Optic Neuritis Research Articles

Ophthalmologic Implications of Connective Tissue Diseases: A Comprehensive Review of Current Knowledge and Innovations.

Connective tissue diseases (CTDs), including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjögren's syndrome, and systemic sclerosis (SSc), represent a diverse group of disorders characterized by abnormalities in the proteins that support tissues and organs. These diseases can affect multiple organ systems and are often associated with significant morbidity and mortality. The eyes are frequently affected among the various organ systems involved, with ocular manifestations ranging from benign conditions such as dry eye syndrome to severe, sight-threatening complications like scleritis, retinal vasculitis, and optic neuritis. Recognizing and managing these ophthalmologic implications is crucial for preventing severe complications, providing diagnostic clues, and improving patients' quality of life. This comprehensive review aims to elucidate the current knowledge and innovations related to the ophthalmologic implications of CTDs. It details the ocular manifestations associated with major CTDs, explores diagnostic approaches to identifying and differentiating these conditions, and discusses management strategies, including pharmacological and surgical interventions. Additionally, the review highlights recent advancements and emerging therapies in diagnosing and treating CTD-related ophthalmologic conditions. The review also addresses this field's challenges and future directions, emphasizing the importance of interdisciplinary collaboration and continuous research. By synthesizing the latest research and clinical insights, this review seeks to enhance the understanding of healthcare professionals regarding the interplay between CTDs and ocular health, ultimately contributing to improved patient care and outcomes.

Treatment Outcomes and Associated Influencing Factors Among Patients with Rifampicin-Resistant Tuberculosis: A Multicenter, Retrospective, Cohort Study in China.

Rifampin-resistant tuberculosis (RR-TB) remains a serious global public health concern. We assessed treatment outcomes and associated influencing factors among RR-TB patients in China. This research enrolled 1339 patients who started RR-TB treatment between May 2018 and April 2020 in China retrospectively. Data were collected from the electronic medical records. Multivariable logistic regression analysis was used to identify the influencing factors related to unfavorable outcomes. Of the 1339 RR-TB patients, 78.8% (1055/1339) achieved treatment success (cured or treatment completed), 5.1% (68/1339) experienced treatment failure, 1.1% (15/1339) died during treatment, 10.1% (135/1339) were lost to follow-up, and 4.9% (66/1339) were not evaluated. About 67.7% (907/1339) of patients experienced at least one adverse event (AE). The most common AE was hypohepatia (507/1339, 37.9%), followed by hyperuricemia (429/1339, 32.0%), anemia (368/1339, 27.5%), electrolyte disturbance (318/1339, 23.7%), peripheral neuritis (245/1339, 18.3%), and gastrointestinal reactions (203/1339, 15.2%). Multivariate analysis showed that age ≥60 years [adjusted oddsratio (aOR): 1.96, 95% confidence interval (CI): 1.39-2.77], national minority (aOR: 2.36, 95% CI: 1.42-3.93), smoking (aOR: 1.50, 95% CI: 1.10-2.04), cardiopathy (aOR: 2.90, 95% CI: 1.33-6.31), tumors (aOR: 9.84, 95% CI: 2.27-42.67), immunocompromise (aOR: 2.17, 95% CI: 1.21-3.91), re-treated TB (aOR: 1.46, 95% CI: 1.08-1.97), and experienced gastrointestinal reactions (aOR: 2.27, 95% CI: 1.52-3.40) were associated with unfavorable outcomes. Body mass index (BMI) ≥18.5 kg/m2, regimens containing bedaquiline and experienced adverse events (AEs) such as hypohepatia, leukopenia, peripheral neuritis, and optic neuritis were associated with favorable outcomes. High rates of treatment success were achieved for RR-TB patients at tertiary tuberculosis hospitals in China. Age ≥60 years, national minority, smoking status, comorbidities, re-treated TB, and experienced gastrointestinal reactions were independent prognostic factors for unfavorable treatment outcomes.

Prognostic factors associated with disability in a cohort of neuromyelitis optica spectrum disorder and MOG-associated disease from a nationwide Portuguese registry

IntroductionNeuromyelitis optica spectrum disorders (NMOSD) and MOG-associated disease (MOGAD) are an increasingly recognized group of demyelinating disorders of the central nervous system. Previous studies suggest that prognosis is predicted by older age at onset, number of relapses, the severity of the first attack and autoantibody status. ObjectiveTo study prognostic factors associated with disability progression and additional relapses in the 3-year follow-up of a national NMOSD/MOGAD cohort. ResultsOut of 180 of the initial Portuguese cohort, data on 82 patients was available at the end of the follow-up period (2019–2022). Two patients died. Twenty (24.4%) patients had one or more attack in this period (25 attacks in total), mostly transverse myelitis (TM) (56.0%) or optic neuritis (32.0%). MOGAD was significantly associated with a monophasic disease course (p = 0.03), with milder attacks (p = 0.01), while AQP4 + NMOSD was associated with relapses (p = 0.03). The most common treatment modalities were azathioprine (38.8%) and rituximab (18.8%). AQP4 + NMOSD more frequently required chronic immunosuppressive treatment, particularly rituximab (p = 0.01). Eighteen (22.5%) had an EDSS ≥6 at the end of the follow-up. AQP4 + NMOSD (p < 0.01) and the occurrence of transverse myelitis (TM) during disease (p = 0.04) correlated with an EDSS≥6 at the end of the follow-up period. MOGAD was significantly associated with an EDSS<6 (p < 0.01), and MOG+ cases that reached an EDSS>6 were significantly older (64.0 ± 2.8 versus 31.0 ± 17.1, p = 0.017). A bivariate logistic regression model including the serostatus and TM attacks during disease history successfully predicted 72.2% of patients that progressed to an EDSS≥6. ConclusionThis study highlights that myelitis predict increased disability (EDSS≥6) in NMOSD/MOGAG and AQP4 positivity is associated with increased disability.

A single-center retrospective series of OCT and MRI findings in pediatric MOGAD optic neuritis patients

BackgroundWhether optical computed tomography (OCT) and magnetic resonance imaging (MRI) findings are associated with final visual acuity in children with myelin oligodendrocyte glycoprotein antibody disease (MOGAD) optic neuritis is unclear. MethodsWe retrospectively reviewed the charts of pediatric patients with MOGAD optic neuritis seen at St. Louis Children's Hospital/Barnes Jewish Hospital since 2016. ResultsIn the 12 patients in this study, presenting visual acuity was worse in the optic neuritis-affected eyes but significantly improved from presentation to follow-up, such that, at last follow-up, there was no longer a statistical difference between the affected and unaffected eyes. The number of affected eyes with nerve enhancement and the amount of optic nerve affected, as well as thickness of the retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), and macula, decreased from presentation to follow-up. Ultimately, none of these variables were associated with final visual acuity. ConclusionIn this cohort, pediatric MOGAD optic neuritis patients had positive visual outcomes despite significant RNFL thinning and involvement of the optic nerve on MRI, leading to a lack of correlation between follow-up visual acuity and OCT and MRI measures of disease severity, respectively.

Visual recovery after oral high-dose methylprednisolone in acute inflammatory optic neuropathy.

High doses of venous corticosteroids are currently the only validated treatment for the management of optic neuritis (ON). The objective is to assess the changes in visual function parameters after oral high-dose methylprednisolone in patients with ON. A retrospective analysis of patients with acute ON was performed. Patients received 1 g per day of oral methylprednisolone for 3 to 5 days. Visual function was measured using the ETDRS test for visual acuity, 30-2 automated visual field test, contrast sensitivity test, and color vision test before treatment, 4 days, 2 weeks, 1 month and 3 months, and 6 months following treatment. To assess anatomical changes, optical coherence tomography of the ganglion cells was performed at various timepoints. Between September 2014 and September 2016, a total of 29 patients were included in the study. More than 80% of patients had recovered normal visual acuity after 3 and 6months. This recovery of all parameters of visual function was observed as early as 4days but occurred predominantly within 15days after the initiation of treatment. We observed a thinning of the ganglion cell layer during the follow-up, which mainly occurs within one month. The P100 wave of visually evoked potentials was discernible in all patients at 6months. During the 6years of follow-up, 2 patients had experienced a relapse of ON. No serious adverse effects were observed. This study demonstrated a rapid recovery of all visual function parameters after oral high-dose methylprednisolone ON with no serious adverse effects.

Cerebrospinal fluid neurofilament light chain in acute optic neuritis and its predictive ability of multiple sclerosis

BackgroundStudies on the capability of cerebrospinal fluid neurofilament light chain (cNfL) to predict multiple sclerosis (MS) conversion in clinically isolated syndromes have yielded varying results.ObjectivesTo expand our understanding of cNfL in optic neuritis (ON) and investigate whether incorporating cNfL into the 2017 McDonald criteria could accelerate the diagnosis of MS in patients with ON.MethodscNfL was measured in diagnostic samples from 74 patients with verified ON. MS was diagnosed using the 2017 McDonald criteria with a minimum observation time of two years from ON onset.Results20.5% of 44 MS-converters did not fulfil the 2017 McDonald criteria at ON onset. A doubling of cNfL was associated with 207% (74%–514%) higher odds of MS (p = 0.00042, adjusted for age). Fulfilment of ≥ 1 MRI criterion for dissemination in space (DIS) and presence of brain contrast-enhancing lesions were associated with higher cNfL. Furthermore, cNfL correlated with inter-eye differences in retinal nerve fiber layer (RNFL) thickness (Spearman’s ρ = 0.46, p = 8 × 10–5). Incorporating cNfL ≥ 906 pg/mL as a substitute for either dissemination in time or one MRI criterion for DIS increased the sensitivity (90.9% vs. 79.6%) and accuracy (91.9% vs. 87.8%), but also reduced the specificity (93.3% vs. 100%) of the 2017 McDonald criteria.ConclusioncNfL was related to MS diagnostic parameters and the degree of RNFL swelling. Clinical use of cNfL may aid in identification of ON patients with increased risk of MS until larger studies have elaborated on the potential loss of specificity if used diagnostically.

Novel model of multiple sclerosis induced by EBV-like virus generates a unique B cell population

Epstein-Barr virus (EBV) is deemed a necessary, yet insufficient factor in the development of multiple sclerosis (MS). In this study, myelin basic protein-specific transgenic T cell receptor mice were infected with murid gammaherpesvirus 68 virus (MHV68), an EBV-like virus that infects mice, resulting in the onset neurological deficits at a significantly higher frequency than influenza or mock-infected mice. MHV68 infected mice exhibited signs including optic neuritis and ataxia which are frequently observed in MS patients but not in experimental autoimmune encephalomyelitis mice. MHV68-infected mice exhibited increased focal immune cell infiltration in the central nervous system. Single cell RNA sequencing identified the emergence of a population of B cells that express genes associated with antigen presentation and costimulation, indicating that gammaherpesvirus infection drives a distinct, pro-inflammatory transcriptional program in B cells that may promote autoreactive T cell responses in MS.

A rare presentation of saccular aneurysm at junction of middle carotid artery and internal carotid artery mimicking the normal tension glaucoma

Acquired (pathologic) excavation of the optic cup is not always due to glaucoma. Ophthalmological signs of pathological disc cupping have been recognised in patients with normal intraocular pressures and with optic atrophy of various causes including ischaemic and compressive optic neuropathy, optic neuritis and trauma. Intracranial lesions have been reported to mimic the clinical presentation of glaucomatous optic disc changes and have also resulted in misdiagnosis. Optic atrophy caused by intracranial lesions shows pallor more than cupping. The macular nasal–temporal ratio compares the nasal and temporal thickness of the macular ganglion cell and inner plexiform layer and can be used to differentiate between compressive and non-compressive lesions as depicted in this case. This case report also suggests the importance of different imaging modalities in diagnosing compressive lesions and differentiating them from similar conditions.

Novel and characteristic radiological features of neurosyphilis: a case series

BackgroundTreponema pallidum can invade the central nervous system (CNS) early in its infection, causing neurosyphilis. Neurosyphilis typically presents with meningovasculitis in the acute or subacute phase, while tabes dorsalis and dementia paralytica are classical conditions in the later stages. However, syphilis is often misdiagnosed as other conditions such as tumors or autoimmune diseases including vasculitis and encephalitis, which is why the condition is known as “The Great Mimicker.” The increasing incidence of syphilis in recent years emphasizes the importance of early diagnosis and treatment; however, its multiple clinical manifestations impose diagnostic challenges for clinicians because it resembles other diseases. In this case series, we present the impressive manifestations of neurosyphilis through three unique radiological presentations.Case presentationCase 1 details optic nerve involvement in an HIV-positive male, where MRI and fundoscopic findings confirmed syphilitic optic neuritis. Case 2 describes a patient in her pregnancy initially suspected of acoustic neuroma on MRI, later diagnosed with syphilitic gumma affecting the inner ear canal. Case 3 is a young male with clinical features mimicking temporal arteritis, ultimately identified as skull osteomyelitis secondarily causing inflammation of the musculus temporalis and meningitis.ConclusionsThese cases underscore the necessity of considering syphilis in differential diagnoses, given the diversity of its clinical presentations. Radiology plays an important role in avoiding unnecessary interventions. The increasing prevalence of recurrent syphilis imposes diagnostic challenges, emphasizing the importance of the early diagnosis and treatment of neurosyphilis by clinicians.

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.

Analysis of magnetic resonance imaging in paranasal mucocele with visual disturbance.

Introduction Ocular imaging strategies have evolved to facilitate the diagnosis of optic neuropathy. This study aimed to evaluate the pathogenesis of visual disturbance associated with paranasal mucocele via magnetic resonance imaging (MRI). Methods A total of 19 patients with mucocele and visual disturbance who underwent endoscopic sinus surgery, orbital MRI, and sinus computed tomography were included. The age, sex, days from onset to surgery, eye pain, and imaging findings were analyzed. The results were compared between two groups: 7 patients with preoperative visual acuity worse than 20/200 (the poor group) and 12 patients with equal or better than 20/200 (the fair group). Results Imaging showed a high compression rate of the orbit in 17 (89.5%) and enlargement of the subarachnoid space around the optic nerve in 15 (78.9%) of 19 patients. Preoperative vision was significantly poor in cases with hyperintense regions in the optic nerve on T2-weighted imaging, indicating the presence of optic neuritis. No cases showed severe inflammation of the cyst or the presence of intraorbital fat tissue. Conclusion MRI-based diagnosis proved useful in evaluating pathological factors, such as orbital compression, ischemia, and optic neuritis, in individual cases. It can help gain insight into the pathogenesis of and developing appropriate treatment strategies for visual disturbances associated with paranasal mucocele.

To study the optic nerve characteristics in patients with optic neuritis

To study the optic nerve characteristics in patients with optic neuritis

Prognostic factors of first-onset optic neuritis based on diagnostic criteria and antibody status: a multicentre analysis of 427 eyes

BackgroundOptic neuritis (ON) prognosis is influenced by various factors including attack severity, underlying aetiologies, treatments and consequences of previous episodes. This study, conducted on a large cohort of first ON...

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder (MOGAD) Presenting as Optic Neuritis: A Case Series

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disorder (MOGAD) Presenting as Optic Neuritis: A Case Series

Are adverse events higher among patients with acute optic neuritis prescribed glucocorticoids? A retrospective, longitudinal cohort study

ObjectiveOptic neuritis (ON) is an acute focal inflammation of the optic nerve routinely treated with glucocorticoids. We aimed to compare adverse events (AE) among glucocorticoid-treated and untreated patients in the...

Azathioprine-Induced Anagen Effluvium: A Case Series of Three Cases

Azathioprine is a commonly used drug in dermatology for immunobullous diseases, vitiligo, and lichen planus alopecia areata. Azathioprine is rarely known to cause the acute onset of anagen effluvium (AE) that may precede myelosuppression associated with it. We report a series of three cases of azathioprine-induced AE. All three of our cases were females who were treated with azathioprine for non-dermatological conditions such as neuromyelitis optica, idiopathic thrombocytopenia, and optic neuritis. We report this series mainly due to its rarity.

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

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

Portable multi-focal visual evoked potential diagnostics for multiple sclerosis/optic neuritis patients

PurposeMultiple sclerosis (MS) is a neuro-inflammatory disease affecting the central nervous system (CNS), where the immune system targets and damages the protective myelin sheath surrounding nerve fibers, inhibiting axonal signal transmission. Demyelinating optic neuritis (ON), a common MS symptom, involves optic nerve damage. We’ve developed NeuroVEP, a portable, wireless diagnostic system that delivers visual stimuli through a smartphone in a headset and measures evoked potentials at the visual cortex from the scalp using custom electroencephalography electrodes.MethodsSubject vision is evaluated using a short 2.5-min full-field visual evoked potentials (ffVEP) test, followed by a 12.5-min multifocal VEP (mfVEP) test. The ffVEP evaluates the integrity of the visual pathway by analyzing the P100 component from each eye, while the mfVEP evaluates 36 individual regions of the visual field for abnormalities. Extensive signal processing, feature extraction methods, and machine learning algorithms were explored for analyzing the mfVEPs. Key metrics from patients’ ffVEP results were statistically evaluated against data collected from a group of subjects with normal vision. Custom visual stimuli with simulated defects were used to validate the mfVEP results which yielded 91% accuracy of classification.Results20 subjects, 10 controls and 10 with MS and/or ON were tested with the NeuroVEP device and a standard-of-care (SOC) VEP testing device which delivers only ffVEP stimuli. In 91% of the cases, the ffVEP results agreed between NeuroVEP and SOC device. Where available, the NeuroVEP mfVEP results were in good agreement with Humphrey Automated Perimetry visual field analysis. The lesion locations deduced from the mfVEP data were consistent with Magnetic Resonance Imaging and Optical Coherence Tomography findings.ConclusionThis pilot study indicates that NeuroVEP has the potential to be a reliable, portable, and objective diagnostic device for electrophysiology and visual field analysis for neuro-visual disorders.

Diagnostic criteria for optic neuritis in the acute and subacute phase: clinical uses and limitations.

A recent international consensus panel proposed diagnostic criteria for optic neuritis and a new classification. We aimed to investigate the clinical relevance of these diagnostic criteria and classification, in a cohort of patients hospitalized for a suspected diagnosis of optic neuritis. We included all patients hospitalized between 2017 and 2022 in our tertiary center for (sub)acute loss of visual acuity suggestive of optic neuritis. Clinical and paraclinical criteria obtained within the first 3months of symptoms were collected, as well as the final diagnosis which could be optic neuritis or non-optic neuritis. We constructed a contingency table comparing diagnoses based on physician experience to those based on the recently proposed criteria. The subtypes of optic neuritis based on the new classification were compared to subtypes based on the clinician experience. Two hundred fifty-seven patients were included in this study. Prevalence of optic neuritis in our cohort was 88.3%. Sensitivity and specificity of a correct diagnosis using the new criteria were, respectively, 99.5% and 86.7%. The proposed diagnostic criteria overdiagnosed four patients with optic neuritis and missed the diagnosis in one patient. According to the recent classification, idiopathic optic neuritis and clinical isolated syndrome were reclassified mainly as single isolated optic neuritis. In our specific cohort of patients hospitalized for acute and subacute optic neuropathy highly suspect of optic neuritis, we found that recently proposed diagnostic criteria and classification of optic neuritis are relevant for our clinical practice. Our interpretation of clinical requirement for definite and possible optic neuritis diagnosis might explain our excellent sensitivity and our high percentage of definite optic neuritis, relative to previous publications. The moderate specificity (86.7%) underlines the importance to include all contextual data in consideration for the diagnosis. The simplification of subgroups is useful, but our study highlights the complexity to find the adequate subgroup for seronegative NMOSD.

Related factors of acute symptomatic seizures and epilepsy in children with myelin oligodendrocyte glycoprotein antibody-associated disease

Objective: To investigate the risk factors of acute symptomatic seizures (ASS) and epilepsy in children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: A ambispective cohort study was used including 74 children with MOGAD who were admitted to the Department of Pediatrics of Peking University First Hospital from January 2013 to June 2023 and were followed up. Demographic information, clinical information, treatment status, ASS and epilepsy status were collected. The clinical phenotypes were classified. According to the presence or absence of ASS in the course of disease, the children and the course of disease were divided into groups with and without ASS. Chi-square test, Fisher exact test and Mann Whitney U test were used to analyze the correlation between symptoms and auxiliary examination characteristics and the occurrence of ASS in the two groups of children. Multivariate Logistic regression analysis was used for multivariate analysis. Results: The onset age of the 74 children with MOGAD was 6.58 (3.80, 9.67) years, including 38 females (51.4%) and 36 males (48.6%). The duration of the final follow-up was 2.67 (1.10, 4.12) years, with a total of 239 times acute clinical episodes. ASS occurred in 39.2% (29/74) children during the course of disease and in 29.3% (70/239) of attacks. The common phenotypes were ADEM (67 times (28.0%)), optic neuritis (37 times (15.4%)) and cerebral cortical encephalitis (31 times (13.0%)) in 239 times acute clinical episodes. The incidence of ASS in ADEM and cerebral cortical encephalitis phenotype was 28.4%(19/67) and 100.0% (31/31), respectively. Multivariate analysis showed that cortical involvement on magnetic resonance imaging during clinical attacks was an independent risk factor for ASS (β=-1.49, OR=0.23) after excluding attacks involving only optic nerve or spinal cord (49 episodes). During the follow-up, 5 children (6.8%) had epilepsy, and all children with epilepsy had multiple clinical attacks of MOGAD and previous ASS. Conclusions: Cortical involvement on magnetic resonance imaging during clinical episodes is an independent risk factor for ASS in children with MOGAD. All MOGAD children with epilepsy had ASS and multiple MOGAD clinical episodes in the past.