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Background: Among young people, multiple sclerosis (MS) accounts for the majority of non-traumatic neurological impairments. Almost all MS patients develop optic neuritis as the illness progresses. In MS, the optic nerve (ON) is vulnerable to demyelinating injury and is considered "eloquent" in comparison with the brain. Thus, it serves as a clinical model for MS. Since the eye is a suitable target for ultrasonography due to its high-water content, non-invasive trans-orbital sonography (TOS) can determine the optic nerve sheath diameter (ONSD). This research aimed to determine the effectiveness of ON ultrasonography as a simple, low-cost method for diagnosing and following up MS patients, facilitating their treatment plan. It correlates the findings of ONSD using TOS and visual-evoked potential (VEP), the number of magnetic resonance imaging (MRI) black holes, and other clinically significant parameters in 30 relapsing–remitting MS (RRMS) patients versus 30 controls. Results: We examined a total of 120 eyes. The ONSD values at 3 mm, 5 mm, and myelination index were significantly lower in the patients (4.7 ± 0.3, 5.5 ± 0.3, and 0.86 ± 0.03, respectively) versus the controls (5.6 ± 0.3, 6.2 ± 0.4, and 0.90 ± 0.03, respectively) (p < 0.001 in each). The mean P 100 latency was significantly delayed in the patients (125.5 ± 6.1 ms) than the controls (100.2 ± 3.3 ms), (p < 0.001). The mean VEP amplitude was significantly lower in the patients (4.9 ± 0.7 μV) than the controls (6.8 ± 0.8 μV) (p < 0.001). There was a highly significant positive correlation between the ONSD and P 100 amplitude, and highly significant negative correlations between the ONSD and disease duration, number of optic neuritis attacks, EDSS, MRI black holes number, and P 100 latency. Conclusions: Trans-orbital sonography is a practical and easily accessible approach for assessing ON atrophy in MS. It can measure axonal loss and brain atrophy indirectly and is a reliable paraclinical diagnostic tool, suggesting that ONSD could be a biomarker of disease activity. [ABSTRACT FROM AUTHOR]

Background: In cases of optic disc edema or a pale optic disc, distinguishing an episode of optic neuritis (ON) from that of non-arteritic anterior ischemic optic neuropathy (NAION) during a clinical examination is challenging. Optical coherence tomography angiography (OCTA) can reveal differences in peripapillary vascular network structures and provide biomarkers for differential diagnosis. Methods: A total of 23 eyes with NAION, 22 eyes with demyelinating ON (DON), and 27 eyes from healthy participants were imaged using OCTA to observe the radial peripapillary capillaries (RPCs). Optical coherence tomography was used to measure peripapillary retinal nerve fiber layer (RNFL) thickness and the macular ganglion cell complex (mGCC). Data for all patients were recorded at 2–3 weeks and more than 3 months after the symptom onset. Results: A total of 23 affected eyes from 23 patients with NAION (average age 52.17 ± 7.92 years), 22 eyes from 22 patients with demyelinating optic neuritis (DON) (average age 47.88 ± 19.24 years), and 27 eyes from 27 healthy individuals (average age 46.43 ± 14.08 years) were included in the study. There were no significant differences in sex, age, and eye laterality between any two groups (F = 0.968, 0.475, 0.870; p > 0.05). Throughout the course of NAION and DON, the superior RPC, superior mGCC, and peripapillary RNFL decreased with time (p < 0.05). In contrast, the inferotemporal RPC and inferior mGCC did not decrease from the acute to chronic stage in NAION (t = 1.639, 0.834, p = 0.117, 0.413). Compared with the normal group, patients with NAION and DON exhibited a sharp reduction in the average RPC, RNFL, and GCC from the acute to the chronic stage (p < 0.05). Patients with DON exhibited a significant decrease in the inferotemporal RPC and inferior mGCC compared with the patients with NAION (p < 0.05). In contrast, there were no significant differences in the inferior mGCC at the chronic stage between the patients with NAION and those with ON (t = 2.547, p = 0.093). Conclusion: Various structural and microvascular changes were observed in patients with NAION and ON, indicating distinct features of the optic nerve during the different stages of NAION and ON. Peripapillary vascular density, measured using spectral domain OCT (SD-OCT), may be a biomarker to distinguish NAION from ON. [ABSTRACT FROM AUTHOR]

BackgroundCase reportConclusionsDeficiency of adenosine deaminase 2 (DADA2) is a rare autosomal recessive autoinflammatory disorder associated with systemic vasculitis and bone marrow failure. Reported ophthalmic findings in DADA2 include optic neuritis, retinal artery occlusion, uveitis, and optic atrophy. We report the case of a child found to have bilateral cataracts.A three-year-old recent immigrant from Mexico with a diagnosis of DADA2 and transfusion-dependent anemia was referred to ophthalmology to screen for deferasirox-associated retinopathy in the setting of hemochromatosis. He was incidentally found to have bilateral posterior subcapsular cataracts with no other ophthalmic abnormalities. The child’s lab findings were significant for chronic hyperferritinemia, and his history was significant for over a year of oral prednisone use in Mexico.This is the first reported case of cataracts in a child with DADA2. While DADA2 is an autoinflammatory disorder, this child’s lack of uveitis suggests a non-inflammatory etiology. Hyperferritinemia is a known cause of cataracts and is common in DADA2, but the child’s history of oral steroid use in Mexico could also explain his cataracts. As pediatric cataracts have not otherwise been reported in DADA2, ophthalmologists should be aware of this possibility, especially in children with hyperferritinemia or a history of steroid use. [ABSTRACT FROM AUTHOR]

Background Study Aim Study Design Measures and Outcomes Results Conclusion Patients suffering from multidrug/rifampicin resistant tuberculosis (MDR/RR‐TB) are treated for prolonged periods with a complex regimen comprised of relatively less effective and more toxic anti‐TB drugs, consequently resulting in high incidence of adverse events (AEs).The current study evaluates the incidence, types, management and predictors of AEs, and their impact on treatment outcomes in MDR/RR‐TB patients receiving all oral treatment regimens.A total of 242 eligible MDR/RR‐TB patients treated at two different study sites from June 2019 to December 2021 were included in this study.Patients' sociodemographic, microbiological, clinical characteristics, reported AEs and treatment outcomes were retrospectively abstracted from their medical records. Chi‐square, and Fisher exact tests (wherever applicable) were used to find the association between the variable and the occurrence of AEs.Majority of the patients were suffered from MDR‐TB (71.9%) and were treated with longer treatment regimen (77.7%). Overall 206/242 patients (85%) experienced at least one AE during their treatment. Gastrointestinal disturbance was the most common AE (49.6%), followed by arthralgia (49.2%), psychiatric disturbances (39.3%), dermatological reactions (27.7%), body/headache (24.8%) and hyperuricemia (19%). Due to AEs, treatment modification was noted in 55 (22.72%) patients. Level of modification in the treatment regimen was higher in optic neuritis (100%) followed by neuropathies (80%) and myelosuppression (59%). Similarly, hepatotoxicity was the most serious AE in which the whole treatment regimen was terminated in 27% of patients. Furthermore, the results revealed that only patients' education status had statistically significant association with the incidence of AEs (p = 0.02). The treatment success rate was 80.6% whereas the ratio of died and LTFU patients were 15.3% and 4.1% respectively. Although patients who experienced AEs were more likely to develop successful treatment outcomes (82%) than their counterparts (72.2%), though this difference was not statistically significant.Although AEs were highly present in the current cohort, but they were successfully managed mostly by nonpharmacological interventions or symptomatic treatment. Besides, the incidence of AEs did not have a negative impact on treatment outcomes. High‐risk patients for AEs must receive special attention and enhanced clinical management. [ABSTRACT FROM AUTHOR]

Background: Optic neuritis (ON) is a common manifestation of multiple sclerosis (MS) and myelin-oligodendrocyte-glycoprotein IgG-associated disease (MOGAD). This study evaluated the applicability of optical coherence tomography (OCT) for differentiating between both diseases in two independent cohorts. Methods: One hundred sixty two patients from seven sites underwent standard OCT and high-contrast visual acuity (HCVA) testing at least 6 months after first ON. Of these, 100 patients (32 MOGAD, 68 MS) comprised the primary investigational cohort, while 62 patients (31 MOGAD, 31 MS) formed a validation cohort. A composite score distinguishing between MOGAD and MS was developed using multivariate logistic regression. Results: Bilateral simultaneous ON occurred more frequently in MOGAD compared to MS (46.9 vs. 11.8%, p < 0.001). OCT revealed more peripapillary retinal nerve fiber layer (pRNFL) atrophy in all segments in MOGAD compared to predominantly temporal pRNFL atrophy in MS (p < 0.001). HCVA was better preserved in MS (p = 0.007). pRNFL thickness in all except for temporal segments was suitable for differentiating MOGAD and MS. Simultaneous bilateral ON and critical atrophy in nasal (< 58.5 µm) and temporal superior (< 105.5 µm) segments were included into the composite score as three independent predictors for MOGAD. The composite score distinguished MOGAD from MS with 75% sensitivity and 90% specificity in the investigational cohort, and 68% sensitivity and 87% specificity in the validation cohort. Conclusion: Following a single ON-episode, MOGAD exhibits more pronounced global pRNFL atrophy and lower visual acuity after ON compared to MS. The introduced OCT-based composite score enabled differentiation between the two entities across both cohorts. [ABSTRACT FROM AUTHOR]

Optic neuritis can be an early sign of demyelinating diseases like multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein (MOG) antibody-associated diseases (MOGAD). We investigated the presence or absence of head and spinal cord lesions on magnetic resonance imaging (MRI) and assessed whether cerebrospinal fluid (CSF) tests are useful in detecting demyelinating disease in patients with first diagnosed optic neuritis. We conducted a retrospective study of 111 patients (47 idiopathic, 19 NMOSD, 16 MOGAD, 16 MS, 6 optic neuritis with cerebral lesions but that does not meet the McDonald’s criteria for MS (ON+)), and 7 chronic relapsing inflammatory optic neuropathy) diagnosed with optic neuritis without cerebral or spinal symptoms. Patients underwent evaluations including orbital, head, and spine MRI, along with CSF analysis. Among the 111 patients, 20 (35.1%: 4 NMOSD, 4 MOGAD, 7 MS, and 6 ON+) exhibited intracerebral or spinal cord lesions. Twelve patients showed findings on both orbital and head MRI, while six had no orbital MRI findings except for optic neuritis but exhibited lesions on head MRI. Five patients had spinal lesions without intracerebral lesions. CSF analysis revealed positive oligoclonal bands and elevated myelin basic protein levels indicate the high likelihood with systemic inflammatory demyelinating diseases. Even in the absence of concomitant encephalitis or myelitis symptoms or a history of these conditions, MRI images of patients with optic neuritis sometimes reveal lesions in the brain or spinal cord. CSF abnormalities were indicative of systemic demyelinating disease presence, extending beyond MS to NMOSD and MOGAD. [ABSTRACT FROM AUTHOR]

Purpose: To describe diverse ocular manifestations in a patient with Myelin oligodendrocyte glycoprotein-associated disease (MOGAD). Methods: A 15-year-old Indian male had severe loss of vision in one eye, followed by a recurrent attack of optic neuritis in the fellow eye a few weeks later. He had a history of vision loss, speech disturbances, altered sensorium and was a confirmed case of Myelin oligodendrocyte glycoprotein-associated disease (MOGAD). Apart from optic neuritis, other rare ophthalmic associations, namely, macular neuroretinopathy, retinal haemorrhages, severe optic nerve head edema, peri neuritis, and orbital enhancement on magnetic resonance imaging (MRI) were noted. Results: He responded dramatically to treatment with intravenous pulse steroids and relapses were controlled with long-term immunomodulation therapy. Conclusion: This case report reiterates the need for early treatment with pulse steroids in MOGAD and depicts the heterogeneous involvement of various ocular structures in the disease. [ABSTRACT FROM AUTHOR]

Myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease is a neuroinflammatory disorder (MOGAD) with heterogeneous phenotype including paroxysms of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, brainstem demyelination, and encephalitis. Fluid-attenuated inversion recovery hyperintense cortical lesions in MOG-associated encephalitis with seizures, or FLAMES, is a manifestation of cerebral cortical encephalitis seen less frequently than other typical MOG antibody–associated disease presentations. Cases of FLAMES are rarer in children, and frequently initially misdiagnosed with infectious meningoencephalitis. Other meningocortical manifestations of MOG antibody–associated disease have been described and likely exist along a continuum. In this retrospective single-center case series, we describe the demographic, clinical, radiographic, laboratory, and electroencephalographic features of 5 children with clinicoradiographic features consistent with the spectrum of MOG-IgG–positive meningocortical syndromes. [ABSTRACT FROM AUTHOR]

Kimura disease (KD) is a rare chronic inflammatory disease that typically presents with soft subcutaneous granulomas in the head and neck regions characterized by elevated blood eosinophils and immunoglobulin E (IgE) level, whose aetiology remains poorly elucidated. Minimal change disease (MCD) has been reported as one of the renal manifestations that KD can present with, indicating that they may share a common pathology. Herein we describe a case of recurrent MCD associated with KD. During a follow‐up period of 15 years, MCD recurred three times with increased disease activity of KD as reflected by flares of skin lesions and elevated peripheral eosinophils, and responded well to increased doses of prednisolone and cyclosporin. Notably, visual field defects in his right monocular vision appeared at the time of third recurrence of MCD, leading to the diagnosis of optic neuritis (ON). Optic nerve involvement associated with KD is extremely rare, and this case is noteworthy in that inflammation in the optic nerve was observed at the time of MCD recurrence with increased disease activity of KD, suggesting the existence of a common pathology between KD, MCD, and ON. In patients with KD, an imbalance of T helper (Th) cells with Th2 cells predominating over Th1 cells is observed, which results in hyperIgEemia and eosinophilia. This Th2‐predominant immunological status in KD considered to predispose to MCD may also predispose to ON. MCD with a background of Th2‐predominant immune state may require attention to the possibility of complication of ON. [ABSTRACT FROM AUTHOR]

Background: Observational studies have shown an increased risk of ocular inflammatory diseases in patients with ankylosing spondylitis (AS), but the genetically predicted association remains unclear. The aim of this study was to systematically assess the causal relationship between AS and ocular inflammatory diseases. Methods: We conducted a two-sample Mendelian randomization (MR) analysis to investigate the causal relationship between AS and several common ocular inflammatory diseases based on genome-wide association study (GWAS) data and public health data. Five methods, namely, inverse-variance weighted (IVW), MR-Egger, weighted median, weighted mode, and simple mode, were used. Sensitivity analysis was performed using MR-Egger intercept, Mendelian Randomization Pleiotropy RESidual Sum and Outlier (MR-PRESSO), Cochran’s Q test, outlier methods, leave-one-out analysis, and funnel plots. Results: The MR analysis showed a significantly increased risk of uveitis (ORIVW = 2.825, 95%CIIVW = 1.709-4.672, and PIVW < 0.001), iridocyclitis (ORIVW = 3.806, 95%CIIVW = 2.809-5.157, and PIVW < 0.001), scleritis (ORIVW = 1.738, 95%CIIVW = 1.190-2.539, and PIVW < 0.001), and episcleritis (ORIVW = 5.113, 95%CIIVW = 2.067-12.645, and PIVW = 0.004) associated with AS. However, no correlation was found between genetically predicted AS and keratitis (ORIVW = 1.041, 95% CIIVW = 0.886-1.222, and PIVW = 0.628) and optic neuritis (ORIVW = 0.868, 95% CIIVW = 0.441-1.709, and PIVW = 0.682). Conclusion: AS increases the genetically predicted risk for uveitis, iridocyclitis, scleritis, and episcleritis. No potential association of AS with keratitis and optic neuritis was found. It may provide clues for the prevention of AS complications. [ABSTRACT FROM AUTHOR]

Introduction: Ethambutol, a key anti-tuberculosis medication, is associated with optic neuropathy, which can lead to significant vision impairment. This population-based cohort study investigated the nationwide usage of ethambutol and the incidence and screening of optic neuropathy among ethambutol users. Methods: The cohort included 206,157 individuals from the Health Insurance Review and Assessment database in South Korea who initiated ethambutol therapy between 1 January 2015, and 31 December 2021. Among them, 117,309 individuals without prior ophthalmic diseases were analyzed to investigate practice patterns. The timing of the baseline examinations (an ophthalmic examination immediately preceding [within 1 month] ethambutol initiation or the first ophthalmic assessment conducted after the initiation) and subsequent monitoring and modalities used were assessed. Results: The cumulative incidences of the optic neuropathy categories, overall optic neuropathy, optic neuropathy/optic neuritis, and optic atrophy, were analyzed. The annual number of ethambutol users declined over time between 2015 and 2021. The cumulative incidences of overall optic neuropathy, optic neuropathy/optic neuritis, and optic atrophy were 2.8%, 2.0%, and 0.7%, respectively. Optical coherence tomography usage for ophthalmic examinations remarkably increased from 23.9% in 2015 to 85.5% in 2021, while visual field examinations were frequently (69.6%) employed for functional assessment at the time of diagnosis. Baseline screening within 6 months of initiating ethambutol therapy was performed in 12.0%-13.1% of ethambutol initiators annually over the study period. The percentage of ethambutol initiators receiving subsequent monitoring within 6 months following baseline screening declined over time, from 13.1% in 2015 to 7.9% in 2021. Discussion: The diminishing trend in monitoring emphasizes the need for improved pharmacovigilance to reduce the risk of optic atrophy and blindness through early detection. [ABSTRACT FROM AUTHOR]

PurposeMaterials and methodsResultsConclusionsTo estimate the number of ocular syphilis (OS) cases diagnosed in a tertiary care centre in Portugal, correlate with increasing syphilis diagnoses and characterize the OS population.Retrospective, observational, single-center study that included patients diagnosed with OS between 2015 and 2023 at the local health unit of Coimbra. Demographic data were collected, and a complete ophthalmological examination was performed with multimodal imaging acquisition. Data on syphilis reports from the National System of Epidemiologic Surveillance were correlated with OS data.Fifty-four patients with OS were observed; mean age was 54.17 ± 14.46 years, 38 (70.37%) were male and 18 (47.37%) men who have sex with men. The proportion of OS in syphilis patients per year ranged from 0% to 10.34%. One-quarter were co-infected with HIV. Forty-nine patients (91.84%) complained of decreased visual acuity on presentation. Twenty-two (40.74%) had systemic findings (mostly skin rash). Two-thirds had bilateral disease and half presented with anterior segment involvement. Eighty-five percent had posterior segment involvement, mostly placoid chorioretinitis. Forty-one percent had optic nerve involvement. All patients were admitted and underwent 2-week treatment with intravenous penicillin. Visual acuity improved from logMAR 0.70 to logMAR 0.26 (p < 0.001).Ocular syphilis is a heterogeneous disease with a wide range of presentations. The incidence is on the rise and therefore OS must be considered in every patient with anterior and posterior uveitis, panuveitis and optic neuritis, with or without systemic manifestations. [ABSTRACT FROM AUTHOR]

Background: Retinal artery occlusions are rare amongst young adults, and relevant risk factors and etiology remain unclear. In this report, we present a case of central retinal artery occlusion (CRAO) as the initial manifestation of mixed connective tissue disease (MCTD) in a young woman. Case presentation: A 22-year-old female presented to the emergency department with a sudden decrease in visual acuity in her right eye for 1 hour. She reported a similar episode in her left eye five years prior, which resolved spontaneously after 2 hours. Initially misdiagnosed with optic neuritis in the right eye at another hospital, she was referred to our institution the following day. Clinical examination revealed a CRAO in her right eye. A detailed medical history revealed that she had developed livedo reticularis (LR) on both lower limbs five years ago, which had been overlooked and untreated. Further rheumatologic history, hematologic tests, and an autoimmune work-up confirmed a diagnosis of mixed connective tissue disease (MCTD). Conclusion: In young patients presenting with CRAO, further examinations should be conducted to investigate systemic disease or an embolic source to prevent future sequelae. [ABSTRACT FROM AUTHOR]

Isolated ocular relapses of leukemia are rare and typically present as infiltrative lesions of the optic nerve. This report presents a case of an isolated ocular relapse of leukemia in a female patient, characterized by occult infiltrative optic neuropathy. Despite the absence of leukemic cell infiltration on MRI, a 33-year-old female patient presented with optic neuritis, which resolved after intrathecal administration of chemotherapeutic agents. However, due to her non-compliance with the treatment regimen, leukemia progressed to infiltrative optic neuropathy. She refused radiation therapy, and while intrathecal injections provided temporary stabilization, they were insufficient for sustained disease control. This case uniquely documents the extended progression of an isolated ocular relapse of leukemia, evolving from an occult infiltrative lesion initially presenting as optic neuritis to a more pronounced infiltrative lesion. It suggests that leukemic optic nerve infiltration can occur in patients with leukemia even in the absence of MRI-detectable abnormalities. Radiation therapy emerges as a critical modality in the management of infiltrative lesions of the optic nerve. [ABSTRACT FROM AUTHOR]

We report the case of a 24-year-old previously healthy man who presented with an unusual combination of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) and Leber’s hereditary optic neuropathy (LHON). Initially experiencing two episodes of painless visual acuity reduction, the patient responded well to glucocorticoid therapy, coinciding with an MOG antibody titre of 1:32. However, a subsequent third episode of painless vision loss did not improve with continued glucocorticoid treatment. Further diagnostic workup revealed a mitochondrial DNA mutation (11778 G>A), confirming the diagnosis of LHON. This unique presentation highlights the necessity of considering multiple aetiologies in cases of optic neuropathy where the cause is not immediately apparent, especially when the response to standard treatments is inconsistent. Our findings suggest that dual pathologies may coexist and should be considered in unexplained cases of optic neuropathy to facilitate appropriate clinical management. [ABSTRACT FROM AUTHOR]

Background/Objectives: This study investigated the frequency and timing of optic neuritis (ON) episodes in relation to the onset of multiple sclerosis (MS) and examined the occurrence of Uhthoff's phenomenon and Lhermitte's sign to understand their roles in early diagnosis and disease progression. Methods: A longitudinal study was conducted with 127 MS patients. Clinical data, including ophthalmological examinations for ON, were collected and questionnaires assessed the presence of Uhthoff's phenomenon and Lhermitte's sign. Results: Results showed that 37% of patients experienced demyelinating retrobulbar ON, with 25.53% having ON as the initial symptom of MS. Residual visual acuity impairment (below 20/40) and dyschromatopsia were reported by 25.53% and 17.02% of patients, respectively. Uhthoff's phenomenon and Lhermitte's sign were present in 26.77% and 36.22% of patients, respectively. The findings underscore the importance of early ophthalmological assessments in diagnosing MS, as ON can be an initial indicator of the disease. Conclusions: The study highlights the need for precise diagnostic tools and personalized therapeutic strategies focused on specific biomarkers and pathways involved in neuroinflammation and demyelination. Early diagnosis through vigilant ophthalmologic evaluation can lead to interventions that significantly alter disease progression, improving patient outcomes and quality of life. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica (NMO) spectrum disorder (NMOSD) is a relapsing inflammatory disease of the CNS, characterized by the presence of serum aquaporin 4 (AQP4) autoantibodies (AQP4-IgGs) and core clinical manifestations such as optic neuritis, myelitis, and brain or brainstem syndromes. Some people exhibit clinical characteristics of NMOSD but test negative for AQP4-IgG, and a subset of these individuals are now recognized to have serum autoantibodies against myelin oligodendrocyte glycoprotein (MOG) — a condition termed MOG antibody-associated disease (MOGAD). Therefore, the concept of NMOSD is changing, with a disease spectrum emerging that includes AQP4-IgG-seropositive NMOSD, MOGAD and double-seronegative NMOSD. MOGAD shares features with NMOSD, including optic neuritis and myelitis, but has distinct pathophysiology, clinical profiles, neuroimaging findings (including acute disseminated encephalomyelitis and/or cortical encephalitis) and biomarkers. AQP4-IgG-seronegative NMOSD seems to be a heterogeneous condition and requires further study. MOGAD can manifest as either a monophasic or a relapsing disease, whereas NMOSD is usually relapsing. This Review summarizes the history and current concepts of NMOSD and MOGAD, comparing epidemiology, clinical features, neuroimaging, pathology and immunology. In addition, we discuss new monoclonal antibody therapies for AQP4-IgG-seropositive NMOSD that target complement, B cells or IL-6 receptors, which might be applied to MOGAD in the near future. This Review summarizes the history and current concepts of neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), including epidemiology, clinical and neuroimaging features and pathophysiology. It also discusses new molecularly targeted therapies for NMOSD that might be also applied to MOGAD in the future. Key points: The concepts of neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are evolving, with increasing recognition of the similarities and differences between these two diseases. This Review provides a critical comparison of NMOSD and MOGAD, including their history, epidemiology, clinical characteristics, neuroimaging findings and immunological aspects. Antibody testing, biomarkers and neuroimaging have crucial roles in the differential diagnosis of NMOSD and MOGAD. The landscape for acute and disease-modifying therapies for NMOSD and MOGAD, including conventional and new molecularly targeted agents, is advancing and could inform the development of therapies for other antibody-mediated neurological diseases. [ABSTRACT FROM AUTHOR]

In 2020, WHO guidelines prioritised the use of a standard fully oral short treatment regimen (STR) consisting of bedaquiline, levofloxacin or moxifloxacin, ethionamide, ethambutol, high-dose isoniazid, pyrazinamide, and clofazimine for the management of rifampicin-resistant tuberculosis. A high prevalence of resistance to constituent drugs precluded its widespread use by countries in the WHO European region. We evaluated three 9-month fully oral modified STRs (mSTRs) in which ethionamide, ethambutol, isoniazid, and pyrazinamide were replaced by linezolid, cycloserine, or delamanid (or a combination). This multicountry, prospective, single-arm, cohort study examined the effectiveness and safety of mSTRs for fluoroquinolone-susceptible, rifampicin-resistant pulmonary tuberculosis in 13 countries in the WHO European region during 2020–23. We enrolled adults and children of all ages with bacteriologically confirmed rifampicin-resistant, fluoroquinolone-susceptible pulmonary tuberculosis, and children (aged 0–18 years) with clinically diagnosed disease and a confirmed contact with rifampicin-resistant, fluoroquinolone-susceptible tuberculosis. Participants aged 6 years or older received one of two regimens: bedaquiline, linezolid, levofloxacin, clofazimine, and cycloserine; or bedaquiline, linezolid, levofloxacin, clofazimine, and delamanid. Children younger than 6 years received delamanid, linezolid, levofloxacin, and clofazimine. Participants were followed up for 12 months after successful treatment completion to detect recurrence and death. The primary outcome was the cumulative probability of not having an unsuccessful study outcome (defined as treatment failure, on-treatment loss to follow-up, death, or recurrence) before 22 months of study follow-up. The primary safety outcome was the incidence of each adverse event of interest (peripheral neuropathy, optic neuritis, myelosuppression, hepatitis, prolonged QT interval, hypokalaemia, and acute kidney injury) of grade 3 or higher severity during the treatment course. Between Aug 28, 2020 and May 26, 2021, 7272 patients were screened and 2636 were included in the treatment cohort. 1966 (74·6%) were male, 670 (25·4%) were female, and median age was 43 years (IQR 33–53). Treatment success was recorded for 2181 (82·7%) participants. The cumulative probability of not having an unsuccessful study outcome 22 months after treatment initiation was 79% (95% CI 78–81). Increasing age (adjusted hazard ratio 2·61 [95% CI 1·70–4·04] for people aged >64 years vs 35–44 years), HIV-positive status (1·53 [1·16–2·01]), presence of bilateral cavities (1·68 [1·29–2·19]), smoking history (1·34 [1·05–1·71]), baseline anaemia (1·46 [1·15–1·86]), unemployment (1·37 [1·04–1·80]), elevated baseline liver enzymes (1·40 [1·13–1·73]), and excessive alcohol use (1·47 [1·14–1·89]) were positively associated with unsuccessful study outcomes. In the safety cohort of 2813 participants who received at least one dose, 301 adverse events of interest were recorded in 252 (9·0%) participants with the most frequent being myelosuppression (139 [4·9%] participants, 157 [52·2%] events). The high treatment success and good safety results indicate considerable potential for the use of mSTRs in programmatic conditions, especially for individuals not eligible for the current WHO-recommended 6-month regimen and in settings with a need for alternative options. The Global Fund to Fight AIDS, Tuberculosis and Malaria; United States Agency for International Development; Government of Germany; and WHO. For the Russian translation of the abstract see Supplementary Materials section. [ABSTRACT FROM AUTHOR]

Purpose: To estimate the incidence of Optic Neuritis (ON) in Colombia using data from the national health registry between January 1, 2015, and December 31, 2020. Methods: A population-based study was conducted using the Integrated Social Protection Information System from the Colombian Ministry of Health and the International Classification of Diseases-10 code for ON to estimate the incidence of ON from 2015 to 2020. We also evaluated the impact of the COVID-19 lockdown on the epidemiology of the disease in 2020. Finally, a standardized morbidity rate map was created to assess a country's ON geographic distribution. Results: From 2015 to 2019, 2,463 new cases of ON were reported. The overall 5-year incidence rate was 1.56 cases per 100,000 inhabitants per year, 66% of the patients were females. The peak of presentation in males was the quinquennium of 50–54 years, and in females, it was 45–49 years. In 2020, coinciding with the COVID-19 pandemic, there was a reduction in the incidence of ON by approximately 0.15 cases per 100,000 inhabitants. The regions with a high number of cases and an increasing risk of new cases were Bogotá, Antioquia, and Valle del Cauca. Conclusions: The incidence of ON in Colombia is lower compared to countries in the northern hemisphere, with women under 50 years being predominantly affected. The onset of the COVID-19 pandemic corresponded with a reduction in identifying new ON cases. This underscores governments' need to implement effective diagnostic strategies in the future. [ABSTRACT FROM AUTHOR]

Aspergillus-induced mycotic aneurysm is difficult to treat and often has poor outcomes with severe symptom progression. Early diagnosis is also difficult, and blood and cerebrospinal fluid tests often fail to reveal any findings. A 74-year-old man presented with recurrent nosebleeds in addition to symptoms of left optic neuritis. Contrast-enhanced computed tomography scan revealed a left internal carotid artery pseudoaneurysm protruding into the left Onodi cells, which was identified as the origin of bleeding. Endovascular left internal carotid artery occlusion was performed. One month postoperatively, external ophthalmoplegia and disorientation occurred. Although antibiotic treatment was continued for 1 month, consciousness loss and haematemesis occurred, and a new contralateral right internal carotid artery pseudoaneurysm ruptured, which resulted in death. At autopsy, Aspergillus infection centred on the skull base was pathologically found, although the sinus mucosal surface was normal. This case suggested a mycotic infection secondary to optic neuritis resulted in a left infectious pseudoaneurysm that spreads to the skull base and formed an aneurysm on the contralateral side 4 months thereafter. Therefore, the possibility that features of the Onodi cells contributed to the spread of inflammation inside and outside the skull and were involved in the formation of aneurysms inside and outside the dura mater was considered for the first time. [ABSTRACT FROM AUTHOR]

To report use trends of plasma exchange (PLEX) as well as sociodemographic and medical comorbidities associated with PLEX in the United States. Retrospective cross-sectional study. Adult patients (≥ 18 years) admitted for inpatient hospitalization with a primary diagnosis of optic neuritis (ON). Data from the National Inpatient Sample database was compiled to assess PLEX use rates between 2000 and 2020. The cohorts of patients receiving PLEX versus not receiving PLEX were analyzed between quarter 4 of 2015 through 2020 (International Classification of Diseases, Tenth Revision [ICD-10], only) for patient sociodemographic variables, medical diagnoses, insurance types, hospital characteristics, cause of disease, time to therapy, length of stay (LOS), and total charges incurred. Incidence of ON, incidence of PLEX, demographics, diagnoses associated with PLEX therapy, total charges, and LOS. From 2000 through 2020, 11 209 patients hospitalized with a primary diagnosis of ON were identified, with a significant majority managed at urban teaching hospitals. Use of PLEX increased steadily over 2 decades from 0.63% to 5.46%. Use was greatest in the western United States and least in the eastern United States. In the subset of ICD-10 cases, 3215 patients were identified. The median time to therapy of PLEX was 1 day after admission, and PLEX use was highest in patients with neuromyelitis optica spectrum disorder (NMOSD) (21.21%) and lowest in multiple sclerosis-associated ON (3.80%). Use of PLEX was associated with significantly longer LOS and higher total charges incurred. Medical comorbidities associated with PLEX included adverse reaction to glucocorticoids (adjusted odds ratio [aOR], 31.50), hemiplegia (aOR, 28.48), neuralgia (aOR, 4.81), optic atrophy (aOR, 3.74), paralytic strabismus (aOR, 2.36), and psoriasis (aOR, 1.76). Over the last 2 decades in the United States, PLEX therapy for ON has increased, with the highest use in the western United States and for patients with the diagnosis NMOSD ON. The author(s) have no proprietary or commercial interest in any materials discussed in this article. [ABSTRACT FROM AUTHOR]

Background: To clarify the risk of adverse ocular events following influenza vaccination. Methods: This self-controlled case series study used a claims database linked to vaccination records of a large city in Japan between April 2014 and September 2021. Individuals aged ≥ 65 years who developed adverse ocular events during the follow-up period were included. The exposure was influenza vaccination. The primary outcome was defined as the occurrence of at least one of the following five eye diseases: uveitis, scleritis, retinal vein occlusion, retinal artery occlusion, or optic neuritis. Conditional Poisson regression was used to estimate the within-subject incidence rate ratio of ocular adverse events during the risk period (0–56 days after vaccination) compared to the control period. Results: A total of 4,527 cases were eligible for the study (median age, 74 years; male, 42%). The incidence rate ratio for the outcome during the risk period was 0.99 (95% confidence interval, 0.87 to 1.14). No increased risk was observed for individual components of the outcome either; the incidence rate ratio was 0.94 (0.78 to 1.13) for uveitis, 1.17 (0.86 to 1.59) for scleritis, 0.98 (0.76 to 1.27) for retinal vein occlusion, 0.89 (0.42 to 1.87) for retinal artery occlusion, and 0.87 (0.44 to 1.70) for optic neuritis. Conclusions: This self-controlled case series showed no apparent increase in the risk of adverse ocular events after influenza vaccination among older adults. These results mitigate the concerns of older adults who may hesitate to receive influenza vaccination for fear of adverse ocular events. Abbreviation: HR = hazard ratio; CI = confidence interval; RVO = retinal vein occlusion; SCCS = self-controlled case series [ABSTRACT FROM AUTHOR]

Ocular involvement is not uncommon in patients with COVID-19. However, the incidence of COVID-19 ophthalmopathy in COVID-19 patients is still not clear. In this prospective case series study, we recruited 2445 consecutive cases presenting at Neuro-ophthalmology clinic of our Eye Center during the last resurgence of SARS-CoV-2 infection from 8 December 2022 to 15 March 2023 in China, 149 cases were diagnosed as COVID-19 ophthalmopathy, 87 cases were female, with a mean age of 43.2 years, and the mean follow-up time was 15.4 weeks. One hundred and twenty of 149 cases suffered from systemic symptoms mostly manifesting as fever, cough and muscle pain prior to or soon after ocular involvement. The most common COVID-19 ophthalmopathy was optic neuritis (51/149), followed by acute zonal occult outer retinopathy complex disease (31/149), uveitis (17/149), ocular mobility disorder-related (third, fourth, or sixth) cranial nerve neuritis (15/149), anterior ischaemic optic neuropathy (9/149), retinal artery occlusion (8/149), retinal microangiopathy including retinal haemorrhage and cotton wool spot (8/149), viral conjunctivitis (7/149), retinal vein occlusion (3/149), viral keratitis (2/149), ptosis (2/149), and other rare ocular diseases. Except 5 cases with central retinal artery occlusion, other 144 COVID-19 ophthalmopathy cases showed good response to steroid therapy. Our study revealed an incidence of 6.09% for COVID-19 ophthalmopathy in outpatients at our Neuro-ophthalmology clinic during last resurgence of COVID-19 in China, and demonstrated that SARS-CoV-2 infection could induce an initial onset or a relapse of ophthalmic diseases, and that ocular involvement might manifest as the initial or even the only presentation of COVID-19. [ABSTRACT FROM AUTHOR]

Background: Multiple sclerosis (MS) and Takayasu's arteritis (TAK) are two autoimmune diseases that affect the Central nervous system (CNS), but the relationship between them has not been established. Case Presentation: Here we report the emergence of MS during treatment. Takayasu's arteritis in a 24-year-old Iranian woman with a severe presentation. She was treated aggressively with IV methylprednisolone 1 g/day for 3 days and continued with oral prednisolone, also IV cyclophosphamide monthly. After 2 months, loss of vision led to a diagnosis of Optic neuritis (ON) caused by concomitant MS. Conclusion: Differentiating CNS vasculitis associated with Takayasu's arthritis from coexisting MS affecting the CNS is challenging and what is important is to avoid giving a TNF inhibitor. [ABSTRACT FROM AUTHOR]

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Objectives: To evaluate the long-term anatomical and functional prognosis of patients with retinal detachment (RD) secondary to acute retinal necrosis (ARN) treated with pars plana vitrectomy (PPV). Methods: This retrospective case series included 21 eyes from 21 patients with RD secondary to ARN. The study analyzed vitreous or aqueous biopsy results, the impact of antiviral therapy, time to retinal detachment, changes in visual acuity (VA), and anatomical and surgical outcomes. All cases underwent 23-gauge PPV with silicone oil tamponade, and an episcleral encircling band was used in 11 cases. All patients received systemic antiviral therapy at diagnosis. Results: Retinal reattachment was achieved in 91% of cases during follow-up, with an average follow-up period of 39.5 ± 36.8 months. The average time from ARN diagnosis to RD onset was 33.3 ± 27.5 days. VZV was detected in 10 eyes through PCR analysis. Significant differences in visual prognosis were found between macula-off and macula-on RD (p = 0.048). Eyes with optic nerve head inflammation had worse final VA (p = 0.010). No significant difference was observed between preoperative VA and VA at the end of follow-up (p = 0.665). Conclusions: VZV was the primary virus associated with ARN-related RD. Early involvement of the macula and optic nerve in retinitis negatively impacted the final visual prognosis. [ABSTRACT FROM AUTHOR]

Background: Rhinosinusitis is a common childhood illness that may result in a number of ocular problems. The goal of this thorough analysis is to compile the available data regarding the kinds, prevalence, and treatment of ocular problems related to juvenile rhinosinusitis. Methods: A comprehensive analysis of electronic databases, such as PubMed, Embase, and Cochrane Library, was carried out to pinpoint pertinent research articles written in English and published between the beginning and August of 2023. Included were studies that discuss ocular problems in young children suffering from rhinosinusitis. Study characteristics, ocular complication kinds, occurrence rates, and management approaches were the main topics of data extraction. Results: A significant number of pertinent research on ocular problems related to juvenile rhinosinusitis was found through the literature search. Preliminary searches indicated that orbital cellulitis, subperiosteal abscess, cavernous sinus thrombosis, and optic neuritis are probably the most often reported ocular problems. It is expected that these problems will occur at a wide range of incidence rates, from somewhat unusual to more prevalent. Depending on the severity of the consequences, management techniques are expected to require a combination of surgical and medicinal procedures. Healthcare professionals will benefit from the findings since they will improve knowledge of the clinical presentation, diagnosis, and treatment of these potentially blinding consequences. The evaluation also assists in identifying knowledge gaps and guides future directions for this field of study, both of which are critical to enhancing patient outcomes. Conclusions: The wide range of topics included in this study will help to better understand the burden of ocular consequences related to juvenile rhinosinusitis and will make it easier to build preventative and treatment plans that work better. [ABSTRACT FROM AUTHOR]

Leber's Hereditary Optic Neuropathy (LHON) is a maternally inherited optic nerve disease primarily caused by mutations in mitochondrial DNA (mtDNA). The peak of onset is typically between 15 and 30 years, but variability exists. Misdiagnosis, often as inflammatory optic neuritis, delays treatment, compounded by challenges in timely genetic diagnosis. Given the availability of a specific treatment for LHON, its early diagnosis is imperative to ensure therapeutic appropriateness. This work gives an updated guidance about LHON differential diagnosis to clinicians dealing also with multiple sclerosi and neuromyelitis optica spectrtum disorders-related optic neuritis. LHON diagnosis relies on clinical signs and paraclinical evaluations. Differential diagnosis in the acute phase primarily involves distinguishing inflammatory optic neuropathies, considering clinical clues such as ocular pain, fundus appearance and visual recovery. Imaging analysis obtained with Optical Coherence Tomography (OCT) assists clinicians in early recognition of LHON and help avoiding misdiagnosis. Genetic testing for the three most common LHON mutations is recommended initially, followed by comprehensive mtDNA sequencing if suspicion persists despite negative results. We present and discuss crucial strategies for accurate diagnosis and management of LHON cases. [ABSTRACT FROM AUTHOR]

Optic neuritis (ON) is characterized by inflammatory lesions involving the optic nerve, with idiopathic optic neuritis being the main subtype. In recent years, a deeper understanding of idiopathic optic neuritis led to the identification of additional diseases corresponding changes in treatment approaches. Furthermore, biological immunotherapy has entered a new era, with some treatments recommended as first-line options according to relevant guidelines. This article introduces and summarizes the standardized application and progress of biological agents in ON, especially in idiopathic demyelinating optic neuritis, aiming to provide a reference for standardized treatment. [ABSTRACT FROM AUTHOR]

Over the past two decades, significant advancements have been made in our understanding of optic neuritis (ON) characterized by demyelination. With the progression of serological testing technologies, a unique antibody associated with a specific subtype of demyelinating neuropathy was discovered in recent years — myelin oligodendrocyte glycoprotein (MOG) antibody. Although MOG antibody- associated disorders (MOGAD) were initially considered to be part of the spectrum of neuromyelitis optical spectrum disorders (NMOSD), accumulating research evidence suggests that it should be regarded as a distinct disease entity. It is crucial to differentiate MOGAD from aquaporin-4 (AQP4) antibody-positive NMOSD because they have different clinical courses and prognoses, necessitating tailored diagnostic and treatment approaches. To enhance the level of diagnosis and treatment and enable greater benefits for these patients, there is a need for an in-depth comprehension of the clinical presentations and therapeutic advancements in MOG-ON. This will contribute to providing improved medical care for a broader range of neuro-ophthalmic patients. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the clinical and image characteristics of primary systemic vasculitis-associated optic neuritis patients. Methods: This is a retrospective study. The patients clinically diagnosed with primary system vasculitis-induced optic neuritis were recruited from March 2013 to December 2023. All cases received orbital magnetic resonance imaging scans were analyzed. The ocular findings, systemic manifestations, laboratory data and prognosis were reviewed retrospectively. In addition, the related literature was reviewed. Results: Fourteen patients (21 eyes), including 10 men and 4 women, were enrolled in this study. The ages ranged from 30 to 86 years in this cohort. Orbits MRI detects the enlargement and/or enhancement of the optic nerve. Cases 1–5 reported a confirmed diagnosis of Takayasu's arteritis, and cases 6–8 had giant cell arteritis. Cases 9–13 were antineutrophil cytoplasmic antibody-associated vasculitis. Case 14 was Cogan's syndrome. Mult organs and tissues, such as the kidneys, heart, paranasal sinuses, meninges, and respiratory system, were involved. In all of the 14 involved patients, the disease onset was either during the fall or winter season. There were no or only slight improvements in visual activity after conventional therapies. Conclusions: The autoantibodies' attack on the optic nerve, ischemic damage, or destruction of the blood–brain barrier may be the potential pathogenesis of vasculitis-associated optic neuritis. Even with prompt and aggressive clinical interventions, the prognosis remains unsatisfactory. [ABSTRACT FROM AUTHOR]

Introduction: Neuromyelitis Optica spectrum disorder (NMOSD) is an autoimmune disease characterized by anti-aquaporin-4 (AQP4) autoantibodies. The discovery of antibodies AQP4 and myelin oligodendrocyte glycoprotein (MOG) has expanded our understanding of the pathogenesis of neuromyelitis optica. However, the molecular mechanisms underlying the disease, particularly AQP4-associated optic neuritis (AQP4-ON), remain to be fully elucidated. Methods: In this study, we utilized Weighted Gene Co-expression Network Analysis (WGCNA) to investigate the transcriptomic profiles of peripheral blood samples from patients with AQP4-ON and MOG-positive optic neuritis (MOG-ON), compared to healthy controls. Results: WGCNA revealed a brown module (ME brown) strongly associated with AQP4-ON, which correlated positively with post-onset visual acuity decline. A total of 132 critical genes were identified, mainly involved in histone modification and microtubule dynamics. Notably, genes HDAC4, HDAC7, KDM6A, and KDM5C demonstrated high AUC values in ROC analysis, indicating their potential as biomarkers for AQP4-ON. Conclusion: Our findings provide novel insights into the molecular signature of AQP4-ON and highlight the potential of systems biology approaches in identifying biomarkers for NMOSD. The identified histone modification genes warrant further investigation for their role in disease pathogenesis and as therapeutic targets. [ABSTRACT FROM AUTHOR]

Purpose: To explore ocular changes in patients with MS and NMOSD via SDOCT and PVEP analysis. Methods: From August 2020 to July 2021, 82 patients (164 eyes) diagnosed with MS, 59 patients (118 eyes) diagnosed with NMOSD and 50 healthy controls (100 eyes) were retrospectively selected. SD-OCT and PVEP were performed to compare retinal nerve fibre layer (RNFL) thickness around the optic disc, ganglion cell inner plexiform layer (GCIPL) thickness in the macula and P100 latency and amplitude between the disease groups and the control group. Results: In the NMOSD and MS groups, the thickness of the GCIPL quadrants in eyes with optic neuritis was thinner than that in eyes without optic neuritis, and the amplitude of the P100 wave decreased. In addition, in eyes with optic neuritis, patients with NMOSD have thinner RNFL thicknesses in the temporal and superior quadrants than patients with MS, and the thickness of the GCIPL is thinner in each region. In eyes without optic neuritis, patients with MS have thinner nasal RNFL than do those with NMOSD. Conclusion: SD-OCT and VEP may be useful for monitoring and distinguishing pathological changes in MS and NMOSD patients. [ABSTRACT FROM AUTHOR]

Background: Neuromyelitis optica (NMO or Devic's syndrome), characterized by acute attacks of optic neuritis and transverse myelitis, is associated with antiaquaporin 4 antibodies (anti-NMO-IgG) and it has a higher prevalence in non-Caucasian populations, where multiple sclerosis is less common. Clinical case: 41-year-old man, with no significant personal medical history, presented with clinical symptoms including paresthesias in the right hemibody and homonymous hemianopsia in the left eye. Three weeks later, he developed lumbalgia associated with paresthesias in both lower limbs up to the T7 sensory level, as well as acute urinary retention, followed by paraplegia. The magnetic resonance imaging (MRI) displayed multiple hyperintense lesions on T2 and FLAIR sequences and hypointense on T1, located in the periventricular white matter, corpus callosum, bilateral corona radiata, as well as at the spinal level C2-C3, without post-contrast enhancement. The MRI of the spine revealed poorly defined hyperintense areas on T2 sequence, with dilation of the spinal canal from T4 to T11. A qualitative test for anti-NMO-IgG antibodies was positive, confirming the diagnosis of NMO. Conclusion: NMO presents a diagnostic challenge. Timely diagnosis and management are crucial to prevent disability, future exacerbations, and to improve prognosis. [ABSTRACT FROM AUTHOR]

Objective: The first international consensus criteria for optic neuritis (ICON) were published in 2022. We applied these criteria to a prospective, global observational study of acute optic neuritis (ON). Methods: We included 160 patients with a first‐ever acute ON suggestive of a demyelinating CNS disease from the Acute Optic Neuritis Network (ACON). We applied the 2022 ICON to all participants and subsequently adjusted the ICON by replacing a missing relative afferent pupillary defect (RAPD) or dyschromatopsia if magnetic resonance imaging pathology of the optical nerve plus optical coherence tomography abnormalities or certain biomarkers are present. Results: According to the 2022 ICON, 80 (50%) patients were classified as definite ON, 12 (7%) patients were classified as possible ON, and 68 (43%) as not ON (NON). The main reasons for classification as NON were absent RAPD (52 patients, 76%) or dyschromatopsia (49 patients, 72%). Distribution of underlying ON etiologies was as follows: 78 (49%) patients had a single isolated ON, 41 (26%) patients were diagnosed with multiple sclerosis, 25 (16%) patients with myelin oligodendrocyte glycoprotein antibody‐associated disease, and 15 (9%) with neuromyelitis optica spectrum disorder. The application of the adjusted ON criteria yielded a higher proportion of patients classified as ON (126 patients, 79%). Interpretation: According to the 2022 ICON, almost half of the included patients in ACON did not fulfill the requirements for classification of definite or possible ON, particularly due to missing RAPD and dyschromatopsia. Thorough RAPD examination and formal color vision testing are critical to the application of the 2022 ICON. [ABSTRACT FROM AUTHOR]

Background: Optic neuropathies represent one of the most frequent causes of vision loss, and they can manifest alone or in conjunction with neurological or systemic symptoms and signs. In recent years, the diagnostic techniques used to detect optic neuropathies have significantly improved, facilitating diagnosis and improving treatment. Among these, ocular ultrasound has assumed a fundamental role, although with conflicting results in the published scientific literature. For this reason, the aim of this review is to analyze the role of ocular ultrasound in the precise and targeted diagnosis of optic neuropathies to better understand the presumed potential of this precious diagnostic tool in the management of these ocular and neurological disorders. Methods: We carried out a search on PubMed and Scopus utilizing terms related to optic neuropathies and ocular ultrasound, including only relevant English full-length research articles, case reports, or case series. Results: Most of the papers published in the scientific literature use only the B-scan ultrasound technique without considering the more precise and objective standardized A-scan technique that allows for performing more accurate diagnostic tests, such as the "30-degree test" and the "optic nerve exercise test". Conclusions: Future clinical trials and research on optic neuropathies should also consider the use of the standardized A-scan technique in order to compare clinical findings not only with B-scan ultrasonography but also with other noninvasive procedures that could be helpful in reaching the correct diagnosis. [ABSTRACT FROM AUTHOR]

This article discusses a case of a 54-year-old Chinese woman with SAPHO syndrome, a rare auto-inflammatory disorder characterized by skin lesions and osteoarticular involvement, combined with optic neuritis. The patient experienced symptoms such as palmoplantar pustulosis, chest wall pain, and blurred vision in the right eye. After unsuccessful treatment with corticosteroids, the patient was switched to tofacitinib, which resulted in significant improvement in skin lesions, bone pain, and vision. The article suggests that tofacitinib may be an effective treatment option for ocular involvement in SAPHO syndrome when conventional treatments are ineffective. [Extracted from the article]

Key Clinical Message: In patients with SLE, concurrent NMOSD can manifest with optic neuritis and transverse myelitis. AQP‐4 antibody positivity confirms the diagnosis. Prompt treatment is critical to manage the acute symptoms and prevent relapses, as highlighted by a young patient's case with optic neuritis and extensive spinal cord lesions. Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system that affects the optic nerve and spinal cord. It is associated with autoantibodies against aquaporin‐4 (AQP‐4) and/or myelin oligodendrocytes glycoproteins. It is diagnosed based on clinical, radiological, and serological criteria, and treated with immunosuppressants in the acute phase. Long‐term immunosuppression is essential to prevent potential relapses. In this case report, we present the case of a 19‐year‐old female patient with systemic lupus erythematosus (SLE), who presented with blurriness and loss of vision in her left eye. Optical coherence tomography was normal, but a gadolinium‐enhanced cervico‐dorsal MRI showed multiple lesions extending from the brainstem to the C7‐T1 junction suggestive of longitudinally extensive transverse myelitis (LETM), the largest of which was a cystic lesion at the cervico‐spinal junction. A contrast injection also revealed left optic neuritis. Cerebrospinal fluid analysis showed elevated IgG and red blood cell count, but no oligoclonal bands. The patient tested positive for AQP‐4 autoantibodies, confirming the diagnosis of NMOSD. Treatment with intravenous methylprednisolone led to partial improvement, but the patient experienced a relapse with severe neurological symptoms, including tetraplegia and bladder and bowel dysfunction. This case illustrates the importance of considering NMOSD in the differential diagnosis of patients with SLE who present with optic neuritis and/or myelitis, especially when MRI findings are suggestive of LETM. Early diagnosis and adherence to treatment are crucial to prevent further relapses and deleterious sequelae. [ABSTRACT FROM AUTHOR]

Background/Objectives: Retinal hyperreflective foci, 25–50 µm in diameter, that can be imaged by noninvasive optical coherence tomography (OCT) may represent microglial activity related to inflammation. This study aimed to detect hyperreflective foci in the OCT-hyporeflective avascular outer nuclear layer of the retina in relapsing–remitting MS (RRMS) patients without ongoing eye or optic nerve disease. Methods: A cohort of 13 RRMS patients (8 eyes with and 18 eyes without prior optic neuritis) underwent retinal OCT at baseline, after 1 month, after 6 months, and then every 6 months for 3 years. The data were compared with single-examination data from 106 eyes in 53 age-matched healthy subjects. Results: The prevalence of hyperreflective foci at baseline was higher in RRMS patients than in healthy subjects (46.2% vs. 1.8%, p < 0.005). Patients with optic neuritis had much more foci than those without (p < 0.001). Hyperreflective foci recurred in 23.1% of RRMS patients, bilaterally in one with prior optic neuritis and unilaterally in two without. Conclusions: Patients with RRMS, notably those with prior optic neuritis, had elevated rates of retinal infiltration in the absence of retinal disease, suggesting that the phenomenon may represent elevated activity of an immune surveillance or housekeeping mechanism rather than retinal disease. [ABSTRACT FROM AUTHOR]

Background: Studies on the capability of cerebrospinal fluid neurofilament light chain (cNfL) to predict multiple sclerosis (MS) conversion in clinically isolated syndromes have yielded varying results. Objectives: To 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. Methods: cNfL 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. Results: 20.5% 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. Conclusion: cNfL 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. [ABSTRACT FROM AUTHOR]

To determine the risk of optic neuritis (ON) after mRNA Coronavirus Disease 2019 (COVID-19) vaccine administration. U.S. National aggregate database retrospective cohort study. Patients were placed into cohorts based on mRNA COVID-19 vaccination status (no vaccine and positive history of COVID-19 infection, 1 vaccine, or 2 vaccines received) from December 2020 to June 2022. Two control cohorts were created with patients vaccinated against influenza or tetanus, diphtheria, and pertussis (Tdap) from June 2018 to December 2019. Patients with any history of ON or significant risk factors for ON development including infectious, inflammatory, and neoplastic diseases were excluded. A large deidentified database was queried for the Common Procedural Technology 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 after vaccination was calculated and compared for all 5 cohorts with 95% confidence intervals (CIs) reported. Risk ratio (RR) of ON 21 days after vaccination (or COVID-19 infection) and incidence of ON per 100 000 individuals. After 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] years and 43.2 [25.5] years, 55% female) the RR of developing ON was 0.44 (95% CI, 0.28–0.80). The first dose of COVID-19 and Tdap vaccinations (n = 797 538, mean age 38.9 [20.0] years, 54.2% female) cohort had 10 and 16 patients develop ON (RR, 0.63; 95% CI, 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, 0.09; 95% CI, 0.07–0.12). The incidence per 100 000 for ON was 1 in the first dose COVID-19 vaccine cohort, 2 in the influenza cohort, and 2 in the Tdap cohort, and 14 in the COVID-19–infected and unvaccinated cohorts. Risk of ON after mRNA COVID-19 vaccination is rare and comparable to Tdap vaccination, decreased compared with influenza vaccination, and decreased compared with COVID-19 infection in the absence of vaccination. Proprietary or commercial disclosure may be found after the references. [ABSTRACT FROM AUTHOR]

Purpose: Optic neuritis is the most common optic neuropathy affecting adults, especially women. Lactational optic neuritis is a rare entity occurring during the postpartum period with a high chance of recurrence. Through this study, we evaluated the clinical profile and visual outcome of lactational optic neuritis. Methods: This was a retrospective study where patients with optic neuritis presenting during the first year of the postpartum period to the neuro-ophthalmology clinic between January 2016 and December 2022 were included in the study. Results: This study included seven eyes of six patients. The mean age of presentation was 24.6 years, with all patients presenting after the first 3 months of the postpartum period. All patients had unilateral involvement except one, presenting with painful vision loss. Best corrected visual acuity ranged from 6/18 to hand movements. Relative afferent pupillary defect and dyschromatopsia were present in all patients. Disc edema was seen in all but one case. Three patients were treated with steroids along with multivitamins, and the remaining three refused treatment with steroids, receiving multivitamins alone. All patients had complete recovery by the end of 12 weeks. Conclusion: Optic neuritis during lactation is attributed more to post-pregnancy immunological changes rather than lactation itself. The presentation and clinical features are similar to classic optic neuritis and remain an important differential diagnosis for visual dysfunction in the postpartum period.