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BACKGROUND: Acute Disseminated Encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, characterize by multiple white matter hyperintensities on T2 MRI. Patients usually present with subacute progressive encephalopathy and polyfocal neurological deficits. Possible treatments are corticosteroids, immunoglobulins and plasma exchange. Full clinical recovery is seen in more than half of the cases. CASE: We describe a case of a 62-year-old patient presenting with thunderclap headache as the first symptom, two weeks after an upper respiratory tract infection. The clinical course was complicated by progressive coma and intracranial hypertension mandating external ventricular drainage and sedation. Initial treatment with methylprednisolone was unsuccessful but clinical resolution and radiological regression was achieved after plasma exchanges and cyclophosphamide. CONCLUSION: To our knowledge, this is the first reported case of ADEM presenting with thunderclap headache. Intracranial hypertension with the need for invasive neuromonitoring and pressure management is also a very rare complication of ADEM. In this report, we describe the findings of the literature review concerning ADEM, thunderclap headache and intracranial hypertension.

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Hurst disease, or Weston-Hurst syndrome, or acute hemorrhagic encephalomyelitis (AHEM), is an infrequent condition that usually gets provoked after a viral infection (respiratory tract infection) or, as reported in many case reports, post-vaccination. Hurst disease is a difficult-to-diagnose condition because it mimics common presentations such as stroke, decreased or loss of consciousness, brain abscess, and seizures, making clinical diagnosis challenging. Radiological imaging, such as magnetic resonance imaging (MRI) of the brain with diffusion-weighted imaging, now serves as the primary modality to identify such conditions, despite its lack of specificity. The treating doctor needs to do an in-depth analysis of the patient's history, as this carries a very high mortality rate. We hereby discuss a case that presented with seizures and deteriorating Glasgow Coma Scale (GCS) score, on imaging revealed posterior circulation acute hemorrhagic leukoencephalitis (AHLE)/AHEM, therein treated with steroids, plasmapheresis resulted in a good outcome implicating, early detection and timely management can reduce the mortality due to this condition.

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Paediatric autoimmune encephalitis, including acute disseminated encephalomyelitis, are inflammatory brain diseases presenting with cognitive deficits, psychiatric symptoms, seizures, MRI and EEG abnormalities. Despite improvements in disease recognition and early immunotherapy, long-term outcomes in paediatric autoimmune encephalitis remain poor. Our aim was to understand functional connectivity changes that could be associated with negative developmental outcomes across different types of paediatric autoimmune encephalitis using magnetoencephalography. Participants were children diagnosed with paediatric autoimmune encephalitis at least 18 months before testing and typically developing children. All completed magnetoencephalography recording at rest, T1 MRI scans and neuropsychology testing. Brain connectivity (specifically in delta and theta) was estimated with amplitude envelope correlation, and network efficiency was measured using graph measures (global efficiency, local efficiency and modularity). Twelve children with paediatric autoimmune encephalitis (11.2 ± 3.5 years, interquartile range 9 years; 5M:7F) and 12 typically developing controls (10.6 ± 3.2 years, interquartile range 7 years; 8M:4F) participated. Children with paediatric autoimmune encephalitis did not differ from controls in working memory (t(21) = 1.449; P = 0.162; d = 0.605) but had significantly lower processing speed (t(21) = 2.463; P = 0.023; Cohen's d = 1.028). Groups did not differ in theta network topology measures. The paediatric autoimmune encephalitis group had a significantly lower delta local efficiency across all thresholds tested (d = -1.60 at network threshold 14%). Theta modularity was associated with lower working memory (ß = -0.781; t(8) = -2.588, P = 0.032); this effect did not survive correction for multiple comparisons (P(corr) = 0.224). Magnetoencephalography was able to capture specific network alterations in paediatric autoimmune encephalitis patients. This preliminary study demonstrates that magnetoencephalography is an appropriate tool for assessing children with paediatric autoimmune encephalitis and could be associated with cognitive outcomes.

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Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune disorder characterized by brain and spinal cord inflammation. In pediatric patients, ADEM presents unique challenges due to its potential for rapid progression and long-term neurological sequelae. Therapeutic plasma exchange (TPE) has emerged as a potential treatment option by targeting the underlying autoimmune process and modulating the inflammatory response. This comprehensive review evaluates the role of TPE in pediatric ADEM, synthesizing evidence from clinical studies and providing insights into its efficacy, safety, and potential benefits. The review highlights the variability in TPE efficacy based on disease severity and patient-specific factors. Implications for clinical practice include considering TPE as a therapeutic option, particularly in severe or refractory cases, and emphasizing the importance of early intervention. Recommendations for future research include long-term prospective studies, comparative effectiveness trials, and efforts to standardize TPE protocols. Overall, continued investigation and innovation in managing pediatric ADEM are essential for improving outcomes and quality of life for affected children and their families.

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Tuberculosis is a leading cause of mortality in children worldwide. One of the greatest challenges in its management is the difficulty of diagnosis as the manifestations are non-specific and often mimic other illnesses. Neurological infection occurs in approximately 1% of patients diagnosed with tuberculosis, and usually takes the form of tuberculous meningitis or tuberculoma. An 11-year-old girl who was diagnosed with acute disseminated encephalomyelitis, a rare immunological manifestation of tuberculosis, is presented. She recovered completely after a course of high-dose systemic corticosteroids in addition to anti-tuberculosis treatment. Considering the immense burden of this infectious disease, recognition and understanding of the uncommon manifestations are important to enable appropriate and timely treatment.Abbreviations: ADEM: acute disseminated encephalomyelitis; ATT: anti-tuberculosis therapy; CBNAAT: cartridge-based nucleic acid amplification test; CNS: central nervous system; CSF: cerebrospinal fluid; CT: computed tomography; FLAIR: fluid attenuated inversion recovery; IFN: interferon; MRI: magnetic resonance imaging; MTB: Mycobacterium tuberculosis; TB: tuberculosis; TNF: tumour necrosis factor.

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Acute disseminated encephalomyelitis and Guillain-Barré syndrome refer to post-infectious or post-vaccination inflammatory demyelinating disorders of central and peripheral nervous system, respectively. We report the case of a 60-year-old male patient presenting with irritability, gait difficulty, asymmetric quadriparesis (mostly in his left extremities), distal sensory loss for pain and temperature in left limbs, and reduced tendon reflexes in his upper limbs and absent in his lower limbs, following an upper respiratory tract infection, 3 weeks earlier. Brain magnetic resonance imaging revealed abnormal T2 signal and peripherally enhancing lesions in hemispheres, brainstem, and cerebellum. Nerve conduction studies were compatible with acute motor and sensory axonal neuropathy. Serology revealed positive IgM and IgG antibodies for Chlamydia pneumoniae, and he also tested positive for myelin oligodendrocyte glycoprotein (MOG) and sulfatide antibodies. Treatment with intravenous immunoglobulin and methylprednisolone led to clinical and radiological recovery within weeks. Even though several cases of combined central and peripheral demyelination have been reported before, it is the first case report with seropositive anti-sulfatide and anti-MOG acute sensorimotor axonal neuropathy and disseminated encephalitis associated with C. pneumoniae.

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Introduction: Acute disseminated encephalomyelitis (ADEM) is a rare neurological disorder characterized by inflammation in the brain and spinal cord. This systematic review aims to investigate the potential association between ADEM and influenza vaccination by analyzing relevant case reports. ADEM is traditionally thought to be a monophasic condition, predominantly affecting children, often following viral illnesses or immunizations. Recent attention has focused on a possible link between ADEM and influenza vaccination, prompting the need for a thorough investigation. Methods: The systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and the AMSTAR2 (A MeaSurement Tool to Assess systematic Reviews 2) guidelines. Electronic searches were conducted on PubMed, Cochrane Library, and clinicaltrials.gov databases, spanning up to August 2023. Inclusion criteria encompassed full-text articles in English, observational studies, case reports, and case series providing comprehensive details for confirming clinical diagnoses of ADEM following influenza vaccination. Data were extracted, including demographic information, vaccination details, clinical symptoms, diagnostic evaluations, treatment modalities, and outcomes. Quality assessment was performed using the Joanna Briggs Institute (JBI) Critical Appraisal tool. Results: A total of 23 cases of ADEM following influenza vaccination were identified from 19 included articles. The mean age of affected individuals was 40.2 years (±25.7) with 60.8% being male. Common presenting symptoms included muscle weakness (52.1%), urinary abnormalities (30.4%), altered consciousness (26%), and sensory disturbances (26%). Neurological examination revealed findings such as extensor plantar reflex (positive Babinski sign) in 26%, hyperreflexia in 30.4%, and generalized hyporeflexia in 13% of the cases. Diagnostic evaluations involved MRI, showing multiple hyperintense lesions in cerebral hemispheres (43.4%), subcortex (60.8%), and spinal cord (39.1%). Cerebrospinal fluid analysis indicated elevated white blood cell count in 69.5% of cases, with lymphocytic pleocytosis in 52.1%. Oligoclonal bands were reported positively in 8.6% of cases. Treatment approaches varied, with intravenous methylprednisolone being the most common (39.1%). Out of the 23 cases, two (8.6%) patients had a fatal outcome, while the rest showed clinical improvement with complete or partial resolution of symptoms. Persisting symptoms included numbness in the lower extremities (8.6%) and impaired ability to walk after 10 months (4.3%). Conclusion: While the association between ADEM and influenza vaccination is rare, healthcare professionals should remain vigilant and consider patients' vaccination history, particularly following an influenza immunization. This systematic review highlights the clinical manifestations, diagnostic tools, treatment approaches, and outcomes of ADEM cases post-influenza vaccination. Further research is essential to understand this association and improve clinical decision-making, ensuring the safety and efficacy of immunization programs.

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Acute disseminated encephalomyelitis (ADEM) has been identified as an Adverse Event of Special Interest in the COVID-19 vaccine programme due to its long-standing temporal association with a wide range of other vaccines. Case reports of ADEM shortly following COVID-19 vaccination have now been documented. There were 217 ADEM admissions in 215 individuals in the period 8th December 2020 to 31st March 2023. An increased risk of ADEM following the first dose of ChAdOx1 vaccine was observed (relative incidence (RI) = 3.13, 95% Confidence Interval (CI) [1.56-6.25]) with a vaccine attributable risk of 0.39 per million doses. When doses 1 and 2 were combined this increased risk remained just significant (1.96 [95%CI 1.01-3.82]). No significant increased risk was observed with any other vaccine or dose. This small, elevated risk after the first dose of ChAdOx1-S vaccine demonstrates how large national electronic datasets can be used to identify very rare risks and provides reassurance that any risk of ADEM following the ChAdOx1-S COVID-19 vaccination is extremely small. Given the rarity of this risk, further studies in settings with access to data on large populations should be carried out to verify these findings.

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BACKGROUND: Relapsing or recurrent tumefactive demyelination is rare and has not been studied beyond individual case reports. OBJECTIVE: We examined the clinical course, neuroimaging, cerebrospinal fluid (CSF), treatment and outcomes of patients with recurrent tumefactive demyelinating lesions (TDLs). METHODS: We used PubMed to identify reports of recurrent TDLs and included the details of an additional, unpublished patient. RESULTS: We identified 18 cases (11F, 7 M). The median age at onset of the index TDL was 37 years (range 12-72) and most were solitary lesions 72 % (13/18). CSF-restricted oligoclonal bands (OCBs) were detected in 25 % (4/16). Only one of those tested (n = 13) was positive for AQP4-IgG. A moderate-to-marked treatment response (high dose corticosteroid with or without additional plasmapheresis, IVIg or disease modifying therapies) was evident in 89 % of treated patients. Median EDSS at the median follow-up of 36 months (range 6-144) was 2 (range 1-10). Most remained ambulatory (EDSS < 4 in 13/18), but 1 patient died. CONCLUSION: The median age of patients with relapsing TDLs is similar to that of typical MS, but differences include a lower female:male sex ratio, larger lesions, and a comparative lack of CSF-restricted OCBs. Outcomes vary among this group of patients ranging from minimal disability through to death.

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INTRODUCTION: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM. MATERIALS AND METHODS: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE. RESULTS: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day. CONCLUSION: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.

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BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently defined demyelinating disorder with a rapidly evolving clinical spectrum. Recently, consensus criteria have been proposed (Banwell et al., 2023) to help with disease diagnosis. However, validation of the proposed criteria in real-life MOGAD patients is lacking. In this study, we applied the proposed criteria to an institutional cohort of MOG antibody-positive patients. METHODS: A retrospective study was conducted at a tertiary neuroimmunology clinic from 2018 to 2023. Patients who had at least one core clinical feature of MOGAD and positive serum MOG antibody by cell-based assay were included. Demographics and clinical data were recorded and analyzed. Cases were divided into definite MOGAD, questionable MOGAD, and false-positive MOG antibody as determined by the treating neuroimmunology and/or neuro-ophthalmology specialists prior to applying the new MOGAD criteria by an independent investigator. We then calculated the sensitivity, specificity, positive predictive value, and negative predictive value of the new criteria compared to the treating physicians' assessment. RESULTS: A total of 27 patients were included of which, 19 (70.4%) were female, the average age of the sample was 44 +/- 15 years. High titer MOG antibody (≥ 1:100) was found in 11 patients (40.7%); low titer (< 1: 100) in 13 (48.1%), and unreported titer in 3 patients. As determined by expert opinion; 18 (66.7%) were identified as definitive MOGAD, 6 (22.2%) as false-positive MOG antibody, and 3 (11.1%) as questionable MOGAD. All 18 patients identified by clinicians as definite MOGAD met the new 2023 criteria. Of the 9 patients with questionable MOGAD or false-positive MOG antibody, four patients met the 2023 MOGAD criteria. Those four patients had the following final diagnoses: CNS vasculitis, primary progressive MS with activity and progression, pseudotumor cerebri, and bevacizumab-induced anterior ischemic optic neuropathy in the setting of paraneoplastic retinopathy. Compared to clinician assessment, applying the 2023 MOGAD criteria to our institutional cohort yielded a sensitivity of 100%, a specificity of 55.5%, a positive predictive value of 81.5% and a negative predictive value of 100%. CONCLUSION: These findings suggest that the 2023 MOGAD criteria are highly sensitive for detection of definite MOGAD but has modest specificity. A number of MOGAD mimickers can resemble the core clinical events of MOGAD and share similar supportive clinical and MRI features. Clinicians should practice caution when evaluating patients with low titer MOG antibody even if they meet the additional supportive features proposed by the 2023 criteria. Further studies are needed to evaluate the 2023 criteria in larger cohorts and in the pediatric population.

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Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a group of central nervous system (CNS) demyelinating diseases caused by autoantibodies against myelin oligosaccharide protein (MOG), a myelin sheath component protein, and present with a variety of symptoms, including optic neuritis, myelitis, acute disseminated encephalomyelitis (ADEM), brainstem encephalitis, and corticobasal encephalitis. It is currently unknown at what point in life MOGAD can develop or how it can be triggered by autoimmune mechanisms. Here, we report a case of a mature woman who suffered from adenoviral meningitis one month after childbirth and developed MOGAD but was able to return to child rearing with high-dose methylprednisolone therapy. This case suggests that the risk of developing MOGAD early after childbirth may be increased. The case also suggested that adenoviral infection may be involved in the development of MOGAD.

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Epstein-Barr virus (EBV) is a common human herpesvirus associated with a wide range of clinical manifestations, primarily affecting the lymphoid system. However, central nervous system (CNS) involvement, although rare, can occur and present a diagnostic challenge, particularly in immunocompetent individuals. We present a case of a 28-year-old healthy female who initially presented with a flu-like illness, her symptoms rapidly progressed, leading to neurological deficits, and altered mental status. The patient's diagnostic workup, including a viral panel and various antibodies, failed to provide a conclusive diagnosis. However, lumbar puncture revealed significant abnormalities in cerebrospinal fluid (CSF), including elevated white blood cell count and elevated CSF protein. Neuroimaging studies demonstrated non-specific findings in subcortical white matter, pontomedullary junction, and extended spinal cord lesion. Tragically, the patient's condition rapidly worsened, with diffuse cerebral edema observed on repeat imaging, leading to the patient's demise even after conventional treatment. CSF analysis, performed at an apex lab, unexpectedly returned positive for EBV PCR, indicating a diagnosis of EBV encephalitis or EBV-associated acute disseminated encephalomyelitis (ADEM). This case highlights the challenges encountered in diagnosing EBV-associated CNS manifestations, especially in immunocompetent individuals, where these presentations are exceedingly rare. The atypical clinical course, negative initial laboratory investigations, and absence of specific radiological findings further complicated the diagnostic process. Early recognition and consideration of infectious etiologies, including EBV, in patients presenting with unexplained encephalitis or ADEM-like symptoms, are essential for timely intervention and optimal patient outcomes.

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Introduction and importance: Coronavirus disease 2019 (COVID-19) is a disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and various vaccines against it have been developed. Acute disseminated encephalomyelitis (ADEM) is a disease of the central nervous system that cause inflammation and demyelination and manifests as a multi-symptom acute neurological condition. Although infections are usually the cause of ADEM, vaccines may cause 5-10% of cases. Case presentation: A 40-year-old woman had received a second dose of the Sinopharm COVID-19 vaccine 4 months before her visit and experienced sudden gait imbalance and vertigo a day after her vaccination, which lasted for more than a month. On examination, no signs of skin bruising or bleeding were observed, and her vital signs were within the normal range. On neurological assessment, the patient had a Glasgow Coma Scale score of 14/15 (E4V5M5), had normal pupil size and light reaction, normal fundus, normal deep tendon reflexes and bilateral extensor plantar response. Meningeal symptoms were absent, and SARS-CoV-2 RNA tests using NAAT (Nucleic Acid Amplification Test) were negative. Development of central nervous system (CNS) manifestations during the recovery phase of fever, along with typical MRI findings; the diagnosis of para-infectious ADEM with COVID-19 vaccination was made. After the treatment with methylprednisolone sodium succinate injection, the patient showed improvement. Clinical discussion: ADEM associated with post-vaccinations is a rare condition. There has been growing evidence that shared epitopes between neuronal proteins and SARS-CoV-2 antigens may trigger autoimmune reactions against the CNS through molecular mimicry as its pathogenesis. Conclusion: We suggest the need for a strict vaccine safety monitoring system and post-vaccine monitoring and surveillance.

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Post-malaria neurological syndrome (PMNS) is a rare, self-limiting condition that presents with a wide range of neurological manifestations after clearance of malarial infection, especially 𝘗𝘭𝘢𝘴𝘮𝘰𝘥𝘪𝘶𝘮 f𝘢𝘭𝘤𝘪𝘱𝘢𝘳𝘶𝘮, most patients recover without residual deficits. Here we present a case of a 29-year-old, male with a recent history of malaria treated successfully, who presented due to a generalized tonic-clonic seizure, without any other neurological symptoms, the examination and labs were unremarkable, he underwent a computer tomography (CT) scan and Magnetic resonant imaging (MRI) which both showed two areas of vasogenic edema involving the subcortical white matter of left frontal and right posterior parasagittal regions, all autoimmune screens, infection workup from blood and CSF were negative, he underwent a brain biopsy that showed intense perivascular inflammation with neuronal loss and gliosis, findings are nonspecific and can be seen in a variety of condition. The patient's condition improved, and he was discharged without any complications.

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INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a demyelinating immune-mediated condition of the central nervous system, whereas antiphospholipid antibody syndrome (APLA) is an autoimmune disorder accompanied by thrombosis and pregnancy-related problems. We present a unique case of a 30-year-old female with ADEM coexisting with APLA, highlighting the importance of early identification and specialized care. PRESENTATION OF CASE: We present a case of a 30-year-old woman with a history of hypertension, multiple miscarriages, and non-compliance with medication, who presented with altered consciousness and weakness in all four limbs. Laboratory tests revealed positive anti-cardiolipin and lupus anticoagulant antibodies, confirming APLA. A neurological examination revealed increased limb tone, heightened reflexes, and extensor plantar responses. MRI revealed confluent white matter lesions that were consistent with ADEM. The patient received prompt treatment with intravenous methylprednisolone and then received oral prednisone, leading to a rapid improvement in neurological status. DISCUSSION: The intricate interaction between ADEM and APLA remains enigmatic. The plausible connection between "molecular mimicry" and weakened blood-brain barrier, substantiated by antiphospholipid antibodies, may help explain their concurrent occurrence. CONCLUSION: This case highlights the significance of early diagnosis and management of the rare and complex coexistence of ADEM and APLA to attain optimal outcomes, as well as the significance of careful examination for simultaneous autoimmune markers in individuals presenting with neurological disturbances.

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Acute disseminated encephalomyelitis (ADEM) is a rare illness. It is characterized by different presentations like encephalopathy, seizures, hemiplegia, and visual symptoms. We present a patient who presented seizures and encephalopathy. Brain MRI showed symmetrical white and gray matter lesions. He was treated with acyclovir for viral encephalitis and given immunotherapy for ADEM. The radiological findings may be inconclusive in some cases, hence differential diagnosis of both viral encephalitis and ADEM needs to be considered. Early immunotherapy is required in such fulminant cases.