Late-onset myelin oligodendrocyte glycoprotein antibody-associated disease: an underrecognized entity in clinical practice; what does its course in later life teach us? A case report and narrative review

MOG antibody-associated disease after vaccination with ChAdOx1 nCoV-19

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a demyelinating disease of the central nervous system (CNS) that manifests as optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, and cortical encephalitis. Some patients with MOGAD present with tumor-like brain lesions. However, hydrocephalus as an initial presentation is rare. We present the case of a 23-year-old Japanese man with an acute onset of headache, nausea, and diplopia, who was initially suspected of having a germinoma but was later diagnosed with MOGAD. Brain magnetic resonance imaging (MRI) revealed a tumor-like lesion with hyperintensity on fluid-attenuated inversion recovery (FLAIR) imaging and contrast enhancement on T1-weighted postcontrast images, extending from the midbrain to the thalamus with obstructive hydrocephalus. Neuroendoscopic third ventriculostomy and brain biopsy were performed. Histopathological analyses revealed demyelination, perivascular lymphocytic infiltration, and MOG loss. MOG antibody tests were positive, confirming MOGAD. The patient was treated with pulse steroid therapy (methylprednisolone 1,000 mg/day) and seven sessions of plasmapheresis, resulting in significant neurological improvement. He was discharged approximately two months after symptom onset, and at the six-month follow-up from discharge, he remained relapse-free with only mild diplopia. In this case, early diagnosis via pathological brain analysis and anti-MOG antibody testing allowed for timely treatment. We emphasize the importance of including MOGAD in the diagnostic workup of tumor-mimicking CNS lesions that can cause hydrocephalus.

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.

Case presentation: A 40-year-old black woman was admitted to our emergency department due to a 30-day history of progressive headache, unresponsive to analgesics, associated with fever, bilateral visual deficit, bladder dysfunction, and lower limb paresthesia. She deteriorated with tetraparesis and encephalopathy, despite treatment with acyclovir and antibiotics. No previous infections, vaccinations, or neurologic symptoms were reported. Neurological examination revealed somnolence, mental confusion, and flaccid bilateral hemiparesis, ataxia, and light-near dissociation. The patient evolved with coma and required ventilatory support. Brain magnetic resonance imaging (MRI) showed multiple T2/FLAIR-hyperintense tumefactive lesions affecting the supratentorial white matter, cerebral cortex, basal ganglia, brain pedunculi, the whole brainstem, optic nerves, optic chiasma, and optic tracts, with gadolinium enhancement. Cervical MRI showed a mild non-tumefactive lesion in C2. The cerebrospinal fluid analysis revealed increased protein with lymphocytic pleocytosis and normal glucose levels, negative bacterioscopy and cultures. Oligoclonal bands were negative. No infectious agents were detected. Electroencephalogram did not reveal epileptic discharges. A comprehensive molecular panel for infectious meningitis and encephalitis came out negative. Of all rheumatological markers, only rheumatoid factor was positive. The whole-body 18F-FDG PET/CT scan showed no hypermetabolic lesions. Brain biopsy revealed an active chronic inflammatory process with necrosis, cellular debris, and secondary demyelination (predominantly perivascular lymphomononuclear inflammatory infiltrate). A Cell Based Assay (CBA) for anti-MOG was positive. Anti-aquaporin-4 antibody was negative. Acyclovir and antibiotics were discontinued. Plasma exchange and high dose methylprednisolone were initiated. The patient had a dramatic response and close to full recovery from neurological deficits. Three-month follow-up showed regression of MRI lesions. Discussion: MOGAD is a recently identified acquired autoimmune central nervous system demyelinating disease. It is typically associated with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON) or transverse myelitis, and is less commonly associated with cerebral cortical encephalitis, brainstem or cerebellar presentations. The MOGAD spectrum is broad and varies according to age, with ADEM being more common in children, and recurrent ON and longitudinally extensive transverse myelitis (LETM) being more common in adults. MOGAD attacks needing ventilatory support are rare. Most attacks respond well to treatment. We report a case of MOGAD with ADEM phenotype in an adult patient, evolving into coma, with need of ventilatory support, an uncommon, life-threatening, complication. Final comments: MOGAD should be considered as a cause of coma in adult patients with inflammatory diseases of the central nervous system suggestive of ADEM.

Aim/backgroundThe concept of neuromyelitis optica spectrum disorder (NMOSD) is changing, with a disease spectrum emerging that includes aquaporin 4 (AQP4) IgG-seropositive NMOSD, myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), and double-seronegative NMOSD. The past years have seen important advances in understanding rare demyelinating central nervous system (CNS) disorders associated with AQP4-IgG and MOG-IgG antibodies. Most of the recent literature has focused on the identification of clinical and magnetic resonance imaging (MRI) features that help distinguish these diseases from each other, simultaneously highlighting major diagnostic pitfalls that may lead to misdiagnosis. The present study aims to understand the epidemiology and disease characteristics of NMOSD and MOGAD in our population and compare them with previously published reports.Materials and methodsThis was a prospective, single-center, comparative, observational study conducted over 18 months. Thirty patients were recruited and categorized into two groups: NMOSD and MOGAD. Each group consisted of 15 patients. Data regarding neurological assessment, neuroimaging, treatment, and outcome were collected. These patients were followed at one, three, six, and 12 months for treatment response, residual disability, and relapse. Disease severity and disability were assessed by using the Expanded Disability Status Scale (EDSS) and modified Rankin scale (mRS).ResultsThe average age at presentation of the NMOSD group of patients was 34.67 ± 15.66 years, which was significantly higher compared to the 26 ± 5.74 years seen for the MOGAD group (p < 0.0001). The MOGAD group of patients had a significantly higher proportion of men compared to the NMOSD group (66.67% in MOGAD versus 0% in NMOSD). Optic neuritis was seen in a significantly higher proportion of MOGAD patients compared to the NMOSD group of patients (p = 0.0061). Bilateral optic neuritis was more common in the MOGAD group (26.67% vs. 6.67% in the NMOSD group). Isolated myelitis was higher in the NMOSD group. A higher proportion of patients in the NMOSD group received steroids along with rituximab (26.67%) compared to the MOGAD subgroup of patients. In terms of the rescue treatment, intravenous immunoglobulin (IVIG) or plasma exchange (PLEX) therapy was required more in the NMOSD group than the MOGAD group. The EDSS and mRS scores of both groups were comparable at baseline. However, on follow-up, the EDSS and mRS levels were significantly lower for the MOGAD group compared to the NMOSD group (p < 0.05). The overall relapse rate was 33.33% in the NMOSD group compared to 20% in the MOGAD group at 12 months.ConclusionNMOSD and MOGAD are two distinct CNS demyelinating disorders having different demographics, clinical profiles, treatment responses, relapse rates, and short-term outcomes. MOGAD patients appear to have younger age at onset, male predominance, less severe clinical presentation, good response to first-line treatment, fewer relapses, and better one-year functional outcomes whereas NMOSD has female predominance, more severe clinical attacks of myelitis and optic neuritis, less response to first-line management of acute attack requiring rescue therapy more often, less response to conventional immunosuppressive treatment with more relapses requiring escalation of maintenance therapy with rituximab, and significant visual and locomotor residual disability at 12 months.

To review the demographics and clinical and paraclinical parameters of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated relapsing disease. In this UK-based, multicentre study, 31 children with MOG antibody-associated relapsing disease were studied retrospectively. Of the 31 children studied, 14 presented with acute disseminated encephalomyelitis (ADEM); they were younger (mean 4.1y) than the remainder (mean 8.5y) who presented with optic neuritis and/or transverse myelitis (p<0.001). Similarly, children who had an abnormal brain magnetic resonance imaging (MRI) at onset (n=20) were younger than patients with normal MRI at onset (p=0.001) or at follow-up (p<0.001). 'Leukodystrophy-like' MRI patterns of confluent largely symmetrical lesions was seen during the course of the disease in 7 out of 14 children with a diagnosis of ADEM, and was only seen in children younger than 7 years of age. Their disability after a 3-year follow-up was mild to moderate, and most patients continued to relapse, despite disease-modifying treatments. MOG antibody should be tested in children presenting with relapsing neurological disorders associated with confluent, bilateral white matter changes, and distinct enhancement pattern. Children with MOG antibody-associated disease present with age-related differences in phenotypes, with a severe leukoencephalopathy phenotype in the very young and normal intracranial MRI in the older children. This finding suggests a susceptibility of the very young and myelinating brain to MOG antibody-mediated mechanisms of damage. Myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination manifest with an age-related phenotype. Children with MOG antibody and 'leukodystrophy-like' imaging patterns tend to have poor response to second-line immunotherapy.

Myelin oligodendrocyte glycoprotein antibody‐associated disease (MOGAD) is an autoimmune demyelinating condition that can affect both the pediatric and adult populations. The initial phenotype for the pediatric group is different from the adult. Young children commonly present with acute disseminated encephalomyelitis followed by optic neuritis and/or transverse myelitis. Here we described the clinical presentation of a young girl diagnosed with MOGAD.A three-year-old girl presented with an acute onset of reduced vision in both eyes preceded by neurological symptoms two weeks prior. Bilateral vision was profoundly reduced, visual acuity (VA) with Cardiff at 50cm in both eyes was 6/96 with positive reverse afferent pupillary defect grade 3 in the left eye. Examination showed bilateral grade one optic disc swelling with upper motor neuron signs. Magnetic Resonance Imaging (MRI) Brain findings were consistent with bilateral optic neuritis with multiple areas of hyperintense lesions with area of enhancement. MRI Spine revealed no spine involvement. Serology analysis showed positive anti-MOG antibody. The patient was treated with high-dose intravenous corticosteroid. Her vision improved subsequently.There was limited knowledge on clinical phenotype and relapsing course in MOGAD, especially in the pediatric group. Knowing about variation in pediatric clinical presentation might guide ophthalmologists and pediatricians to reach an accurate diagnosis.

IntroductionAlthough acquired demyelinating syndromes (ADS) are rare in children, the incidence and prevalence of ADS vary internationally. As data on pediatric ADS in Saudi Arabia is limited, the aim of this study was to describe the clinical spectrum of pediatric ADS, its clinical characteristics, and management options at a tertiary hospital in Saudi Arabia.MethodsA retrospective observational study was conducted at King Abdulaziz Medical City (KAMC) and King Abdullah Specialized Children Hospital (KASCH) in Riyadh, Saudi Arabia between January 2016 and December 2022. All patients with ADS fulfilling criteria of each subtype (multiple sclerosis (MS), clinically isolated syndrome (CIS), acute disseminated encephalomyelitis (ADEM), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)) were included in this study.ResultsForty-five pediatric patients with ADS were analyzed, with the majority diagnosed with MS. The median age of onset was higher in the MS group compared to monophasic CIS and MOGAD, with statistically significant differences in age at onset between the MS group and both the CIS and MOGAD groups (p = 0.0002). Significant differences were also observed in the type of initial central nervous system (CNS) attack, with optic neuritis being more common in MS and transverse myelitis in CIS (p < 0.0001). Laboratory results revealed a higher incidence of cerebrospinal fluid (CSF) oligoclonal bands in patients with MS, which was statistically significant (p = 0.04), and MOG antibodies were found in all patients with MOGAD. Intravenous pulse steroids were administered in most patients, while disease-modifying drugs (DMTs) were employed most frequently in patients with MS. The Expanded Disability Status Scale scores indicated little disability in most patients with MS and CIS, with more disability noted in a subgroup of ADEM. Overall, the study underscores the clinical heterogeneity of pediatric ADS and points out the statistically significant difference in age at onset, presenting features, and laboratory findings among ADS subtypes.ConclusionsThis study provides a thorough overview of pediatric ADS, including important distinctions between MS, ADEM, CIS, and MOGAD. Marked differences in age at onset, presentation, and imaging among these subtypes are informative for maximizing diagnosis and treatment. The key findings are the subsequent development of MS from CIS and MOGAD, varying patterns of first attack, and imaging characteristics like callososeptal interface lesions in MS and posterior fossa hyperintensities in MOGAD. All these indicate the need for individualized diagnostic and therapeutic approaches for improved outcomes.Supplementary InformationThe online version contains supplementary material available at 10.1007/s40120-025-00768-0.

Clinical and radiographic features of a cohort of adult and pediatric subjects in the Pacific Northwest with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)

BackgroundMyelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) comprises various age-dependent clinical phenotypes and may be monophasic, multiphasic, or chronic. Optic neuritis (ON) is a common manifestation and frequently appears in combination with other MOGAD phenotypes, particularly in young children. Despite permanent structural damage to the retinal nerve fiber layer (RNFL), children often experience complete visual recovery. AimsTo analyze the progression and impact of MOGAD on the visual system of pediatric patients independently of the history of ON. MethodsThis retrospective study included children who met specific criteria: myelin oligodendrocyte glycoprotein (MOG) immunoglobulin G (IgG) seropositivity, acute presentation of MOGAD, and written general consent. Main outcome measures were global peripapillary retinal nerve fiber layer (pRNFL) thickness, and near and distance visual acuity, analyzed using descriptive statistics. ResultsWe identified 10 patients with median age of 7.7 years at first event: 7 patients manifested with acute disseminated encephalomyelitis (ADEM) (with ON 5/7, ADEM only 1/7, with transverse myelitis (TM) 1/7), 2 with isolated ON, and 1 patient with neuromyelitis optica spectrum disorder (NMOSD)-like phenotype with ON. Among ON patients, 5/8 were affected bilaterally, with 3 initially diagnosed with unilateral ON but experiencing subsequent involvement of the fellow eye. None of the patients without previous ON showed a deterioration of visual acuity and, if evaluated, a reduction of the pRNFL. ConclusionMost pediatric MOGAD-ON patients in our cohort presented with acute vison loss and optic disc edema. All patients achieved complete visual recovery, independent of number of relapses or initial visual loss. The pRNFL thickness decreased for several months and stabilized at reduced levels after 12 months in the absence of further relapses. MOGAD may not have subclinical/’silent’ effects on the visual system, as visual acuity and pRNFL were not affected in patients without ON.

Background and Objective: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a significant component of demyelinating diseases in pediatric populations. Recently, diagnostic criteria for MOGAD were established. This study aims to evaluate and compare the diagnostic efficacy of the fixed-cell-based assay (Fixed-CBA) and the live cell-based assay (Live-CBA) in patients who meet the 2023 clinical diagnostic criteria for MOGAD. Methods: This retrospective study included patients suspected of having MOGAD who were enrolled between June 2023 and June 2024. Patients were selected based on the “core clinical demyelinating events” outlined in the 2023 proposed criteria of the International MOGAD Panel. Patients with multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) with aquaporin-4 antibody-positive (AQP4-Abs-positive), and non-central nervous system (non-CNS) inflammatory diseases were chosen as controls. Serum samples were simultaneously tested for MOG-Abs using Fixed-CBA and Live-CBA. Results: A total of 86 patients were enrolled in the study: 52 in the suspected MOGAD group and 34 in the control group. Out of these patients studied, 16 presented with optic neuritis (ON), 5 with myelitis, 8 with acute disseminated encephalomyelitis (ADEM), and 7 with cortical encephalitis. Sixteen patients could not be classified by clinical phenotype. The highest MOG-Ab positivity rate was among patients with cortical encephalitis [85.7% (Live-CBA)/71.4% (Fixed-CBA)]. Both assays identified 22 positive samples, with Fixed-CBA and Live-CBA sensitivities at 44.2% and 55.8%, respectively, and a specificity of 97%. Of the patients suspected of having MOGAD, 19 cases were confirmed using the Fixed-CBA, while 28 cases were confirmed using the Live-CBA. This resulted in an upgrade in diagnostic classification for nine cases. This led to a diagnostic reclassification in nine cases. Conclusions: Both the Fixed-CBA and Live-CBA were associated with higher sensitivity for patients selected based on the 2023 MOGAD clinical diagnostic criteria. The Live-CBA exhibited an 11.6% increase in sensitivity, contributing to a 17.3% (9/52) enhancement in clinical diagnostic accuracy.

Background and ObjectivesAcute inflammatory CNS diseases include neuromyelitis optica spectrum disorders (NMOSDs) and myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD). Both MOGAD and acute disseminated encephalomyelitis (ADEM) have been reported after vaccination. Consequently, the mass SARS-CoV-2 vaccination program could result in increased rates of these conditions. We described the features of patients presenting with new acute CNS demyelination resembling NMOSDs or MOGAD within 8 weeks of SARS-CoV-2 vaccination.MethodsThe study included a prospective case series of patients referred to highly specialized NMOSD services in the UK from the introduction of SARS-CoV-2 vaccination program up to May 2022. Twenty-five patients presented with new optic neuritis (ON) and/or transverse myelitis (TM) ± other CNS inflammation within 8 weeks of vaccination with either AstraZeneca (ChAdOx1S) or Pfizer (BNT162b2) vaccines. Their clinical records and paraclinical investigations including MRI scans were reviewed. Serologic testing for antibodies to myelin oligodendrocyte glycoprotein (MOG) and aquaporin 4 (AQP4) was performed using live cell–based assays. Patients' outcomes were graded good, moderate, or poor based on the last clinical assessment.ResultsOf 25 patients identified (median age 38 years, 14 female), 12 (48%) had MOG antibodies (MOGIgG+), 2 (8%) had aquaporin 4 antibodies (AQP4IgG+), and 11 (44%) had neither. Twelve of 14 (86%) antibody-positive patients received the ChAdOx1S vaccine. MOGIgG+ patients presented most commonly with TM (10/12, 83%), frequently in combination with ADEM-like brain/brainstem lesions (6/12, 50%). Transverse myelitis was longitudinally extensive in 7 of the 10 patients. A peak in new MOGAD cases in Spring 2021 was attributable to postvaccine cases. Both AQP4IgG+ patients presented with brain lesions and TM. Four of 6 (67%) seronegative ChAdOx1S recipients experienced longitudinally extensive TM (LETM) compared with 1 of 5 (20%) of the BNT162b2 group, and facial nerve inflammation was reported only in ChAdOx1S recipients (2/5, 40%). Guillain-Barre syndrome was confirmed in 1 seronegative ChAdOx1S recipient and suspected in another.DiscussionChAdOx1S was associated with 12/14 antibody-positive cases, the majority MOGAD. MOGAD patients presented atypically, only 2 with isolated ON (1 after BNT162b2 vaccine) but with frequent ADEM-like brain lesions and LETM. Within the seronegative group, phenotypic differences were observed between ChAdOx1S and BNT162b2 recipients. These observations might support a causative role of the ChAdOx1S vaccine in inflammatory CNS disease and particularly MOGAD. Further study of this cohort could provide insights into vaccine-associated immunopathology.

To summarize the clinical features and neuroimaging findings of the patients with acute disseminated encephalomyelitis (ADEM) involved in corpus callosum (CC) so as to distinguish it from other diseases. A total of 12 ADEM patients with the involvement of CC during the period of 2010-2012 were recruited. There were 9 males and 3 females with a mean age of 31±14 years (range: 10-54). Their clinical and neuroimaging features were retrospectively reviewed and all data analyzed by SPSS 18.0. (1) All of them had an acute or subacute onset. Two patients had a history of vaccination and 5 suffered upper respiratory tract infection or diarrhea. (2) The presenting symptoms included fever (n=5), headache (n=4), unsteady gait (n=2), urinary retention (n=1), indifference (n=1) and delirium (n=1). (3) The main clinical symptoms included memory loss (n=9), delirium (n=5), somnolence (n=4), urinary retention (n=9), paraplegia (n=4) and unsteady gait (n=5). (4) The examinations of cerebrospinal fluid (CSF) revealed increased intracranial pressure (n=4), leucocytosis (n=3) and increased protein (n=7) of 7 cases. All oligoclonal bands were negative. (5) The lesions were involved in bilateral CCs in 12 patients. Among them, splenium was the most commonly affected (n=9), secondly stem (n=5) and lastly genu (n=4). For 6 patients, the intracranial lesions were all in their CCs. And among them, 2 cases were involved in spinal cord. Except for CC, there were other focal lesions in brain stem and cerebellum (n=4) and spinal cord (n=6). (6) On magnetic resonance imaging (MRI), all cases showed long T2 signal intensity with blurred images. And among them, 2 cases' lesions in brain were discerned only by diffuse weighing imaging (DWI) or T2 fast fluid-attenuated inversion recovery (T2FLAIR) instead of T2-weighted. The lesions of CCs showed on gadolinium-enhanced MRI were significantly enhanced and the shapes were sheet-like (4/6). Spinal cord lesions was found in 6 cases and most spinal cord lesions were discontinuous. And the number of spinal cord segments with lesions was from 4 to 8. The shapes of lesions of spinal cord showed on enhanced MRI were like thin line. (7) Most of them were misdiagnosed as viral encephalitis (n=5), tuberculous meningoencephalitis (n=1) and brain neoplasms (n=2). And another case was admitted into urology surgery ward due to urinary retention. There are three key points about the characteristics of the ADEM patients with CC lesions: (1) They may have an adult male preponderance. The distinctive symptoms include fever, headache, delirium, somnolence, memory loss, unsteady gait and urination disorders, etc.. (2) The number of lesions on brain MRI can be multiple or single, especially the lesions of CC (mostly in splenium). On MRI, all cases showed long T2 signal intensity with blurred images so that DWI and T2 FLAIR may have a higher efficiency of detecting the lesions. In particular, multiple lesions may be all enhanced or not enhanced at equal pace on enhanced MRI. (3) In ADEM patients with CC lesions, many indices of CSF chemical examination, such as increased intracranial pressure, leucocytosis, increased protein, low sugar and low chloride, indicate the presence of intracranial infective diseases. Therefore they are most likely to be misdiagnosed as viral encephalitis or tuberculous meningoencephalitis. However, CC is not the predilection site for viral encephalitis since CC belongs to white matter but not gray matter. So ADEM should be a more appropriate diagnosis for these cases.

Myelin oligodendrocyte glycoprotein antibody-associated disease following DTaP vaccination: A case report

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated diseases (MOGAD), which has been recognized as a distinct entity in patients with neuromyelitis optica spectrum disorders, often presents with acute disseminated encephalomyelitis (ADEM) symptoms in pediatric patients. Appropriate treatment based on accurate diagnosis is challenging in relapsing pediatric patients with MOGAD. An 11-year-old girl experienced relapses four times, exhibiting brainstem symptoms, an ADEM episode, seizures, and optic neuritis (ON). She was initially diagnosed with multiple sclerosis and received interferon beta-1a therapy with a mild effect on relapse suppression. She was then transferred from the pediatric department to the department of neurology of our hospital. Two months before her referral visit, she experienced left optic neuritis, and her annualized relapse rate reached 0.6. She desired to switch from the injectable treatment to oral dimethyl fumarate (DMF) administration. At that time, she was found to be seropositive for MOG antibody, but after that had no relapses for more than five years. Moreover, her seropositivity for serum MOG-antibody turned out to be seronegative. DMF showed long-term effects on suppressing relapses in a pediatric patient with MOGAD, revealing its potential as a treatment option for such patients.