antibodies

2026-07-01 | Delayed Myelin Oligodendrocyte Glycoprotein Antibody-Associated Demyelination Five Years After an Acute Disseminated Encephalomyelitis-Like Episode in an Adolescent: A Case Report

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an immune-mediated inflammatory demyelinating disorder of the central nervous system with a broad pediatric clinical spectrum. Acute disseminated encephalomyelitis (ADEM)-like presentations are more frequent in younger children, whereas older children and adolescents may present with optic neuritis, transverse myelitis, or multifocal central nervous system involvement. We report the case of a 14-year-old male adolescent who developed a severe MOG-IgG-positive demyelinating event five years after an initial ADEM-like episode. At nine years of age, he presented with back pain, gait disturbance, urinary incontinence, mild drowsiness, fever, cerebrospinal fluid pleocytosis, subcortical and medullary brain lesions, and longitudinally extensive myelitis. MOG-IgG testing was not available at that time, and the episode was diagnosed as ADEM based on the overall clinico-radiological presentation, with complete recovery after corticosteroid therapy. Five years later, he presented with fever, acute quadriparesis predominating in the lower limbs, pyramidal signs, and sphincter dysfunction. Brain MRI showed multiple ill-defined subcortical T2-weighted/fluid-attenuated inversion recovery (T2/FLAIR) hyperintense lesions, and spinal MRI demonstrated longitudinally extensive cervical myelitis from the cervicomedullary junction to C6. Cerebrospinal fluid analysis showed marked pleocytosis, elevated protein, absence of oligoclonal bands, and negative infectious studies. Serum MOG-IgG was positive at a titer of 1:100 using a fixed cell-based assay, while AQP4-IgG was negative. He received intravenous methylprednisolone followed by intravenous immunoglobulin because of insufficient initial improvement, with complete clinical recovery at one month and marked radiological regression at six months. This case emphasizes that a confirmed MOG-IgG-positive demyelinating event may occur after a prolonged relapse-free interval following an earlier ADEM-like demyelinating episode, while also underscoring that the first episode should be interpreted cautiously because MOG-IgG testing was unavailable. Long-term follow-up remains essential because short-term clinical and radiological improvement does not establish durable disease control.

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2026-06-20 | Suspected Acute Disseminated Encephalomyelitis Presenting With Persistent Vegetative State and Full Neurological Recovery in an Adult: A Case Study

Acute disseminated encephalomyelitis (ADEM) is an autoimmune inflammatory disease of the central nervous system that typically presents with headache, fever, altered consciousness, and seizures. Diagnosis is made clinically and radiologically, supported by the exclusion of mimics. Delayed treatment can lead to severe neurological impairment, including a persistent vegetative state. A 69-year-old Asian female with rheumatic heart disease and prior transverse myelitis presented with hypoxic respiratory failure. On hospital day (HD) 4, she developed a rapid decline in mental status, progressing to a vegetative state with electroclinical seizures. Brain MRI showed diffuse, symmetric T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities involving subcortical white matter, which is an atypical finding for classic ADEM. Extensive infectious and autoimmune testing was unrevealing. Lumbar puncture on HD 11 (prior to steroids) showed no pleocytosis. She remained vegetative for 39 days without response to high-dose corticosteroids. Following a five-day course of intravenous immunoglobulin (IVIG), she showed marked neurological recovery. At discharge, she returned to her baseline functional status. This case illustrates the diagnostic challenges of suspected ADEM in an older adult with atypical symmetric imaging, prolonged vegetative state, and delayed response to immunotherapy. The case adds to the limited literature on extended encephalopathy in suspected ADEM and underscores the role of IVIG when steroid response is unclear.

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2026-04-21 | Patient profiles and disease characteristics of the IMSRN cohort: a multicentre study (2021-2025).

Data of Central Nervous System (CNS) demyelinating disorders from India has been published from limited centres. The Indian Multiple Sclerosis And Allied Demyelinating Disorders Registry and Research Network (IMSRN) is a multicentric database for multiple sclerosis and allied demyelinating disorders in the Indian subcontinent. This study aimed to describe the demographic, clinical, laboratory, treatment, and follow-up details of patients in the IMSRN and summarise the distribution of major disease phenotypes in a real-world cohort. This was a prospective, observational, registry-based analysis of patients with CNS demyelinating disorders enrolled in the IMSRN between 16 August 2021 and 25 October 2025. Data were collected in a predefined case record form at recruitment and periodically every six months on a secure database. We performed descriptive and comparative analyses, including temporal trends, treatment patterns, and longitudinal follow-up. As of 25th October, 2025, 4976 patients have been recruited including radiologically isolated syndrome (RIS), 15 (0.30%); clinically isolated syndrome (CIS), 200 (4.02%); multiple sclerosis (MS), 2479 (49.82%); neuromyelitis optica spectrum disorder (NMOSD), 793 (15.94%); myelin oligodendrocyte antibody associated disease (MOGAD), 698 (14.03%); acute disseminated encephalomyelitis (ADEM), 76 (1.53%); chronic relapsing inflammatory optic neuritis (CRION), 34 (0.68%); chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), three (0.06%), and others, 562 (11.29%). The mean (SD) age of the entire cohort at enrolment is 34.62 (12.16) years, at disease onset was 29.12 (11.80) years and median (IQR) disease duration at recruitment is 2.79 (0.51-7.22) years; longest for MS. There were 65.33% females and 34.67% males, with a female-to-male ratio of 1.9:1; highest in NMOSD (3.58:1). Most patients are from urban areas and educated. Optic nerve and spinal cord involvement were the dominant areas at first presentation. The median (IQR) number of relapses per patient over follow-up was 2 (1-3) highest in the NMOSD population; dominated by optic nerve and spinal cord symptoms. The median (IQR) EDSS was 2 (1-4.5). Rituximab was the commonest disease modifying therapy (DMT) used. Longitudinal trends reflect improvement in time-to-diagnosis and significant shift in the pattern of DMT use in MS towards oral DMTs and B-cell inhibitors. The paper describes a cohort of patients with MS and allied disorders. The key disease characteristics of the MS population seem similar to those reported in international MS registries. The data adds to the existing literature to ascertain disease patterns, response to treatment, and long-term outcome. The IMSRN registry is funded by the Indian Council of Medical Research (ICMR) vide Grant number 5/4-5/192/NeuroTF/2019-NCD-1.

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2026-04-20 | Adult Onset of Acute Disseminated Encephalomyelitis (ADEM) With Associated Myelin Oligodendrocyte Glycoprotein (MOG) Antibody.

ADEM is an inflammatory and demyelinating autoimmune disorder of the central nervous system (CNS). One recognized cause of ADEM is myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). MOGAD affects approximately one in 10,000 people worldwide and is more prevalent in children. Patients typically present with optic neuritis, ADEM, and transverse myelitis. Many patients will experience relapses of demyelinating attacks, and thus, both acute and maintenance therapies are typically used to manage the disease. This case considers an adult female who presented with neurological deficits as a result of MOG-associated ADEM. She had minimal response to traditional therapies but responded well to treatment with IV immunoglobulin (IVIG). This case report explores her clinical course as well as unique clinical significance and conclusions due to the nontraditional use of IVIG.

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2026-03-28 | Severe retinal vasculopathy associated with myelin oligodendrocyte glycoprotein antibody optic neuritis in a toddler.

We report the case of a 22-month-old boy with retinal vascular disease caused by optic neuritis due to myelin oligodendrocyte glycoprotein antibody disease. He presented with acute disseminated encephalomyelitis without optic nerve involvement. Despite treatment, he developed optic neuritis and vision loss within days. In addition to the usual findings of optic neuritis, he had evidence of severe retinal venous stasis including intraretinal, subretinal, preretinal, and choroidal hemorrhages that were rapidly progressive. The retinal perfusion did not recover, leading to neovascularization. This is a rare case of MOG antibody-associated disease with retinal venous stasis in which long-term follow-up revealed permanent vision loss from widespread retinal ischemia and optic atrophy. We propose that hemorrhages beyond the peripapillary region and macula can be a sign of retinal venous insufficiency related to vascular obstruction at the optic nerve head. Patients exhibiting this sign should undergo prompt antibody testing when it is associated with optic neuritis. Careful monitoring and treatment of retinal ischemia and neovascularization in severe cases is warranted to prevent late complications.

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cell therapies

2026-05-01 | Combined central and peripheral nervous system involvement in acute disseminated encephalomyelitis in a young male: A case report.

Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system, typically presenting after infection or vaccination. Rarely, ADEM may involve the peripheral nervous system, presenting with pure motor axonal neuropathy, which poses a diagnostic and therapeutic challenge. We report a 22-year-old Asian male with no prior medical history who initially experienced a mild upper respiratory infection followed by headache and a single generalized tonic-clonic seizure. He subsequently developed altered consciousness and flaccid paraplegia. Brain magnetic resonance imaging demonstrated asymmetric, poorly marginated supratentorial white-matter hyperintensities on T2-weighted and fluid-attenuated inversion recovery sequences. Cerebrospinal fluid analysis revealed mild lymphocytic pleocytosis and elevated protein, while autoimmune encephalitis panels and infectious studies were negative. Nerve conduction studies revealed predominantly motor axonal neuropathy affecting the lower limbs, with sensory sparing. The patient received high-dose intravenous methylprednisolone followed by therapeutic plasma exchange due to incomplete motor recovery. After 2 weeks, he regained the ability to stand and ambulate with support, and repeat magnetic resonance imaging at 3 months demonstrated complete resolution of the lesions. This case highlights a rare presentation of ADEM with peripheral nervous system involvement in a young adult, distinguished by predominant motor axonal neuropathy and a favorable response to plasma exchange. Recognition of peripheral involvement in ADEM is essential, particularly in younger patients with persistent weakness despite corticosteroid therapy, as timely diagnosis and intervention can significantly improve functional outcomes.

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2026-04-20 | Plasma Exchange as a Bridge to Recovery in Severe Pediatric Neurological Diseases in Pediatric Intensive Care.

Therapeutic plasma exchange (TPE) removes circulating pathogenic antibodies, immune complexes, cytokines, and complement components. While established in adult neurology, evidence on its safety and efficacy in pediatric neurocritical care remains limited. To evaluate the indications, clinical efficacy, and safety of TPE in children with acute or subacute immune-mediated neurological disorders admitted to the pediatric intensive care unit (PICU). A retrospective cohort of 24 pediatric patients (aged 1-18 years) treated with TPE between February 2023 and October 2025 was analyzed. Diagnoses included multiple sclerosis (n = 8), Guillain-Barré syndrome (n = 5), acute disseminated encephalomyelitis (n = 3), transverse myelitis (n = 3), autoimmune encephalitis (n = 3), optic neuritis (n = 1), and myasthenia gravis (n = 1). Patients received 3-7 TPE sessions. Clinical response was measured using the modified Rankin Scale (mRS) and GBS disability scores before and after treatment. A total of 130 sessions were performed. Clinical improvement occurred in 20 of 24 patients (83%)-moderate in 8 (33%) and mild in 12 (50%). Median mRS/GBS scores decreased from 4.0 to 3.0 (p = 0.001). Early initiation of TPE (≤ 7 days from symptom onset) was associated with a higher likelihood of neurological improvement in multivariable analysis. No life-threatening complications were observed. Transient, reversible events included hypocalcemia (2.3%), hypotension (1.5%), and urticaria (1.6%). TPE appears to be a safe and potentially beneficial intervention for severe or refractory pediatric neuroimmunological disorders. Earlier initiation of TPE was associated with improved neurological outcomes; however, these findings should be interpreted cautiously given the small sample size and heterogeneous disease groups. These findings support integrating TPE into multidisciplinary PICU management and add to the growing evidence supporting its use in pediatric neuroimmunology.

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2026-02-18 | Clinical response to therapeutic plasma exchange in patients with immune-mediated inflammatory diseases of the central nervous system.

Inflammatory immune-mediated diseases of the central nervous system, such as autoimmune encephalitis, acute disseminated encephalomyelitis or Guillain-Barré syndrome, pose diagnostic and therapeutic challenges in the pediatric population. Therapeutic plasma exchange has emerged as a useful option in these cases. This study evaluated the clinical response to therapeutic plasma exchange in children with these conditions. We conducted a retrospective cohort study at Fundación Hospital Pediátrico La Misericordia in Bogotá (2018-2022), including 50 patients with a confirmed diagnosis of antibody-mediated inflammatory disease of the central nervous system who were managed with therapeutic plasma exchange. We collected data on clinical, therapeutic and outcome variables and performed descriptive, survival, and Cox regression analyses. The mean age was 10 years, with a uniform sex distribution. The most frequent conditions were autoimmune encephalitis (36%) and acute disseminated encephalomyelitis (14%). At discharge, 56% of patients had persistent neurologic symptoms and 46% had recovered their prior functional status. The mortality was 6%. The use of antiepileptic drugs was significantly associated with a lower risk of relapse (hazard ratio, 0.081; P = .024) and steroid use with increased survival, particularly in patients with autoimmune encephalitis, acute disseminated encephalomyelitis and myasthenia gravis (P < .05). Therapeutic plasma exchange is a viable and safe treatment strategy for children with immune-mediated inflammatory diseases of the central nervous system. The concomitant use of antiepileptic drugs and steroids showed benefits in reducing relapse and mortality.

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2025-09-02 | Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Pathophysiology, Clinical Patterns, and Therapeutic Challenges of Intractable and Severe Forms

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is characterized by the predominance of optic neuritis, myelitis, acute disseminated encephalomyelitis (ADEM), and cortical encephalitis, and can be diagnosed by the presence of pathogenic immunoglobulin G (IgG) antibodies targeting the extracellular domain of MOG in the serum and cerebrospinal fluid (CSF). Initially considered a variant of multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD), it is now widely recognized as a separate entity, supported by converging evidence from serological, pathological, and clinical studies. Patients with MOGAD often exhibit better recovery from acute attacks; however, their clinical and pathological features vary based on the immunological role of MOG-IgG via antibody- or complement-mediated perivenous demyelinating pathology, in addition to MOG-specific cellular immunity, resulting in heterogeneous demyelinated lesions from vanishing benign forms to tissue necrosis, even though MOGAD is not a mild disease. The key is the immunological mechanism of devastating lesion coalescence and long-term degenerating mechanisms, which may still accrue, particularly in the relapsing, progressing, and aggressive clinical course of encephalomyelitis. The warning features of the severe clinical forms are: (1) fulminant acute multifocal lesions or multiphasic ADEM transitioning to diffuse (Schilder-type) or tumefactive lesions; (2) cortical or subcortical lesions related to brain atrophy and/or refractory epilepsy (Rasmussen-type); (3) longitudinally extended spinal cord lesions severely affected with residual symptoms. In addition, it is cautious for patients refractory to acute stage early 1st treatment including intravenous methylprednisolone treatment and apheresis with residual symptoms and relapse activity with immunoglobulin and other 2nd line treatments including B cell depletion therapy. Persistent MOG-IgG high titration, intrathecal production of MOG-IgG, and suggestive markers of higher disease activity, such as cerebrospinal fluid interleukin-6 and complement C5b-9, could be identified as promising markers of higher disease activity, worsening of disability, and poor prognosis, and used to identify signs of escalating treatment strategies. It is promising of currently ongoing investigational antibodies against anti-interleukin-6 receptor and the neonatal Fc receptor. Moreover, due to possible refractory issues such as the intrathecal production of autoantibody and the involvement of complement in the worsening of the lesion, further developments of other mechanisms of action such as chimeric antigen receptor T-cell (CAR-T) and anti-complement therapies are warranted in the future.

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2024-11-18 | Plasma Exchange an Effective Measure in Rabies Vaccine Induced Acute Disseminated Encephalomyelitis

We describe a case of a 57 year of patient who had a history of dog bite and then was subsequently vaccinated with anti-rabies vaccine of 2 doses.She developed lower limb weakness and areflexia and was intubated after going into respiratory distress.She was given IV immunoglobulin, methyl prednisolone and then underwent seven cycles after which she showed improvement.

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proteins

2026-05-15 | Myelin Oligodendrocyte Glycoprotein Antibody Associated Acute Disseminated Encephalomyelitis Post Varicella Infection in a Child: A Case Report

Myelin Oligodendrocyte Glycoprotein Antibody associated Disease (MOGAD), an autoimmune demyelinating disorder, often overlaps with features of Acute Disseminated Encephalomyelitis (ADEM), optic neuritis and transverse myelitis in paediatric population. Recent advances have identified Myelin Oligodendrocyte Glycoprotein (MOG) antibody as a marker in a subset of paediatric demyelinating diseases. MOGAD is increasingly being recognised as a distinct entity among paediatric demyelinating disorders. Identification of MOG antibodies is important as it has diagnostic, therapeutic and prognostic implications particularly in post-infectious demyelination. Herein, the authors report a case of 10-year-old male who presented with fever and vesicular lesions for 10 days, suggesting chickenpox infection (varicella), which was followed by paraparesis. Neurological examination suggested hypotonia of lower limbs and absent deep tendon reflexes with rest of central nervous examination within normal limits. Radiological and biochemical investigations (serum MOG positive) confirmed the diagnosis of ADEM likely to be post varicella infection. He was managed successfully with intravenous immunoglobulin, high dose steroids and is currently doing well on followup. This case highlights the importance of early detection, diagnosis and testing for anti-MOG in children presenting with ADEM like features. Early recognition of MOG antibody associated ADEM and timely initiation of immunotherapy are crucial for improving neurological outcomes in affected children. This case emphasises the need to consider MOGAD in children presenting with acute demyelination after varicella.

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2025-05-16 | The role of immunotherapy in a refractory case of acute hemorrhagic leukoencephalitis in a pediatric patient.

Acute hemorrhagic leukoencephalitis (AHLE) is a rare neurologic inflammatory disorder characterized by severe and rapidly progressive encephalopathy; typically considered a severe variant of acute demyelinating encephalomyelitis (ADEM). There is limited pediatric data on treatment modalities for refractory illness. We report here the case of an 8-year-old girl who presented with acute concern for encephalopathy, ataxia, and seizures in the context of preceding infectious symptoms and neuroimaging findings highly suspicious for ADEM. Treatment was promptly initiated with high-dose glucocorticoids followed by addition of intravenous immunoglobulin therapy. Despite ongoing treatment and permissive hypernatremia and hyperosmolar therapy to treat signs of increased intracranial pressure, she showed no clinical signs of improvement, so plasmapheresis was initiated. Follow-up magnetic resonance imaging (MRI) brain showed progression of lesions with more hemorrhagic conversion concerning for AHLE, so anakinra was started. With the combination of pheresis and anakinra, the patient began to show signs of neurologic recovery. Her course was complicated by delirium and physical deconditioning, requiring inpatient rehab, although her neurologic function showed daily improvement. Final MRI brain to date reflected these improvements. Our case highlights the importance of rapid escalation of care to improve mortality rates and reduce neurologic sequelae of AHLE. Future studies are needed to address the safety profile and efficacy of anakinra in refractory disease.

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2022-07-28 | Transforming growth factor-β1 protects against LPC-induced cognitive deficit by attenuating pyroptosis of microglia via NF-κB/ERK1/2 pathways

Demyelinating diseases in central nervous system (CNS) are a group of diseases characterized by myelin damage or myelin loss. Transforming growth factor beta1 (TGF-β1) is widely recognized as an anti-inflammatory cytokine, which can be produced by both glial and neuronal cells in CNS. However, the effects of TGF-β1 on demyelinating diseases and its underlying mechanisms have not been well investigated.A demyelinating mouse model using two-point injection of lysophosphatidylcholine (LPC) to the corpus callosum in vivo was established. Exogenous TGF-β1 was delivered to the lesion via brain stereotactic injection. LFB staining, immunofluorescence, and Western blot were applied to examine the severity of demyelination and pyroptosis process in microglia. Morris water maze test was used to assess the cognitive abilities of experimental mice. Furthermore, lipopolysaccharide (LPS) was applied to induce pyroptosis in primary cultured microglia in vitro, to explore potential molecular mechanism.The degree of demyelination in LPC-modeling mice was found improved with supplement of TGF-β1. Besides, TGF-β1 treatment evidently ameliorated the activated proinflammatory pyroptosis of microglia, with downregulated levels of the key pyroptosis effector Gasdermin D (GSDMD), inflammasomes, and cleaved-IL-1β, which effectively attenuated neuroinflammation in vivo. Evaluated by behavioral tests, the cognitive deficit in LPC-modeling mice was found mitigated with application of TGF-β1. Mechanistically, TGF-β1 could reverse pyroptosis-like morphology in LPS-stimulated primary cultured microglia observed by scanning electron microscopy, as well as decrease the protein levels of cleaved-GSDMD, inflammasomes, and cleaved-IL-1β. Activation of ERK1/2 and NF-κB pathways largely abolished the protective effects of TGF-β1, which indicated that TGF-β1 alleviated the pyroptosis possibly via regulating NF-κB/ERK1/2 signal pathways.Our studies demonstrated TGF-β1 notably relieved the demyelinating injury and cognitive disorder in LPC-modeling mice, by attenuating the inflammatory pyroptosis of microglia via ERK1/2 and NF-κB pathways. Targeting TGF-β1 activity might serve as a promising therapeutic strategy in demyelinating diseases.

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2018-08-18 | TIMP-1 Promotes Oligodendrocyte Differentiation Through Receptor-Mediated Signaling

The extracellular protein tissue inhibitor of metalloproteinase (TIMP)-1 is both a matrix metalloproteinase (MMP) inhibitor and a trophic factor. Mice lacking TIMP-1 exhibit delayed central nervous system myelination during postnatal development and impaired remyelination following immune-mediated injury in adulthood. We have previously determined that the trophic action of TIMP-1 on oligodendrocyte progenitor cells (OPCs) to mature into oligodendrocytes is independent of its MMP inhibitory function. However, the mechanism by which TIMP-1 promotes OPC differentiation is not known. To address this gap in our understanding, herein, we report that TIMP-1 signals via a CD63/β1-integrin receptor complex to activate Akt (protein kinase B) to promote β-catenin signaling in OPCs. The regulation of β-catenin by TIMP-1 to promote OPC differentiation was counteracted, but not abrogated, by canonical signaling evoked by Wnt7a. These data provide a previously uncharacterized trophic action of TIMP-1 to regulate oligodendrocyte maturation via a CD63/β1-integrin/Akt pathway mechanism. These findings contribute to our emerging understanding on the role of TIMP-1 as a growth factor expressed to promote CNS myelination during development and induced in the adult to promote myelin repair.

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2016-09-26 | The usefulness of immunotherapy in pediatric neurodegenerative disorders: A systematic review of literature data.

Immunotherapeutic strategies to treat neurodegenerative disorders have inspired the scientific community. The aim of our review is to address the translational aspects of neuroimmunology to describe the efficacy of immunotherapy in the treatment of pediatric neurodegenerative disorders. In the studies we analyzed IVIG were found to be efficient in the treatment of post-streptococcal neurodegenerative disorders, even if in PANDAS, plasma-exchange (PE) showed a higher efficiency. IVIG were also successfully used in ADEM and Guillan-Barré syndrome. In Sydenham Chorea the use of methylprednisolone was found in most cases as efficient as IVIG, while in Tourette's Syndrome, Colecoxib was successfully used in one patient. Pediatric Multiple Sclerosis seems to respond better to immunosuppressant agents (Mitoxantrone, Cyclophosphamide, Natalizumab), as well as Neuromyelitis optica (Rituximab, Mycofenolate). The importance of this review relies in the attempt to draw standardized guidelines for immunotherapy in pediatric neurodegeneratve disorders.

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small molecules

2026-08-04 | Treatment of acute demyelinating encephalomyelitis associated with recent rickettsial infection.

Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated demyelinating disorder of the central nervous system that typically follows viral or bacterial infections. There are only two known case reports that describe an association with ADEM post rickettsial infection. A female in her 80s presented with progressive neurological decline, including dysarthria, dysphagia and hemiparesis, following recent treatment in Hong Kong for rickettsial infection. Initial CT brain imaging showed a right subcortical infarct. She deteriorated with encephalopathy and respiratory failure, requiring intubation. MRI revealed extensive brain and spinal demyelination. Infectious, autoimmune and paraneoplastic investigations were negative, while serology revealed markedly elevated spotted fever group antibodies. Treatment with high-dose corticosteroids and plasma exchange therapy resulted in significant neurological recovery. She was discharged after rehabilitation with a good recovery at 70%-80% of pre-morbid function. This case supports a possible post-infectious immune-mediated mechanism linking rickettsia and ADEM and underscores the importance of early recognition and treatment of ADEM.

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2026-07-07 | Myelin oligodendrocyte glycoprotein antibody-related autoimmune encephalitis misdiagnosed as acute cerebral infarction: A case report.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory demyelinating autoimmune disorder of the central nervous system that exhibits a broad spectrum of known clinical phenotypes, including optic neuritis, myelitis, acute disseminated encephalomyelitis, encephalitis, meningoencephalitis, and brainstem encephalitis. However, new evidence indicates that a small subset of patients may present with cortical cerebral encephalitis (CCE). When CCE manifests as acute unilateral limb weakness, it may closely mimic acute cerebral infarction (ACI), resulting in misdiagnosis and delayed initiation of immunotherapy. Here, we report a case initially diagnosed as ACI and highlight key diagnostic clues and therapeutic considerations that may help to facilitate earlier recognition and appropriate management. A young woman was admitted with left-sided limb weakness and was initially diagnosed with ACI. The final diagnosis was myelin oligodendrocyte glycoprotein (MOG) antibody-related autoimmune encephalitis, which was established by integrating the clinical presentation, contrast-enhanced brain magnetic resonance imaging (MRI) findings, cerebrospinal fluid (CSF) antibody testing, and systematic exclusion of alternative etiologies. After an initial misdiagnosis, the patient received neuroprotective agents without meaningful improvement. Subsequent lumbar puncture and CSF testing supported the diagnosis of MOG antibody-related autoimmune encephalitis. Treatment was then adjusted to include both high-dose intravenous corticosteroid pulse therapy and immunotherapy, which resulted in marked clinical improvement. The patient's condition remained stable at 2-month follow-up, with no disease progression. Contrast-enhanced MRI showed no abnormalities in the unilateral cerebral cortex. CCE associated with MOGAD may be misdiagnosed as ACI, particularly when focal motor deficits are the predominant presenting feature. Early recognition, prompt diagnostic evaluation, and timely initiation of immunotherapy are crucial for improving clinical outcomes. Greater clinician awareness and improved neuroimaging-based differentiation between ACI and CCE are therefore needed. In patients with stroke-mimicking presentations but atypical imaging findings, contrast-enhanced MRI and cerebrospinal fluid testing for MOG immunoglobulin G should be prioritized. High-dose steroid pulse therapy combined with immunotherapy remains the recommended first-line treatment for suspected MOG antibody-related autoimmune encephalitis.

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2026-05-26 | Recurrent Myelin Oligodendrocyte Glycoprotein Antibody-Positive Aseptic Meningitis at the Identical Site in a Child: Leptomeningeal Enhancement Detected by Contrast-Enhanced FLAIR Imaging.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an acquired demyelinating syndrome of the central nervous system mediated by MOG antibodies. Clinical phenotypes include optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. MOG antibody-associated meningitis (MOGAM) is a form of aseptic meningitis characterized by the presence of serum MOG antibodies. Parenchymal lesions are typically absent in MOGAM; however, some cases may progress to MOGAD. Leptomeningeal lesions are rarely detected on contrast-enhanced T1-weighted magnetic resonance imaging (MRI) but may be conspicuous on contrast-enhanced fluid-attenuated inversion recovery (FLAIR) imaging. We report a case of a 5-year-old boy who initially presented with a prolonged headache and fever. Contrast-enhanced FLAIR imaging revealed localized leptomeningeal enhancement (LME), and the patient was treated for suspected aseptic meningitis. Subsequently, additional parenchymal lesions developed, and serum MOG antibodies were detected, leading to a diagnosis of MOGAD. He achieved remission with corticosteroid therapy but experienced a relapse a year later, with LME localized to the same site on MRI. At recurrence, the contrast-enhanced T1-weighted image showed faint leptomeningeal enhancement, whereas contrast-enhanced FLAIR imaging clearly depicted LME. Intravenous methylprednisolone pulse therapy was promptly initiated, which resulted in resolution without progression to MOGAD. This is the first pediatric report of recurrent MOGAM with LME recurring at the identical site. Contrast-enhanced FLAIR imaging enabled early detection of LME at recurrence and facilitated timely corticosteroid therapy, which may have prevented progression to MOGAD.

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2026-04-13 | Analysis of risk factors for relapse in pediatric MOG antibody-associated disease: a single-center retrospective cohort study.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an immune-mediated demyelinating disorder of the central nervous system. It has a higher incidence in children than in adults, carries a relatively high risk of relapse with unclear mechanisms, and significantly impacts patient prognosis. This study aimed to investigate the clinical characteristics of MOGAD and identify independent risk factors for relapse, to provide a basis for early intervention and individualized treatment. A total of 108 children diagnosed with MOGAD at the Children's Hospital of Nanjing Medical University between January 2020 and June 2024 were retrospectively enrolled. Clinical, laboratory, and radiological data were collected. Univariate analysis and multivariate logistic regression models were used to screen for risk factors associated with relapse. The median follow-up time was 37 months, with a relapse rate of 30.6%. The proportion of patients presenting with seizures (54.5% vs. 20.0%, p < 0.001) and limb weakness (27.3% vs. 9.3%, p = 0.018) at onset was significantly higher in the relapse group compared to the monophasic group. The most common initial clinical phenotype was acute disseminated encephalomyelitis (ADEM) type (38.9%), while optic neuritis (ON) type became predominant at relapse (48.9%). Multivariate analysis identified seizures (OR = 7.155, 95% CI: 2.265-22.604, p < 0.001) and limb weakness (OR = 5.157, 95% CI: 1.322-20.117, p = 0.018) as independent risk factors for relapse, whereas a normal brain MRI (OR = 0.186, 95% CI: 0.035-0.985, p = 0.048) was a protective factor. The relapse group had a higher proportion of patients with high serum MOG antibody titers (≥1:100) (54.5% vs. 32.0%, p = 0.027) and elevated cerebrospinal fluid cell counts (>30 × 106/L) (66.7% vs. 44.0%, p = 0.03). Patients receiving oral corticosteroids for ≥6 months during the remission phase had a significantly lower relapse rate (42.4% vs. 65.3%, p = 0.026). In pediatric MOGAD, the ADEM phenotype is most common at onset. Seizures and limb weakness at initial presentation are independent risk factors for relapse in children with MOGAD, while a normal brain MRI suggests a lower relapse risk. Prolonging the corticosteroid treatment course during remission (≥6 months) may help reduce the relapse risk. Enhanced follow-up and individualized treatment should be considered for children with high-risk factors. These findings may help clinicians identify high-risk patients and tailor long-term immunosuppressive therapy.

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2026-03-01 | Acute disseminated encephalomyelitis in a young female from rural Nepal: A rare case report

Abstract: Introduction: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated demyelinating disease of the central nervous system characterized by myriad neurological deficits and symptoms, including encephalopathy. The heterogeneity of the clinical presentation and the rarity of the condition can potentially delay diagnosis. Early diagnosis could be lifesaving, but is often challenging, especially in resource-limited settings. Case presentation: We report a case of ADEM in a 27 years old young Asian female from a remote village in Nepal who presented with a rapidly deteriorating neurological decline. Early clinical suspicion, timely diagnosis, and prompt treatment with intravenous corticosteroids led to favorable outcomes. Over a three-year follow up period, she achieved complete neurological recovery and exhibited a typical monophasic disease course. Conclusion: This case underscores the importance of maintaining a broad differential diagnosis of acute neurological presentations and highlights how early intervention can fully reverse treatable neurological diseases.

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2025-07-11 | Post anti-rabies vaccine encephalitis presenting as acute psychosis: Case reports.

Rabies, an invariably fatal zoonotic disease, remains a major public health concern in resource-limited settings, where access to cell culture-derived vaccines constrained hampers comprehensive immunization programs and compromises effective disease control and prevention efforts. As a result, several countries, including Ethiopia, continue to utilize nerve tissue-derived vaccines (NTVs)-notably phenolized sheep brain formulations-despite their well-documented neurotoxicity and the global recommendation to transition to safer, cell culture-based alternatives. This report describes two clinically significant cases of severe neuroinflammatory syndromes temporally associated with the administration of nerve tissue-derived phenolized sheep brain rabies vaccine. The first involves a 50-year-old male who developed acute neuropsychiatric symptoms including agitation, confusion, hallucinations, ataxia, and profound sleep disturbances approximately two weeks after completing a full course of the nerve tissue anti-rabies vaccine. Magnetic resonance imaging (MRI) of the brain demonstrated bilateral, asymmetric hyperintensities on T2-weighted and FLAIR sequences involving the cingulate gyrus, basal ganglia, medial thalami, hypothalamus, and fornix, findings that are radiologically consistent with limbic encephalitis. The second case describes a previously healthy 4-year-old boy who experienced rapidly progressive quadriplegia, encephalopathy, and seizures following vaccination, with MRI findings characteristic of acute disseminated encephalomyelitis (ADEM), showing diffuse, symmetrical involvement of deep grey matter structures, brainstem, and cerebellar hemispheres. These cases show rare but serious central nervous system complications from nerve tissue rabies vaccines and highlight the urgent need to replace them with safer, WHO-recommended cell culture vaccines.

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2020-06-11 | Herpes Simplex Virus 1 Induces Brain Inflammation and Multifocal Demyelination in the Cotton Rat Sigmodon hispidus.

Demyelinating central nervous system (CNS) disorders like multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) have been difficult to study and treat due to the lack of understanding of their etiology. Numerous cases point to the link between herpes simplex virus (HSV) infection and multifocal CNS demyelination in humans; however, convincing evidence from animal models has been missing. In this work, we found that HSV-1 infection of the cotton rat Sigmodon hispidus via a common route (lip abrasion) can cause multifocal CNS demyelination and inflammation. Remyelination occurred shortly after demyelination in HSV-1-infected cotton rats but could be incomplete, resulting in "scars," further supporting an association between HSV-1 infection and multifocal demyelinating disorders. Virus was detected sequentially in the lip, trigeminal ganglia, and brain of infected animals. Brain pathology developed primarily on the ipsilateral side of the brain stem, in the cerebellum, and contralateral side of the forebrain/midbrain, suggesting that the changes may ascend along the trigeminal lemniscus pathway. Neurologic defects occasionally detected in infected animals (e.g., defective whisker touch and blink responses and compromised balance) could be representative of the brain stem/cerebellum dysfunction. Immunization of cotton rats with a split HSV-1 vaccine protected animals against viral replication and brain pathology, suggesting that vaccination against HSV-1 may protect against demyelinating disorders.IMPORTANCE Our work demonstrates for the first time a direct association between infection with herpes simplex virus 1, a ubiquitous human pathogen generally associated with facial cold sores, and multifocal brain demyelination in an otherwise normal host, the cotton rat Sigmodon hispidus For a long time, demyelinating diseases were considered to be autoimmune in nature and were studied by indirect methods, such as immunizing animals with myelin components or feeding them toxic substances that induce demyelination. Treatment against demyelinating diseases has been elusive, partially because of their unknown etiology. This work provides the first experimental evidence for the role of HSV-1 as the etiologic agent of multifocal brain demyelination in a normal host and suggests that vaccination against HSV-1 can help to combat demyelinating disorders.

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2014-03-19 | The spectrum of post-vaccination inflammatory CNS demyelinating syndromes.

A wide variety of inflammatory diseases temporally associated with the administration of various vaccines, has been reported in the literature. A PubMed search from 1979 to 2013 revealed seventy one (71) documented cases. The most commonly reported vaccinations that were associated with CNS demyelinating diseases included influenza (21 cases), human papilloma virus (HPV) (9 cases), hepatitis A or B (8 cases), rabies (5 cases), measles (5 cases), rubella (5 cases), yellow fever (3 cases), anthrax (2 cases),meningococcus (2 cases) and tetanus (2 cases). The vast majority of post-vaccination CNS demyelinating syndromes, are related to influenza vaccination and this could be attributed to the high percentage of the population that received the vaccine during the HI1N1 epidemia from 2009 to 2012. Usually the symptoms of the CNS demyelinating syndrome appear few days following the immunization (mean: 14.2 days) but there are cases where the clinical presentation was delayed (more than 3 weeks or even up to 5 months post-vaccination) (approximately a third of all the reported cases). In terms of the clinical presentation and the affected CNS areas, there is a great diversity among the reported cases of post-vaccination acute demyelinating syndromes. Optic neuritis was the prominent clinical presentation in 38 cases, multifocal disseminated demyelination in 30, myelitis in 24 and encephalitis in 17. Interestingly in a rather high proportion of the patients (and especially following influenza and human papiloma virus vaccination-HPV) the dominant localizations of demyelination were the optic nerves and the myelon, presenting as optic neuritis and myelitis (with or without additional manifestations of ADEM), reminiscent to neuromyelitic optica (or, more generally, the NMO-spectrum of diseases). Seven patients suffered an NMO-like disease following HPV and we had two similar cases in our Center. One patient with post-vaccination ADEM, subsequently developed NMO. Overall, the risk of a demyelinating CNS disease following vaccination, although non-negligible, is relatively low. The risk of onset or relapse of CNS demyelination following infections against which the vaccines are aimed to protect, is substantially higher and the benefits of vaccinations surpass the potential risks of CNS inflammation. This does not in any way exempt us from“learning” the lessons taught by the reported cases and searching new and safer ways to improve vaccination techniques and increase their safety profile.

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2004-09-14 | The neurite outgrowth inhibitor Nogo A is involved in autoimmune-mediated demyelination.

Inhibitors associated with CNS myelin are thought to be important in the failure of axons to regenerate after spinal cord injury and in other neurodegenerative disorders. Here we show that targeting the CNS-specific inhibitor of neurite outgrowth Nogo A by active immunization blunts clinical signs, demyelination and axonal damage associated with experimental autoimmune encephalomyelitis (EAE), a model of multiple sclerosis (MS). Mice vaccinated against Nogo A produce Nogo-specific antibodies that block the neurite outgrowth inhibitory activity associated with CNS myelin in vitro. Passive immunization with anti-Nogo IgGs also suppresses EAE. Our results identify Nogo A as an important determinant of the development of EAE and suggest that its blockade may help to maintain and/or to restore the neuronal integrity of the CNS after autoimmune insult in diseases such as MS. Our finding that Nogo A is involved in CNS autoimmune demyelination indicates that this molecule may have a far more complex role than has been previously anticipated.

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