appropriate diagnosis, as cases of COVID-19 related optic neuritis may exhibit mild presentations, as was the case with our patient.

A Rare Case of Evans Syndrome Associated With Optic Neuritis: A Case Report

Purpose: To report a case of Evans Syndrome associated with severe optic neuritis. Case Report: HAF, male, 26 years old, diagnosed with Evans Syndrome at the age of nine, with a history of headache and right eye pain with worsening movement. Fifteen days after the initial condition, visual clouding and decreased visual acuity began. Ophthalmological examination revealed visual acuity of 20/200 in the right eye with progressive worsening as the condition progressed; and 20/20 in the left eye. Visual field with absolute scotomata in the right eye and relative and absolute scotomatous points in all quadrants of the left eye. Gluorescein angiography findings included optic disc attenuation, without hyperfluorescent lesions. Conclusion: Evans Syndrome is a pathology that is gradually expanding in the field of clinical research, which has allowed new concepts related to pathogenesis and association with other comorbid conditions. In this case, we report the association with unilateral Optic Neuritis, responsible for progressive visual loss. Such knowledge aggregates the scientific community and translates into the need for an early multidisciplinary approach.

Atypical myelin oligodendrocyte glycoprotein antibody–associated optic neuritis and acute demyelinating polyneuropathy after SARS-CoV-2 infection: Case report and literature review

Post-infectious immune-mediated neurological complications of Sars-Cov-2 have been increasingly recognized since the novel pandemic emerged. We describe the case of a 74 years-old patient who developed a Myelin Oligodendrocyte Glycoprotein (MOG) antibody-associated unilateral retrobulbar optic neuritis a few weeks after paucisymptomatic COVID-19 disease and, subsequently, after the resolution of the optic neuritis, an acute inflammatory demyelinating polyneuropathy. So far, no cases of these two neurological manifestations have been reported in the same patient. We herein report a case characterized by both manifestations and review the accumulating literature regarding MOG antibody-associated disease following SarsCov-2 infection.

Neurobrucellosis Manifesting as an Altitudinal Visual Field Defect.

Brucellosis is a zoonotic disease with a wide range of ophthalmic manifestations, including optic neuritis. It is most commonly caused by the consumption of raw or unpasteurised dairy products. We report a case of a 43-year male patient with an altitudinal visual field defect and unilateral optic neuritis caused by Brucella. While his visual acuity improved within 6 months of receiving anti-Brucella therapy and corticosteroid therapy, the altitudinal visual field defect persisted. Brucellosis can affect the central nervous system and eyes, in addition to having systemic manifestations. The ocular involvement of Brucella remains poorly understood. Early diagnosis and appropriate treatment can prevent serious complications in endemic areas. Key Words: Optic neuritis, Neurobrucellosis, Ocular brucellosis.

Application of the International Interocular Difference Thresholds into Practice: Localising the Patient Experience

ABSTRACT Demyelinating diseases of the central nervous system (CNS) often have neuro-ophthalmological manifestations, and retinal examination can be helpful in making the diagnosis. The latest iteration of optical coherence tomography (OCT)-based criteria for optic neuritis in multiple sclerosis has been developed in the research realm, but its application to clinical practice, and to the more uncommon demyelinating diseases requires further study. The ability to use OCT data to distinguish between various CNS demyelinating disorders could provide additional paraclinical tools to accurately diagnose patients. Furthermore, neuro-ophthalmological testing can define the extent of inflammatory damage in the CNS, independent of patient-reported history. New referrals for OCT at a tertiary multiple sclerosis and neuro-immunology referral centre (n = 167) were analysed retrospectively for the self-reporting of optic neuritis, serological test results, and diagnosis. Only approximately 30% of patients with a clinical history of unilateral optic neuritis solely had a unilateral optic neuropathy, nearly 40% of those subjects actually having evidence of bilateral optic neuropathies. Roughly 30% of patients reporting a history of bilateral optic neuritis did not have any evidence of structural disease, with 20% of these patients having a separate, intervenable diagnosis noted on macular scans. OCT is a useful adjunct diagnostic tool in the evaluation of demyelinating disease and has the ability to aid in a more accurate diagnosis for patients. Application of the international interocular difference thresholds to a clinical patient population generally reproduces the original results, emphasising their appropriateness. The analysis distinguishing the demyelinating diseases needs to be replicated in a blinded, multi-centre setting.

Unilateral optic neuritis with contralateral central retinal artery and central retinal vein occlusion in a post-COVID-19 case of rhino-orbital-cerebral mucormycosis: An unusual presentation

Introduction: The corona virus disease (COVID)-19 is a severe acute respiratory syndrome (SARS-CoV-2) which is posing a great threat to mankind and has been associated with a high risk of opportunistic fungi infection presenting as rhino-orbital-cerebral-mucormycosis. We report a rare and never reported case of unilateral optic neuritis with contralateral central retinal artery (CRA) and central retinal vein (CRV) occlusion in a patient of post-COVID-19 rhino-orbital-cerebral-mucormycosis. Case Report: A 45-year-old diabetic, Indian gentleman reported to our clinic in Dehradun, Uttarakhand, India with complaints of bilateral diminution of vision in right eye (RE) five days and left eye (LE) five weeks duration. He provided recent history of COVID-19 infection for which he was hospitalized and treated. Fundus examination confirmed optic neuritis in right eye and a combined established central retinal artery and vein occlusion in left eye. Fundus fluorescein angiography, visual evoked potential, and magnetic resonance imaging (MRI) were crucial in clinching the diagnosis. Conclusion: Rhino-orbital-cerebral-mucormycosis invades tissue through multiple routes. However, it is extremely rare to see a combination of angioinvasion leading to visual loss in one eye and perineural spread leading to optic neuritis and visual loss in the contralateral eye. Clinicians must be aware of such rare presentations which could serve as benchmark in diagnosis and treatment.