Myelin Oligodendrocyte Glycoprotein Antibody-associated Disease Research Articles

Advancing Optical Coherence Tomography Diagnostic Capabilities: Machine Learning Approaches to Detect Autoimmune Inflammatory Diseases.

In many parts of the world including India, the prevalence of autoimmune inflammatory diseases such as neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and multiple sclerosis (MS) is rising. A diagnosis is often delayed due to insufficient diagnostic tools. Machine learning (ML) models have accurately differentiated eyes of patients with MS from those of healthy controls (HCs) using optical coherence tomography (OCT)-based retinal images. Examining OCT characteristics may allow for early differentiation of these conditions. The objective of this study was to determine feasibility of ML analyses to distinguish between patients with different autoimmune inflammatory diseases, other ocular diseases, and HCs based on OCT measurements of the peripapillary retinal nerve fiber layer (pRNFL), ganglion cell-inner plexiform layer (GCIPL), and inner nuclear layers (INLs). Eyes of people with MS (n = 99 patients), NMOSD (n = 40), MOGAD (n = 74), other ocular diseases (OTHER, n = 16), and HCs (n = 54) from the Mangalore Demyelinating Disease Registry were included. Support vector machine (SVM) classification models incorporating age, pRNFL, GCIPL, and INL were performed. Data were split into training (70%) and testing (30%) data and accounted for within-patient correlations. Cross-validation was used in training to choose the best parameters for the SVM model. Accuracy and area under receiver operating characteristic curves (AUROCs) were used to assess model performance. The SVM models distinguished between eyes of patients with each condition (i.e., MOGAD vs NMOSD, NMOSD vs HC, MS vs OTHER, etc) with strong discriminatory power demonstrated from the AUROCs for these comparisons ranging from 0.81 to 1.00. These models also performed with moderate to high accuracy, ranging from 0.66 to 0.81, with the exception of the MS vs NMOSD comparison, which had an accuracy of 0.53. ML models are useful for distinguishing between autoimmune inflammatory diseases and for distinguishing these from HCs and other ocular diseases based on OCT measures. This study lays the groundwork for future deep learning studies that use analyses of raw OCT images for identifying eyes of patients with such disorders and other etiologies of optic neuropathy.

Brain and Spine Magnetic Resonance Imaging (MRI) Characteristics of a Pediatric Cohort With MOGAD.

Myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently defined demyelinating disorder with an age-related phenotypic spectrum. At disease onset, there is considerable clinical overlap between MOGAD and other demyelinating conditions, and it remains difficult to identify MOGAD radiographically. This study aims to further describe neuroimaging findings in the brain and the spine at presentation and throughout relapses in children with MOGAD. We present a retrospective cohort study including all children presenting to a single center between 2010 and 2020 with acute demyelination who were positive for serum MOG-IgG antibodies and negative for serum aquaporin-4 antibodies. For each patient, magnetic resonance imaging (MRI) scans of the brain and spine at presentation and on each relapse were reviewed and categorized in a blinded fashion by 2 pediatric neuroradiologists. Sixteen patients met the inclusion criteria. Four had diffuse and bilateral fluid-attenuated inversion recovery signal in the white matter, but only on initial presentation. The area postrema was never affected. All 5 patients with optic neuritis had pre-chiasmatic (but not chiasmatic) involvement on presentation. The brachium pontis was involved in 3 patients on initial presentation, and in 8 patients at any time. Eleven patients demonstrated spinal cord involvement, and the cervical, thoracic, and lumbar regions were involved at similar frequencies. The radiographic features of MOGAD in children appear to reflect their presenting demyelinating syndromes. However, certain features, such as diffuse fluid-attenuated inversion recovery hyperintensities and expansile fluid-attenuated inversion recovery signal in the brachium pontis, may be more frequent in MOGAD compared with other demyelinating disorders.

MRI management for NMOSD and MOGAD: Proposals from the French Expert Group NOMADMUS

BackgroundCurrently, there are no available recommendations or guidelines on how to perform MRI monitoring in the management of neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). The issue is to determine a valuable MRI monitoring protocol to be applied in the management of NMOSD and MOGAD, as previously proposed for the monitoring of multiple sclerosis. ObjectivesThe objectives of this work are to establish proposals for a standardized and feasible MRI acquisition protocol, and to propose control time points for systematic MRI monitoring in the management of NMOSD and MOGAD. MethodsA steering committee composed of 7 neurologists and 5 neuroradiologists, experts in NMOSD and MOGAD from the French group NOMADMUS, defined 8 proposals based on their expertise and a review from the literature. These proposals were then submitted to a Rating Group composed of French NMOSD / MOGAD experts. ResultsIn the management of NMOSD and MOGAD, a consensus has been reached to perform systematic MRI of the brain, optic nerve and spinal cord, including cauda equina nerve roots, at the time of diagnosis, both without and after gadolinium administration. Moreover, it has been agreed to perform a systematic MRI scan 6 months after diagnosis, focusing on the area of interest, both without and after gadolinium administration. For long-term follow-up of NMOSD and MOGAD, and in the absence of clinical activity, it has been agreed to perform gadolinium-free MRI of the brain (+/- optic nerves) and spinal cord, every 36 months. Ideally, these MRI scans should be performed on the same MRI system, preferably a 3T MRI system for brain and optic nerve MRI, and at least a 1.5T MRI system for spinal cord MRI. ConclusionsThis expert consensus approach provides physicians with proposals for the MRI management of NMOSD and MOGAD.

The value of CSF diagnostic and prognostic biomarkers in NMOSD and MOGAD in real-life use.

Diagnosing neuromyelitis optica spectrum disorder (NMOSD) may be challenging owing to overlapping clinical features with multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), particularly in AQP4-IgG seronegative NMOSD patients. We evaluated cerebrospinal fluid (CSF) biomarkers, specifically glial fibrillary acidic protein (GFAP) and albumin quotient (QAlb), to improve diagnostic accuracy in NMOSD. In this retrospective study, we analyzed CSF samples for biomarkers GFAP, QAlb, neurofilament light, and total-Tau, from patients with AQP4-NMOSD, DNNMOSD, MOGAD, and MS in the Region Västra Götaland, Sweden. Receiver operating characteristic (ROC) analysis with calculation of the area under the curve (AUC) was used to identify optimal cut-off levels for discriminating AQP4-NMOSD from the other groups. Logistic regression models assessed the diagnostic power of GFAP and QAlb combined. Patients with AQP4-NMOSD (N = 19) had significantly higher CSF GFAP levels than the others: median 2470 ng/L vs 330 ng/L (p < 0.001). The GFAP cutoff >715 ng/L gave a sensitivity of 81 % and specificity of 92 %. Combining GFAP and QAlb further increased the diagnostic accuracy (AUC = 0.96). MOGAD patients (N = 29) had the highest CSF lymphocyte counts, with elevated lymphocyte counts correlating with polyphasic MOGAD (R = 0.63; p = 0.016). CSF GFAP is a valuable biomarker for distinguishing AQP4-NMOSD from other demyelinating diseases: Combining GFAP with QAlb enhances diagnostic precision.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) as a cause of new-onset refractory status epilepticus (NORSE): Case report and review of literature.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare cause of NORSE. We describe the case of a young girl who presented with NORSE associated with MOGAD along with a systematic review of all cases of NORSE associated with MOGAD till date. Seizures associated with MOGAD are usually associated with good outcome but can occasionally be catastrophic and non-responsive to conventional therapies. Early initiation of anti-IL6 therapies might help improve outcomes in such patients.

Painand Headache in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

The purpose of this review is to evaluate the current knowledge and recent findings on different pain and headache presentations associated with Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) disease. MOGAD is an inflammatory autoimmune disease affecting mostly the central nervous system, presenting with optic neuritis, transverse myelitis and other forms of inflammatory demyelination. Pain and headache in MOGAD have been recognized more recently and acute and chronic forms of pain can occur in both the adult and pediatric population. An important recent observation is that MOGAD can affect the peripheral nervous system, which can contribute to pain symptoms. Acute and chronic forms of pain have been described in MOGAD, including ocular pain, different types of headaches and neuropathies, involving the central and peripheral nervous system. Awareness of these types of pain in both the pediatric and adult population is crucial, to ensure timely diagnosis and treatment.

Challenges in the Diagnosis and Management of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD).

Myelin oligodendrocyte glycoprotein antibody-associated disease has been recently identified to be a distinct autoimmune central nervous system disorder. There is significant clinical and radiological overlap with multiple sclerosis and aquaporin-4-IgG-associated neuromyelitis optica spectrum disorders. Clinical course is variable in that patients may have a monophasic or relapsing course, disease severity is unpredictable, and unlike other idiopathic autoimmune inflammatory disorders, there is no gender predilection and it is more likely to affect pediatric population. There are no clear-cut treatment guidelines. Duration and dose of oral steroids after the first attack, role of immunosuppressants in relapsing disease, and duration of therapy for the latter are not certain. Currently, there are no disease-specific therapies available, though some novel therapies are under trial. Some of these challenges will be addressed in this paper.

Performance of the 2023 diagnostic criteria for MOGAD: real-world application in a Chinese multicenter cohort of pediatric and adult patients

BackgroundThe clinical phenotypes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have been found to overlap with several other diseases. The new criteria proposed in 2023 were designed to better identify the disease but require validation across various populations to ascertain its clinical utility. We aimed to investigate the diagnostic performance in phenotypically diverse patients.MethodsThis multicenter study retrospectively included adult and pediatric patients who were hospitalized for a first suspected demyelinating event and tested positive for MOG immunoglobulin G (IgG) during the acute phase. The 2023 Lancet Neurology criteria were assessed against the benchmark of empirical clinical diagnosis, and the 2018 JAMA Neurology and Journal of Neuroinflammation criteria were also evaluated for comparative analysis.ResultsAmong the 291 eligible patients (82 adults, 209 children), 282 (96.9%) were clinically diagnosed as definite MOGAD (77 adults, 205 children), while 262 (90.0%) fulfilled the 2023 diagnostic criteria (78 adults, 184 children). A total of 265 patients met the criteria for core clinical demyelinating events, and 76 (26.1%) had serum clear positive MOG-IgG (≥ 1:100). The sensitivity of the 2023 criteria was 0.91 (adults vs. children = 0.97 vs. 0.89), the specificity was 0.56 (adults vs. children = 0.40 vs. 0.75), positive likelihood ratio was 2.06 (adults vs. children = 1.62 vs. 3.57), and negative likelihood ratio (NLR) was 0.15 (adults vs. children = 0.06 vs. 0.14). Additionally, 264 and 256 cases were classified as definite MOGAD by the 2018 JAMA Neurology and Journal of Neuroinflammation criteria, respectively. Compared to the 2023 diagnostic criteria, the 2018 JAMA Neurology criteria demonstrated similar diagnostic performance. However, the 2018 Journal of Neuroinflammation criteria exhibited comparable sensitivity (0.92, adults vs. children = 0.96 vs. 0.89), higher specificity (1.00, adults vs. children = 1.00 vs. 1.00) and better NLR (0.09, adults vs. children = 0.04 vs. 0.11).ConclusionsThe 2023 criteria demonstrated good sensitivity in adult and pediatric patients in China yet modest specificity. Close follow-up is needed for patients with atypical phenotypes but high-titer MOG-IgG to avoid underdiagnosis.

Evaluation of the predictive value of CSF-restricted oligoclonal bands on residual disability and risk of relapse in adult patients with MOGAD: MOGADOC study.

The clinical course of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is variable. However, robust markers of poor outcome and/or relapse risk are still missing. To evaluate the frequency of cerebrospinal fluid-restricted oligoclonal bands (CSF-OCB) in a national cohort of adult MOGAD patients and to assess their prognostic value for the risk of relapse and severity. We included MOGAD adult patients fulfilling the MOGAD 2023 criteria who underwent CSF analysis at maximum 3 months from onset. Data from 190 patients were collected. We found the presence of CSF-OCB in 32 patients (16.8%). Positive and negative CSF-OCB patients were similar for median age at onset, sex, clinical presentation, severity at onset, and residual disability. Relapses were more frequent in the CSF-OCB+ group (p = 0.049), particularly within the first year of follow-up (p = 0.007). Although CSF-OCB+ was more frequently associated with imaging features suggestive of multiple sclerosis (MS) (p = 0.014), 78% of these patients fulfilled the 2023 supportive features and 65% experienced lesion vanishing at follow-up magnetic resonance imaging (MRI). We found a higher risk of relapse in MOGAD with CSF-OCB particularly during the first year. Close attention is recommended regarding the risk of misdiagnosis with MS.

MOG antibody-associated disease epidemiology in Olmsted County, USA, and Martinique.

To report myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) epidemiology in two American regions using 2023 diagnostic criteria. We compared age- and sex-adjusted incidence and prevalence of MOGAD per 2023 diagnostic criteria in Olmsted County (Minnesota [USA]) and Martinique (Caribbean [FR]) (01/01/2003-12/31/2018, prevalence day) using Poisson regression. Archived sera in 68-85% were available for MOG-IgG testing by live cell-based assay at Mayo Clinic. Of 21 patients with MOG-IgG positivity identified, 16 fulfilled MOGAD criteria (38% female; median age of 27years, interquartile-range [IQR 23-42]) and five with low-positive MOG-IgG did not (optic neuritis lacking supportive criteria, 2; alternative diagnosis of multiple sclerosis, 3). MOGAD prevalence was similar in Olmsted County (3.70/100,000, 95% confidence interval [CI] 0.74-6.66]) and Martinique (2.61/100,000; 95% CI 0.85-4.37, P = 0.46). MOGAD incidence was 3.00/million-person-years (95% CI 0.78-5.22) in Olmsted County and 1.18/million-person-years (95% CI 0.30-2.07) in Martinique (P = 0.08). Children represented 29% of MOGAD in Olmsted County and 11% in Martinique. During their disease course the attacks included: optic neuritis (13/16 [81%]); myelitis (6/16 [38%]); and acute disseminated encephalomyelitis (2/16 [13%]). The proportion of MOGAD among incident CNS demyelinating diseases was greater in children (13-14%) than adults (2-4%; P = 0.005). At last follow-up (median, 5years, IQR 2-9), the median EDSS was 1.0 (IQR 0.5-2.75) with 1/16 (6%) blind in one eye and 9/16 (56%) had relapsing MOGAD. This study provides estimates of incidence and prevalence of MOGAD in the USA and Martinique and shows that, although children are predisposed, the disease is spread broadly across the age spectrum and population-based outcomes are favorable.

18F-FAZA, 18F-FDG, and 123I-IMP Imaging Reveal Hypoxia and Metabolism in Acute MOGAD.

We report the first case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) evaluated with hypoxic imaging using 18F-FAZA PET/CT. A healthy woman in her 20s presented to our hospital with seizures, headaches, and vomiting. MRI and CT scans suggested a wide range of differential diagnoses, from neoplastic lesions, such as malignant lymphoma, to inflammatory diseases, such as vasculitis, making her case challenging to diagnose. MOGAD was confirmed by positive myelin oligodendrocyte glycoprotein antibodies, and her condition improved with steroid therapy. The 18F-FAZA PET/CT findings in this case may aid in understanding the pathogenesis of MOGAD.

Clinical characteristics of anti-myelin oligodendrocyte glycoprotein antibody among aquaporin-4 negative neuromyelitis optica spectrum disorders in Egyptian patients

Some patients with neuromyelitis optica spectrum disorder (NMOSD)-like symptoms test negative for anti-aquaporin-4 (anti-AQP4) antibodies. Among them, a subset has antibodies targeting myelin oligodendrocyte glycoprotein (MOG), a condition now termed MOG antibody-associated disease (MOGAD). MOGAD shares features with NMOSD, like optic neuritis and myelitis, but differs in pathophysiology, clinical presentation, imaging findings, and biomarkers. The present study investigated the prevalence of anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibodies in anti-AQP4 seronegative Egyptian patients initially diagnosed with NMOSD and the link between their presence and clinical characteristics and disease-induced disability to gain insights into MOGAD. This pilot cross-sectional study included 40 anti-AQP4 antibody-negative patients initially diagnosed with NMOSD, six children and 34 adults. They were screened for anti-MOG antibodies by the indirect immunofluorescence cell-based assay. Of all included patients, only 7.5% (n = 3) were positive for anti-MOG antibodies and had significantly higher disability scores than seronegative patients (p = 0.021). The presence of anti-MOG antibodies was not significantly associated with age (p = 0.696), gender (p = 0.232), type of relapse (p = 0.488), number of attacks (p = 0.968), family history of consanguinity (p = 0.211), family history of autoimmune disease (p = 0.608), nor with smoking (p = 0.608). Detecting anti-MOG antibodies in anti-AQP4-negative NMOSD patients is essential for accurate diagnosis and personalized treatment, as MOGAD is now recognized as a separate clinical entity.

Neurofilament Light Chain as a Discriminator of Disease Activity Status in MOG Antibody-Associated Disease.

In patients with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), acute disease activity is generally identified through medical history, neurologic examination, and imaging. However, these may be insufficient for detecting disease activity in specific conditions. This study aimed to investigate the dynamics of serum neurofilament light chain (sNfL) and serum glial fibrillary acidic protein (sGFAP) after clinical attacks and to assess their utility in discriminating attacks from remission in patients with MOGAD. We conducted a multicenter, retrospective, longitudinal study including 239 sera from 62 MOGAD patients assessed from 1995 to 2023 in a discovery and validation setup. Sera were measured for sNfL and sGFAP with a single-molecule array assay and for MOG-IgG with a live cell-based assay. sNfL and sGFAP Z scores and percentiles adjusted for age, body mass index, and sex (sGFAP) were calculated from a healthy control normative database. Mixed-effects regression models were used to characterize biomarkers' dynamics and to investigate associations between serum biomarkers, clinical variables, and disease activity status. Among the 62 study participants, 29 (46.8%) were female, with a median age at baseline of 40.0 years (interquartile range [IQR] 29.5-49.8) and a median duration of follow-up of 20.0 months (IQR 3.0-62.8). sNfL and sGFAP Z scores were nonlinearly associated with time from attack onset (p < 0.001 and = 0.002, respectively). During attacks, both biomarkers presented higher median values (sNfL Z score 2.9 [IQR 1.4-3.5], 99.8th; sGFAP Z score 0.4 [IQR -0.5 to 1.5], 65.5th) compared with remission (sNfL Z score 0.9 [IQR -0.1 to 1.6], 81.6th, p < 0.001; sGFAP Z score -0.2 [IQR -0.8 to 0.5], 42.1th; p < 0.001) across all clinical phenotypes. sNfL values consistently discriminated disease activity status in the discovery and validation cohorts, showing a 3.5-fold increase in the odds of attacks per Z score unit (odds ratio 3.5, 95% confidence interval 2.3-5.1; p < 0.001). Logistic models incorporating sNfL Z scores demonstrated favorable performance in discriminating disease activity status across both cohorts. sNfL Z scores may serve as a biomarker for monitoring disease activity in MOGAD.

Clinical Analysis of MOG Antibody-Associated Disease Overlapped With Anti-NMDA Receptor Encephalitis: A Long-Term Retrospective Study.

To summarise the clinical characteristics, radiological features, treatments and prognosis of patients with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) overlapped with NMDA receptor (NMDAR) encephalitis. We retrospectively analysed patients who exhibited dual positivity for MOG antibodies and NMDAR antibodies in serum/CSF from Jan 2018 to Jun 2023. Ten patients with MOGAD and NMDAR encephalitis were enrolled. The median age of initial attacks was 23 (range: 10-43) years old. Common symptoms were cortical encephalopathies (8/10), focal neurological deficits (4/10), as well as other presentations including headache, fever, optic neuritis and transverse myelitis. CSF pleocytosis was general (9/10, median 63.9 cells/μl). Lesions on brain MRI included brainstem (37.5%), cerebral cortex (33.3%), basal ganglia (25.0%) and hippocampus (20.8%). The average follow-up duration was 25.4 months. 10/10 patients developed more than one relapse attacks, with MOG positivity before (10%), simultaneous (40%) or after anti-NMDAR encephalitis (50%). Most patients (7/10) had good response to first-line therapy but experienced next relapse with an average interval of 6.7 (range: 2-14) months. We conducted initial analysis of lymphocyte subsets in these patients, which revealed that CD3+ and CD4 + T cells increased after immunosuppressants medication (p < 0.01 and p < 0.05, respectively). We concluded that MOGAD overlapping with NMDAR encephalitis presents a distinct clinical phenotype which differs from either MOGAD or NMDAR encephalitis. Brainstem in combination with cortical lesions might be warning signs for this overlapping syndrome. Due to the high recurrent rates, we recommend early diagnosis and timely treatment with efficient immunosuppressants at onset.

Complement Activation Profiles Predict Clinical Outcomes in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

The role of the complement system in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is not completely understood, and studies exploring its potential utility for diagnosis and prognosis are lacking. We aimed to investigate the value of complement factors (CFs) as diagnostic and prognostic biomarkers in patients with MOGAD. Multicentric retrospective cohort study including patients with MOGAD, multiple sclerosis (MS) and aquaporin-4 seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD) with available paired serum and CSF samples. A panel of CFs were measured by multiplex ELISA, and the levels were compared between the 3 conditions. Univariable and multivariable analyses were performed to evaluate the association between levels of CFs and relapse and disability outcomes in MOGAD patients. Ninety-four patients (MOGAD, n = 60; MS, n = 18; AQP4-NMOSD, n = 16) were included. Mean (SD) age at sampling was 39.4 (16.7), 40.7 (7.0), and 43.3 (21.0), respectively. Female were predominant, especially in AQP4-NMOSD (88%). Combination of the serum levels of C3a, C4a, and C3a/C3 ratio showed excellent potential to discriminate MOGAD from patients with MS (area under the curve [AUC] [95% CI] 0.95 [0.90-0.99]) and from AQP4-NMOSD (AUC 0.88 [0.76-1.00]). In patients with MOGAD, CSF levels of CFs of the classical/lectin pathway influenced relapse-related outcomes, and lower C4 levels were associated with higher number of relapses during follow-up (incidence rate ratio [95% CI] 0.88 [0.78-0.99]; p = 0.04 in multivariable analysis), and a high C4a/C4 ratio was associated with increased risk of second relapse during the first year (hazard ratio [95% CI] 3.68 [1.26-10.78]; p = 0.02 in multivariable analysis). Time to second relapse was shorter in patients with MOGAD with a high CSF C4a/C4 ratio (log-rank p = 0.01). CSF levels of the membrane attack complex SC5b9 influenced disability-related outcomes, and baseline CSF SC5b9 levels were higher in patients who reached the final Expanded Disability Status Scale (EDSS) ≥ 3.0 (p = 0.002), and elevated SC5b9 levels were associated with increased risk of reaching EDSS ≥ 3.0 (odds ratio [95% CI] 1.79 [1.16-3.67]; p = 0.04 in multivariable analyses). Our results suggest that serum and CSF levels of CFs have diagnostic and prognostic value respectively in patients with MOGAD. These findings support the use of complement inhibitors as a therapeutic approach in these patients.

Pseudotumoural optic neuritis in myelin oligodendrocyte glycoprotein antibody-associated disease in children: Pseudotumoural optic neuritis in MOG antibody-associated disease.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a neuro-inflammatory condition affecting adults and children. The presentations vary and include acute disseminated encephalomyelitis, optic neuritis or transverse myelitis. Optic neuritis associated with anti-MOG antibodies is typically bilateral, anterior and initially severe but usually resolves quickly and completely. Here, we describe 3 children with pseudotumoural optic neuritis associated with anti-MOG antibodies and their outcome. Recognizing these unusual presentations may reduce unnecessary work-ups and improve functional prognosis by implementing rapid and adequate treatment.

Epstein-Barr virus infection in patients with MOGAD

Background: Epstein-Barr virus (EBV) infection increases the risk of having multiple sclerosis (MS). Data on adults with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are lacking. Objective: To compare EBV serological status in MOGAD versus MS. Methods: We measured antibodies to Epstein-Barr nuclear antigen (EBNA-1) and viral capsid antigen (VCA) antigens in 129 patients (MS = 74, MOGAD = 55) by chemiluminescence immunoassays. Results: VCA-IgG were detected in 97.3% of MS and 96.4% of MOGAD cases, while EBNA-1-IgG in 97.3% of MS and 80% of MOGAD (p = 0.001). EBNA-1 (p < 0.001) and VCA (p = 0.03) antibodies levels were higher in MS patients. Conclusion: EBV antibodies are higher in MS versus MOGAD, suggesting a possible different role of EBV in the pathogenesis of the two conditions.

Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children.

To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children's Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. The median age at onset was 7 years (range 2-16 years). The median number of relapses was 2 (range 1-8), and patients were followed up for a median of 65 months (range 5-214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as a Diffuse Pachymeningitis

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as a Diffuse Pachymeningitis

Real-world multicentre cohort study on choices and effectiveness of immunotherapies in NMOSD and MOGAD

BackgroundRecurrent attacks in neuromyelitis optica spectrum disorders (NMOSDs) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can lead to severe disability. We aimed to analyse the real-world use of immunotherapies in...