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Copyright of Hungarian Medical Journal / Orvosi Hetilap is the property of Akademiai Kiado and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Key Clinical Message: Concurrent recurrence of acute disseminated encephalomyelitis (ADEM) and poststreptococcal glomerulonephritis (PSGN) in a thalassemia intermedia patient is rare and underscores the complexity of autoimmune disorders. This case emphasizes the importance of considering ADEM in the differential diagnosis of children presenting with PSGN accompanied by neurological symptoms. Post‐streptococcal glomerulonephritis (PSGN) is a common group A streptococcal (GAS) infection sequela. The pathophysiology of PSGN involves immune complex deposition, with type 3 hypersensitivity reaction triggered by GAS. Certain neurological conditions may also arise following a GAS infection, possibly due to molecular mimicry in the brain, a pathophysiology similar to rheumatic fever, another common sequel of GAS infection. We present the case of a child with β‐thalassemia intermedia who exhibited the classic triad (edema, hypertension, hematuria) of PSGN along with neurological manifestations, including a low glasgow coma scale (GCS) score and seizures. Magnetic resonance imaging (MRI) of the brain indicated changes consistent with acute disseminated encephalomyelitis (ADEM). Initially treated with methylprednisolone, the patient eventually received intravenous immunoglobulin (IVIG) due to lack of response. The patient had a good outcome, with complete resolution of all symptoms and no residual neurological deficits. This case underscores the importance of considering ADEM in the differential diagnosis for patients presenting with neurological signs and symptoms following a recent throat infection with GAS. Furthermore, given the increased risk of infection in thalassemia, patients with thalassemia who have a throat infection and neurological symptoms should be evaluated for the possible presence of ADEM. [ABSTRACT FROM AUTHOR]

Background Pediatric acquired demyelinating syndrome (ADS) constitutes a group of treatable disorders with acute neurologic dysfunction. Neuroimaging has played a significant role in diagnosis of ADS. We describe clinico-radiologic spectrum, outcomes, and comparison of the groups: acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD), clinically isolated syndrome (CIS), multiple sclerosis (MS), and myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD). Methods Retrospective review of 70 children with ADS at a tertiary care hospital over 15 years (2008–2023) was performed. Diagnosis was assigned as per International Pediatric Multiple Sclerosis Study Group criteria 2016. Fisher's exact and chi-square tests were applied. Results Thirty-nine boys and 31 girls aged 8.2 ± 4.0 years with CIS (n = 27), ADEM (n = 16), NMOSD (n = 13), MS (n = 1), and MOGAD (n = 13) were included. Clinical syndromes with positive significant association included polyfocal symptoms, encephalopathy in ADEM, optic neuritis (ON) in MOGAD, brainstem, area postrema syndrome in NMOSD. MOGAD presented with atypical presentations like prolonged fever (PF; 76.9%) and aseptic meningitis (23%). Seropositivity for myelin oligodendrocyte glycoprotein immunoglobulin-G was 62% and for NMO-IgG 2.6%. Neuroimaging of MOGAD showed lesions predominantly in basal ganglia/thalami (69.2%), optic nerve (46.2%), and cerebellum (46.2%). Imaging patterns between ADEM and MOGAD were comparable except for more ON (p = 0.004), spinal cord (p = 0.01), and cerebellar lesions (p = 0.03) in MOGAD. Area postrema lesion was unique to NMOSD. All patients received immunotherapy, of whom 91.4% (n = 64) had good recovery, 8.6% (n = 6) had functional limitation on modified Rankin scale at discharge, and 12 (17.1%) relapsed. Conclusion The largest group was CIS. Seropositivity of MOG was high with atypical presentations like PF and aseptic meningitis. Specific neuroimaging patterns correlated with ADS categories. Short-term outcome with immunotherapy was favorable in spite of relapses. [ABSTRACT FROM AUTHOR]

Copyright of Neurologie Pro Praxi is the property of SOLEN sro and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: To determine the clinical spectrum, neuroimaging findings, and outcome of Acute Disseminated Encephalomyelitis (ADEM) in children. Method: We conducted a descriptive cross sectional study of all children aged 6 months to 18 years, diagnosed with ADEM at Aga Khan University Hospital, Karachi from January 2018 till December 2022. Results: This retrospective study enrolled 30 cases of ADEM, with a mean age of 6.43 ± 4.079, including 13 males and 17 females. The average hospital stay was 7.29 ± 4.379 days. The most common clinical features were fever, headache, and altered consciousness, while motor deficit was observed in 15 (53.5%) patients. Abnormal cerebrospinal fluid was found in 14 (46.6%) patients. Brain MRI identified bilateral and multifocal lesions in 22 (78.6%) patients, with brainstem lesions detected in 7 (25%) patients. Treatment included IV methylprednisolone (22; 73%), IVIG (9; 30%), or both (6; 20%). Clinical improvement was observed in 25 (89.3%) patients, with residual weakness present in eight (26%) patients at discharge. There was one reported death. Long-term complications included motor deficits, seizures, poor scholastic performance, and behavioral issues. Conclusion: The clinical presentation of ADEM is variable, but the most common symptoms are fever, headache, and altered consciousness. Despite generally favorable outcome, long-term monitoring revealed that patients may experience motor deficits, seizures, cognitive impairment, and academic difficulties. [ABSTRACT FROM AUTHOR]

Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating immune‐mediated disease characterized by bilateral and confluent lesions in white matter (WM), with an acute onset. This condition may arise due to a myriad of etiological factors, encompassing mainly vaccines and viral infections. This case report describes a 39‐y‐old patient who presented with a sudden onset of fever, confusion, and reduced level of consciousness, associated with paraparesis in the lower limbs and urinary retention, 2 d before admission to the neurological emergency department. The work‐up included analysis of the cerebrospinal fluid (CSF), which showed 1.6 cells/mm3 and elevated proteins (91 g/dL); in addition to magnetic resonance imaging (MRI) of the brain and the spinal cord, in which hyperintense ovoid lesions with asymmetrical and bilateral distribution in the WM and basal ganglia were observed in the T2 and FLAIR. Later, chikungunya virus was detected in a molecular viral panel in the CSF. The patient exhibited an improvement radiologically, and in his condition following pulse with methylprednisolone and intravenous immunoglobulin therapy, and 40 mg of prednisone was prescribed for management during outpatient follow‐up. This study highlights arbovirus infections as a possible cause of acute neurological conditions, involving both the brain and the spinal cord. Furthermore, the findings observed in the report were compared with those described in the literature, including other arboviruses. In conclusion, it was observed that the majority of patients responded to treatment with corticosteroids or immunoglobulins, with some neurological deficits eventually persisting. Therefore, more studies are needed to better investigate therapeutic options. [ABSTRACT FROM AUTHOR]

A minority of initial multiple sclerosis (MS) presentations clinically or radiologically resemble other central nervous system (CNS) pathologies, acute disseminated encephalomyelitis (ADEM) or tumefactive demyelination (atypical demyelination presentations). With the aim of better defining the long-term outcomes of this group we have performed a retrospective cohort comparison of atypical demyelination versus 'typical' MS presentations. Twenty-seven cases with atypical presentations (both first and subsequent demyelinating events) were identified and compared with typical MS cases. Disease features analysed included relapse rates, disability severity, whole brain and lesion volumes, lesion number and distribution. Atypical cases represented 3.9% of all MS cases. There was considerable overlap in the magnetic resonance imaging (MRI) features of ADEM-like and tumefactive demyelination cases. ADEM-like cases tended to be younger but not significantly so. Atypical cases showed a trend towards higher peak expanded disability severity score (EDSS) score at the time of their atypical presentation. Motor, cranial nerve, cerebellar, cerebral and multifocal presentations were all more common in atypical cases, and less likely to present with optic neuritis. Cerebrospinal fluid (CSF) white cell counts were higher in atypical cases (p = 0.002). One atypical case was associated with peripheral blood myelin oligodendrocyte glycoprotein (MOG) antibodies, but subsequent clinical and radiological course was in keeping with MS. There was no difference in long-term clinical outcomes including annualised relapse rates (ARR), brain volume, lesion numbers or lesion distributions. Atypical demyelination cases were more likely to receive high potency disease modifying therapy early in the course of their illness. Despite the severity of initial illness, our cohort analysis suggests that atypical demyelination presentations do not confer a higher risk of long-term adverse outcomes. [ABSTRACT FROM AUTHOR]

Purpose of Review: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a distinct neuroinflammatory condition characterized by attacks of optic neuritis, transverse myelitis, and other demyelinating events. Though it can mimic multiple sclerosis and neuromyelitis optica spectrum disorder, distinct clinical and radiologic features which can discriminate these conditions are now recognized. This review highlights recent advances in our understanding of clinical manifestations, diagnosis, and treatment of MOGAD. Recent Findings: Studies have identified subtleties of common clinical attacks and identified more rare phenotypes, including cerebral cortical encephalitis, which have broadened our understanding of the clinicoradiologic spectrum of MOGAD and culminated in the recent publication of proposed diagnostic criteria with a familiar construction to those diagnosing other neuroinflammatory conditions. These criteria, in combination with advances in antibody testing, should simultaneously lead to wider recognition and reduced incidence of misdiagnosis. In addition, recent observational studies have raised new questions about when to treat MOGAD chronically, and with which agent. Summary: MOGAD pathophysiology informs some of the relatively unique clinical and radiologic features which have come to define this condition, and similarly has implications for diagnosis and management. Further prospective studies and the first clinical trials of therapeutic options will answer several remaining questions about the peculiarities of this condition. [ABSTRACT FROM AUTHOR]

Acute disseminated encephalomyelitis (ADEM) is a common phenotype in children with myelin oligodendrocyte glycoprotein IgG (MOG-IgG)-associated disease. We aimed to identify novel genetic variants that distinguish children with MOG-IgG-positive ADEM (MOG-IgG+ ADEM) from children with MOG-IgG-negative ADEM (MOG-IgG− ADEM) using whole exome sequencing (WES) analysis. We conducted a two-stage study design. First, we performed WES on five patients with MOG-IgG+ ADEM and five patients with MOG-IgG− ADEM. Following bioinformatics analysis, the candidate variant list was constructed. Second, 29 children with MOG-IgG+ ADEM and 27 children with MOG-IgG− ADEM, together with discovery cohort, were genotyped to identify the novel variants. WES resulted in 33,999 variants, and 5388 nonsynonymous variants were selected for downstream analysis. In total, 118 protein-affecting variants that were significantly different between the two groups were identified. Together with the five variants extracted from the literature, 49 variants were selected as the candidate variant list for genotyping in the replication cohort. Finally, we identified three variants: rs11171951 in NACα , rs231775 in CTLA4, and rs11171951 in GOLGA5 , which were significantly different between MOG-IgG+ ADEM and MOG-IgG− ADEM. Only rs12440118 in NACα remained significant after Bonferroni correction for multiple testing (P adj < 0.001). We identified strong associations between NACα, CTLA4, and GOLGA5 variants and MOG-IgG+ ADEM in a Han Chinese population of Northern China, which may present novel genetic risk factor distinguishing patients with MOG-IgG+ ADEM from those with MOG-IgG− ADEM. [ABSTRACT FROM AUTHOR]

Various factors contribute to the development of infection-related acute encephalopathy (AE) in children, such as infectious agents and chronic underlying disorders. We studied underlying disorders in children with AE to identify predisposing factors of AE. We investigated underlying disorders or past histories in patients with two types of AE from the database in the Tokai area of Japan between 2009 and 2022: 204 patients with AE with reduced subcortical diffusion (AED) and 137 with clinically mild encephalopathy with a reversible splenial lesion (MERS). We compared them with 89 patients with acute disseminated encephalomyelitis (ADEM) to clarify the specific disorders in the two AE types. The prevalence of underlying disorders in AED (34%, 70 patients) was significantly higher than that in ADEM (12%, 11 patients) (P < 0.01). The prevalence of underlying disorders in MERS was 23% (32 patients). The underlying disorders included seizure disorders, premature birth, genetic/congenital disorders, and endocrine/renal diseases. In patients with seizure disorders in AED, five patients (18%) had Dravet syndrome and four (15%) had West syndrome, whereas none with MERS had these syndromes. Twenty-five (12%) of 204 patients with AED, three (2%) with MERS, and one (1%) with ADEM were preterm or low birth weight. The high prevalence of seizure disorders suggests that seizure susceptibility is an important predisposing factor in AED. Premature birth also has an impact on the development of AED. Caution is required regarding the development of AE in patients with chronic seizure disorders or premature birth. [ABSTRACT FROM AUTHOR]

Key Clinical Message Concurrent recurrence of acute disseminated encephalomyelitis (ADEM) and poststreptococcal glomerulonephritis (PSGN) in a thalassemia intermedia patient is rare and underscores the complexity of autoimmune disorders. This case emphasizes the importance of considering ADEM in the differential diagnosis of children presenting with PSGN accompanied by neurological symptoms. Abstract Post‐streptococcal glomerulonephritis (PSGN) is a common group A streptococcal (GAS) infection sequela. The pathophysiology of PSGN involves immune complex deposition, with type 3 hypersensitivity reaction triggered by GAS. Certain neurological conditions may also arise following a GAS infection, possibly due to molecular mimicry in the brain, a pathophysiology similar to rheumatic fever, another common sequel of GAS infection. We present the case of a child with β‐thalassemia intermedia who exhibited the classic triad (edema, hypertension, hematuria) of PSGN along with neurological manifestations, including a low glasgow coma scale (GCS) score and seizures. Magnetic resonance imaging (MRI) of the brain indicated changes consistent with acute disseminated encephalomyelitis (ADEM). Initially treated with methylprednisolone, the patient eventually received intravenous immunoglobulin (IVIG) due to lack of response. The patient had a good outcome, with complete resolution of all symptoms and no residual neurological deficits. This case underscores the importance of considering ADEM in the differential diagnosis for patients presenting with neurological signs and symptoms following a recent throat infection with GAS. Furthermore, given the increased risk of infection in thalassemia, patients with thalassemia who have a throat infection and neurological symptoms should be evaluated for the possible presence of ADEM.

Acute disseminated encephalomyelitis (ADEM) is an autoimmune inflammatory demyelinating disease of the central nervous system (CNS) involving the spinal cord and white matter of the brain, usually affecting children and young adults after vaccination or an infection. Neuropsychiatric manifestations are common but are less frequently reported in literature. This case details a young man with no previous comorbidities, who presented with a range of neuropsychiatric manifestations including blank stares, catatonic-like symptoms, selective mutism, irritability, aggression, and visual hallucination. Magnetic resonance imaging showed focal abnormal signal intensity at the splenium of the corpus callosum, notably mildly hypointense on T1W, hyperintense on T2W/FLAIR with restricted diffusion on ADC/DWI. Minimal patchy enhancement was noted postcontrast. Subtle T2/FLAIR hyperintensities were also seen involving the right medial thalamus. He was diagnosed with monophasic ADEM and initiated on intravenous methylprednisolone 1 g daily; however, he developed potential steroid-induced psychosis and was subsequently changed to orodispersible olanzapine and intravenous immunoglobulin (IVIG) 0.4 g/kg/h for 5 days to which he responded satisfactorily. This case report highlights the importance of treating ADEM with steroids and immunoglobulins as it can result in high psychiatric and physical morbidity and mortality if untreated early.

Tumefactive demyelinating lesions (TDL) are a rare occurrence among inflammatory demyelinating diseases of the central nervous system, distinguished by tumor-like lesions exceeding 2 cm in diameter. While various etiologies have been associated with TDL, only a limited number of case reports document the coexistence of acute disseminated encephalomyelitis (ADEM) and TDL. Here, we present the case of a female diagnosed with dengue fever two weeks prior, who subsequently developed left hemiparesis and encephalopathy. Both her brain magnetic resonance imaging (MRI) and clinical course align with the characteristics of tumefactive ADEM. [ABSTRACT FROM AUTHOR]

• We have identified an association between ChAdOx1 nCoV-19 and an increased risk of Acute Disseminated Encephalomyelitis incident admissions. • Dose-specific analysis suggests that dose 1 of ChAdOx1 nCoV-19 may be associated with an increased risk of both Acute Disseminated Encephalomyelitis and Transverse Myelitis incident admissions. • These findings build upon the recently published Global Vaccine Data Network study that suggested an excess of Acute Disseminated Encephalomyelitis and Transverse Myelitis following adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines compared to historically expected background rates. • This study demonstrates the value of the Global Vaccine Data Network collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare potential associations, as well as the value of linked population level datasets such as VSHL able to rapidly explore associations that are identified. Acute Disseminated Encephalomyelitis (ADEM) and Transverse Myelitis (TM) are within the group of immune mediated disorders of acquired demyelinating syndromes. Both have been described in temporal association following various vaccinations in case reports and case series and have been evaluated in observational studies. A recent analysis conducted by The Global Vaccine Data Network (GVDN) observed an excess of ADEM and TM cases following the adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines, compared with historically expected background rates from prior to the pandemic. Further epidemiologic studies were recommended to explore these potential associations. We utilized an Australian vaccine datalink, Vaccine Safety Health-Link (VSHL), to perform a self-controlled case series analysis for this purpose. VSHL was selected for this analysis as while VSHL data are utilised for GVDN association studies, they were not included in the GVDN observed expected analyses. The VSHL dataset contains vaccination records sourced from the Australian Immunisation Register, and hospital admission records from the Victorian Admitted Episodes Dataset for 6.7 million people. These datasets were used to determine the relative incidence (RI) of G040 (ADEM) and G373 (TM) ICD-10-AM coded admissions in the 42-day risk window following COVID-19 vaccinations as compared to control periods either side of the risk window. We observed associations between ChAdOx1 adenovirus vector COVID-19 vaccination and ADEM (all dose RI: 3.74 [95 %CI 1.02,13.70]) and TM (dose 1 RI: 2.49 [95 %CI: 1.07,5.79]) incident admissions. No associations were observed between mRNA COVID-19 vaccines and ADEM or TM. These findings translate to an extremely small absolute risk of ADEM (0.78 per million doses) and TM (1.82 per million doses) following vaccination; any potential risk of ADEM or TM should be weighed against the well-established protective benefits of vaccination against COVID-19 disease and its complications. This study demonstrates the value of the GVDN collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare outcomes, as well as the value of linked population level datasets, such as VSHL, to rapidly explore associations that are identified. [ABSTRACT FROM AUTHOR]

This article explores the use of therapeutic plasma exchange (TPE) as a treatment for neuroimmunological disorders in children. The study found that TPE was effective and well-tolerated, with a partial response observed in most cases. However, despite positive clinical response, a high rate of neurological sequelae was observed. The article emphasizes the need for further research on the efficacy and safety of TPE in pediatric patients with neurological disorders. It also discusses the use of TPE in specific conditions such as Guillain-Barré syndrome and acute disseminated encephalomyelitis, highlighting the need for more research in these areas as well. Overall, the article suggests that TPE is a promising treatment option but more studies are needed to establish standard therapeutic modalities for pediatric patients. [Extracted from the article]

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare systemic inflammatory disease commonly characterized by histiocyte infiltration in multiple organs, such as the liver, spleen, lymph nodes, bone marrow, and central nervous system. The clinical features of HLH include fever, splenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and elevated blood ferritin levels. HLH is categorized as either primary or secondary. Coronavirus disease 2019 (COVID-19) vaccines may occasionally trigger secondary HLH, which is related to hyperinflammatory syndrome. Case presentation: A 58-year-old woman, previously diagnosed with Graves’ disease, presented with cognitive decline 2 weeks after receiving the first dose of the ChAdOx1 nCoV-19 vaccine. Brain MRI revealed a hyperintense lesion on T2-weighted and fluid-attenuated inversion recovery images in the bilateral subcortical white matter and right periventricular area. Vaccination-associated acute disseminated encephalomyelitis was suspected and methylprednisolone and intravenous immunoglobulin (IVIg) were administered. From the 5th day of IVIg administration, the patient developed fever and pancytopenia. In the findings of bone marrow biopsy, hemophagocytosis was not observed; however, six of the eight diagnostic criteria for HLH-2004 were met, raising the possibility of HLH. Although there was no definitive method to confirm causality, considering the temporal sequence, suspicion arose regarding vaccine-induced HLH. Splenectomy was considered for therapeutic and diagnostic purposes; however, the patient died on the 28th day of hospitalization owing to multiple organ failure. Conclusion: To date, 23 cases of COVID-19 vaccine-related HLH have been reported. Additionally, HLH in COVID-19 patients has been reported in various case reports. To the best of our knowledge, this is the first reported case of central nervous system involvement in HLH related to any type of COVID-19 vaccine. This case suggests that even when there are no systemic symptoms after COVID-19 vaccination, HLH should be considered as a differential diagnosis if brain lesions are suggestive of CNS demyelinating disease. [ABSTRACT FROM AUTHOR]

The neurological manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection are underrecognized. Ischemic stroke and thrombotic complications have been documented in patients with SARS-CoV-2 infection. Acute disseminated encephalomyelitis (ADEM) associated with coronavirus disease 2019 (COVID-19) is rare but can occur; the incidence of COVID-19-associated ADEM is still not clear due to the lack of reporting of cases. ADEM may have atypical stroke-like manifestations, such as hemiparesis, hemiparesthesia and dysarthria. The treatment strategies for ADEM and acute stroke are different. Early identification and prompt management may prevent further potentially life-threatening complications. We report a patient with SARS-CoV-2 infection presenting with stroke-like manifestations. We also make a comparison between demyelinating diseases, COVID-19-associated ADEM and acute stroke. This case can prompt physicians to learn about the clinical manifestations of SARS-CoV-2-associated ADEM. [ABSTRACT FROM AUTHOR]

Saint Louis encephalitis virus (SLEV) infection is an arbovirosis associated with a broad spectrum of neurological complications. We present a case of a 55-year-old man hailing from Manaus, a city situated in the heart of the Amazon Rainforest, who exhibited symptoms of vertigo, tremors, urinary and fecal retention, compromised gait, and encephalopathy 3 weeks following SLEV infection. Neuroaxis MRI revealed diffuse, asymmetric, and poorly defined margins hyperintense lesions with peripheral and ring enhancement in subcortical white matter, as well as severe spinal cord involvement. Serology for SLEV was positive both on serum and cerebrospinal fluid. To the best of our knowledge, the present report is the first to show brain lesions along with myelitis as a post-infectious complication of SLEV infection. [ABSTRACT FROM AUTHOR]

The objective of this study was to look at the clinical outcomes, and to determine the proportion of children with visual recovery after the first demyelinating event of optic neuritis (ON). In this observational study, children with the first clinical event of optic neuritis at an age less than 18 years were evaluated. High-contrast visual acuity, colour vision, Expanded Disability Status Scale (EDSS), Anti-MOG and AQP-4 antibodies were assessed. Of the 55 screened, 45 children (77 eyes), median age-98 months, 30 (67%) bilateral were enrolled. Fifty of 77 eyes (67%) had Snellen visual acuity less than 6/60. Twelve children (27%) were MOG seropositive and 3 had AQP-4 positivity. At median follow up of 35 months, 10 (22%) children had one or more relapses. At follow up, the median (IQR) visual acuity improved from nadir of 2.1 (1–2.7) logMAR to 0 (0–0.18) logMAR and 64/77 eyes (83%) had visual recovery. The diagnosis at last follow up was isolated ON in 39/45 (86.6%), relapsing ON (5, 11%), AQP-4 positive NMOSD (3, 7%), MOG antibody associated demyelination (12, 27%), dual seronegative ON (30,67%) and Multiple sclerosis (1, 2%). Most children with first demyelinating event as ON have a monophasic illness. Despite severe acute-phase visual loss, most eyes with ON will recover good visual functions. The risk of AQP-4 disease and multiple sclerosis is low in this group. • This study observational study looked at visual recovery after optic neuritis in children. • At a median follow up of 35 months, 10 (22%) children had one or more relapses. • Eighty-three percent of affected eyes had visual recovery. • At last follow up, MOG antibody associated demyelination was the cause in 12 (27%). • The risk of AQP-4 disease and multiple sclerosis was less than 10%. [ABSTRACT FROM AUTHOR]

Background: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an autoimmune demyelinating disorder of the central nervous system. Methods: Extracted proteins from 34 cerebrospinal fluid (CSF) samples [patients with MOGAD (MOG group, n = 12); healthy controls (HC group, n = 12); patients with MOG seronegative and metagenomics next-generation sequencing-negative inflammatory neurological diseases (IND group, n = 10)] were processed and subjected to label-free quantitative proteomics. Supervised partial least squares-discriminant analysis (PLS-DA) and orthogonal PLS-DA (O-PLS-DA) models were also performed based on proteomics data. Functional analysis of differentially expressed proteins (DEPs) was performed using Gene Ontology, InterPro, and Kyoto Encyclopedia Genes and Genomes. An enzyme-linked immunosorbent assay was used to determine the complement levels in serum from patients with MOGAD. Results: Four hundred and twenty-nine DEPs (149 upregulated and 280 downregulated proteins) were identified in the MOG group compared to the HC group according to the P value and fold change (FC). Using the O-PLS-DA model, 872 differentially abundant proteins were identified with variable importance projection (VIP) scores > 1. Five proteins (gamma-glutamyl hydrolase, cathepsin F, interalpha-trypsin inhibitor heavy chain 5, latent transforming growth factor beta-binding protein 4 and leukocyte-associated immunoglobulin-like receptor 1) overlapping between the top 30 DEPs with top-ranked P value and FC and top 30 proteins in PLS-DA VIP lists were acquired. Functional analysis revealed that the dysregulated proteins in the MOG group were primarily involved in complement and coagulation cascades, cell adhesion, axon guidance, and glycosphingolipid biosynthesis compared to the HC group. Conclusion: The proteomic alterations in CSF samples from children with MOGAD identified in the current study might provide opportunities for developing novel biomarker candidates. [ABSTRACT FROM AUTHOR]

Various vaccines have been developed in response to the SARS-CoV-2 pandemic, and the safety of vaccines has become an important issue. COVID-19 vaccine-related central nervous system inflammatory demyelinating diseases (CNS IDDs) have been reported recently. We present one case of AstraZeneca vaccine-related myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease and a literature review of another 78 patients published from January 2020 to October 2022. Patients were divided into three vaccine types (viral vector, mRNA, and inactivated vaccines) for further analyses. Among 79 patients with COVID-19 vaccine-related CNS IDDs, 49 (62%) cases received viral vector vaccines, 20 (25.3%) received mRNA vaccines, and 10 (12.7%) received inactivated vaccines. Twenty-seven cases (34.2%) were confirmed with autoantibodies, including fifteen patients (19%) with anti-MOG, eleven (13.9%) with anti-aquaporin 4 (AQP4), and one (1.3%) with both antibodies. Significantly, more males developed CNS IDDs post viral vector vaccines compared to mRNA and inactivated vaccines. Patients receiving mRNA vaccines were older than those receiving other types. Furthermore, mRNA and inactivated vaccines correlated more with anti-AQP4 antibodies, while viral vector vaccines showed higher MOG positivity. This research suggests potential associations between COVID-19 vaccine-related CNS IDDs and gender, age, and autoantibodies, contingent on vaccine types. Protein sequence analysis implies similarities between the S protein and AQP4/MOG. Further studies may elucidate the mechanisms of CNS IDDs, aiding vaccine selection for specific types. [ABSTRACT FROM AUTHOR]

Background: We describe a case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in a 16-year-old patient who initially presented with clinical features of septic meningoencephalitis. This case outlines the importance of considering a diagnosis of MOGAD in patients who fail to improve with appropriate antimicrobial therapy or show a positive clinical response to glucocorticoids (often used in treatment of meningococcal meningitis). We emphasise the importance of recognising that an infectious prodrome can precede MOGAD. Case description: A 16-year-old male was admitted with vomiting, fever, headache, photophobia and altered mental state. He was treated for meningoencephalitis with initial clinical improvement. Lumbar puncture findings were suggestive of viral meningoencephalitis. During admission the patient went through several periods of transient clinical and biochemical improvement, alternating with periods of symptomatic relapse. On day 17 of admission, he was transferred to a tertiary centre for suspected autoimmune disseminated meningoencephalitis (ADEM) and two days later, he suffered a catastrophic neurological decline with new dysarthria, dysphagia, aphasia, horizontal nystagmus and facial paralysis. He made a remarkable neurological recovery after commencing treatment with IV immunoglobulin, IV methylprednisolone and plasma exchange, with complete resolution of symptoms. Conclusion: MOGAD can run a variable course and present soon after a central nervous system infection, making the diagnosis more challenging. Nonetheless, patients can achieve a full neurological recovery with early recognition, diagnosis and treatment of this rare entity.

Acute disseminated encephalomyelitis (ADEM) is a severe acute inflammatory process of the Central Nervous System (CNS) consisting of multiple foci of perivascular demyelination. It is a relatively frequent complication of infections and immunization among children. Systemic lupus Erythematosus (SLE), is a chronic autoimmune disease, of unknown etiology, that touches multiple organs simultaneously or additionally, with serious morbidity and mortality. More frequent in females, it can be associated with other auto-immune diseases. ADEM is rarely reported as the initial presentation of SLE, even rarer in the pediatric population. We present one case of a 13 years old girl with a history of ADEM, who was diagnosed with SLE eight months later.

Abstract Background The application of evoked potentials (EPs) to the diagnosis of acute disseminated encephalomyelitis (ADEM ) has not been investigated in detail. The aim of this study, therefore, was to analyze the value of multimodal EPs in the early diagnosis of pediatric ADEM. Methods This was a retrospective study in which we enrolled pediatric ADEM patients and controls (Cs) from neurology units between 2017 and 2021. We measured indices in patients using brainstem auditory evoked potentials (BAEPs), visual evoked potentials (VEPs) and somatosensory evoked potentials (SEPs), and then we analyzed their early diagnostic value in ADEM patients. Results The mean age of the ADEM group was 6.15 ± 3.28 years (range,1–12 years) and the male/female ratio was 2.1:1 The mean age of the Cs was 5.97 ± 3.40 years (range,1–12 years) and the male/female ratio was 1.3:1. As we used magnetic resonance imaging (MRI) as the diagnostic criterion, the sensitivity, specificity, and accuracy (κ was 0.88) of multimodal EPs were highly consistent with those of MRI; and the validity could be ranked in the following order with respect to the diagnosis of ADEM: multimodal Eps > single SEP > single VEP > single BAEP. Of 34 patients with ADEM, abnormalities in multimodal EPs were 94.12%, while abnormalities in single VEPs, BAEPs and SEPs were 70.59%,64.71%and 85.3%, respectively. We noted significant differences between single VEP/BAEPs and multimodal EPs (χ2 = 6.476/8.995,P = 0.011/0.003). Conclusions The combined application of multimodal EPs was superior to BAEPs, VEPs, or SEPs alone in detecting the existence of central nerve demyelination, and we hypothesize that these modalities will be applicable in the early diagnosis of ADEM.

Acute disseminated encephalomyelitis (ADEM) is an acute and rapidly progressive auto-immune demyelinating disorder in the central nervous system. It is a rare disease but is more frequently observed in the pediatric population. We report a case of a monophasic postvaccination ADEM, which presented with paraparesis associated with fever. It showed a favorable evolution under corticosteroids, without recurrence after 3 years of follow-up. The diagnosis was established due to the postvaccination context and the MRI abnormalities characteristics. This case prompted a general discussion about the etiologies of myelin insults in children, especially demyelinating disorders, by shedding the light on their MRI features.

In recent years, there is growing evidence of associations between antibodies against myelin oligodendrocyte glycoprotein (MOG) and several phenotypes of acute inflammatory demyelinating diseases such as acute disseminated encephalomyelitis (ADEM), optic neuritis, brainstem, and cerebral cortical encephalitis, called MOG antibody associated disorders (MOGAD). Monophasic course is known in about half of cases especially in pediatric onset ADEM and optic neuritis, mainly in cases with transient positivity of MOG antibody. Pathological features of MOGAD are considered as acute demyelinating lesions with CD4 dominant cell infiltrations, the deposition of humoral immunity, perivascular inflammation and perivenous demyelination, which is distinct from multiple sclerosis. Now the diagnosis of MOGAD is based on the international panel criteria of MOGAD launched in 2023, which the diagnostic frameworks are three parts, including MOGAD‐specific clinical features, MOG antibody positivity, and the exclusion of other diseases. The prognosis of MOGAD patients is considered relatively mild, but the problem is refractory relapsing cases. For its prevention, there are no approved drugs, but oral tapering corticosteroids, immunosuppressants such as azathioprine and mycophenolic mofetil, rituximab, and the maintenance intravenous immunoglobulin are recommended, and now there are a few clinical trials of promising biological drugs already approved in other neurological disorders. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder that typically follows an infection or recent vaccination. Symptoms such as encephalopathy and focal neurological deficits appear weeks after the initial illness, leading to swift and progressive neurological decline. While ADEM in the brain has been well documented, reports of ADEM, specifically in the spinal cord, are relatively limited. A 58-year-old male presented with rapidly progressive bilateral lower extremity tingling, numbness, and mild gait disturbance approximately two days prior to visiting the emergency room. Spinal magnetic resonance imaging revealed a diffuse, longitudinal, high-signal lesion with mild enlargement of the conus and proximal cauda equina. The lesions were predominantly localized in the distal conus and cauda equina, and serial electrodiagnostic studies showed that the lesions progressed toward the proximal conus in tandem with symptom evolution and lacked clear lateralization. The patient was subsequently treated with high-dose steroids for seven days (intravenous methylprednisolone, 1 mg/kg). The patient's lower extremity weakness gradually improved and he was able to walk independently under supervision three weeks after symptom onset. In this case of spinal ADEM in a middle-aged adult, high-dose steroid treatment led to outstanding neurological recovery from both the initial occurrence and subsequent attacks. [ABSTRACT FROM AUTHOR]

Wernicke encephalopathy is a neuropsychiatric syndrome characterized by three main symptoms: oculomotor disturbances, cerebellar ataxia, and psychiatric disturbances. The condition is associated with a high mortality and morbidity rate. Wernicke encephalopathy is most commonly seen in adolescent children presenting with a vitamin B1 deficiency. Thiamine deficiency may also cause polyneuritis syndrome, with or without the aforementioned symptoms. The condition is characterized by sensory-motor impairments in a symmetrical pattern, dysarthria, and paresis or even paralysis of the lower limbs. This report focuses on an adolescent case presenting acute oculomotor paresis, nystagmus, leg weakness, impaired gait, decreased deep tendon reflexes, cognitive impairment, and a history of recurrent vomiting, prolonged starvation, and eating behaviour disorders. The magnetic resonance imaging scan reveals symmetrical pathological foci of increased intensity in T2 in the periaqueductal region, at the Magendie’s central aperture. The patient displays a mixed motor-sensory polyneuropathic syndrome affecting both lower limbs, primarily of the axonopathy type, based on electroneuromyography data. Positive outcomes such as restored eyeball movement, enhanced gait, increased muscle strength in the lower legs and feet, and better management of sensory disorders have occurred due to thiamine treatment.

INTRODUCTION: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetically determined and hereditary cerebral small vessel disease caused by mutations in the NOTCH3 gene. The onset is in the 3rd and 4th decade of life and is preceded by leukodegenerative changes, which are best visualized by magnetic resonance imaging (MRI). The prevalence of CADASIL is estimated at 2 to 5 people in 100,000. The clinical picture is heterogenous. The main clinical features include recurrent subcortical ischemic events, mood disturbances, progressive cognitive impairment and migraines with aura. Despite the growing recognition of CADASIL, this disease is still a diagnostic challenge. CASE REPORT: A 60-year-old man was referred to the genetic clinic for suspected CADASIL. The initial clinical symptoms probably occurred at the age of 40 in the form of radiological changes. The MRI performed then revealed periventricular zones of a T2 hyperintense signal. Based on follow-up MRIs, the patient was diagnosed with multiple sclerosis or acute disseminated encephalomyelitis. At the age of 58, following a seizure attack he was admitted to hospital and a performed MRI that revealed periventricular white matter degeneration and lacunes. CADASIL was suspected to be the cause. The diagnosis was confirmed by molecular testing. A pathogenic variant NM_000435: c.268C > T (p.Arg90Cys) was detected. CONCLUSIONS: It is relevant to pay attention to radiological findings and family history in the diagnostic process of a patient presenting symptoms which may suggest CADASIL to differentiate this genetic condition from demyelinating diseases.

Acute disseminated encephalomyelitis is an acute autoimmune demyelinating disease that manifests as a widespread polyfocal asymmetric lesions of the central nervous system and, in some cases, optic nerves with the formation of corresponding neurological symptoms. This lecture presents basic information about acute disseminated encephalomyelitis with consideration of 5 clinical cases demonstrating the variety of manifestations of this disease in the practice of a neurologist.

Abstract Background Acute disseminated encephalomyelitis (ADEM) is an extremely rare complication of COVID-19 vaccination with very few reports worldwide. Concomitant peripheral nervous tissue involvement in ADEM is very uncommon. Case presentation We report the case of a 52 year aged lady who developed headache and focal neurological deficits after 10 days of COVID-19 vaccination. Her evaluation suggested ADEM with optic neuritis and mononeuritis multiplex. She responded to pulse methylprednisolone therapy. Conclusions COVID-19 vaccine may be associated with ADEM, optic neuritis and concurrent peripheral nervous system inflammation in rare instances.

After the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) epidemic emerged, the virus spread rapidly worldwide, and outbreaks continued to occur intermittently. Here, we present the case of a 5-year-old boy with acute disseminated encephalomyelitis (ADEM) and initial symptoms of dysphoria and pain in the right lower extremity. Around the time of this episode, the patient exhibited no fever or respiratory symptoms. Brain magnetic resonance imaging (MRI) revealed multiple T2-weighted image/fluid-attenuated inversion recovery high-signal areas bilaterally subcortical to the deep white matter, corpus callosum, and bilateral basal ganglia. MRI of the cervical and thoracic regions indicated a long lesion with continuous T2WI high signal intensity in the central gray matter. Serum aquaporin-4 antibody and serum myelin oligodendrocyte glycoprotein antibody tests were negative and positive, respectively. A polymerase chain reaction test using nasopharyngeal swab fluid upon admission was positive for SARS-CoV-2. Patients with severe coronavirus disease 2019 (COVID-19) in the acute phase may show central nervous system symptoms. There have been no previous reports of ADEM in the subacute phase of COVID-19, lacking symptoms in the acute phase, as in the present case. Notably, ADEM can develop in the subacute phase of asymptomatic COVID-19 infection.

Encephalomyeloradiculoneuropathy (EMRN) is characterized by progressive neurological symptoms in the central and peripheral nervous systems. The autoantibodies against neutral sphingolipids are disease-specific antibodies against EMRN. Although adults with EMRN typically present with symptoms of peripheral nervous system involvement, the symptoms in pediatric patients are not well understood. A 4-year-old boy was admitted to our hospital on the 10th day of fever due to poor oral intake and hyponatremia. The day after admission, he developed seizures and impaired consciousness and was transferred to our hospital. When he arrived at our hospital, he experienced disturbances in consciousness, neck rigidity, and opisthotonus. MRI of the head revealed scattered white matter lesions and pleocytosis in the cerebrospinal fluid (CSF). During treatment with intravenous methylprednisolone (IVMP), the patient developed diminished deep tendon reflexes in the lower extremities four days later, with no improvement in cervical stiffness or opisthotonos. Additional evaluations revealed enlarged cerebral white matter lesions on brain MRI, cauda equina enhancement on MRI of the spinal cord, axonal neuropathy in the bilateral tibial nerves, and positive anti-neutral glycosphingolipid (GSL) antibodies in both serum and CSF. Intensive immunomodulatory therapy, and neurorehabilitation, led to substantial neurological recovery within three months of onset. Pediatric antineutral GSL antibody-positive EMRN may initially present with extensive cerebral white matter lesions and delayed onset of peripheral radiculoneuropathy. Our case extends the disease spectrum of EMRN and may aid in the early diagnosis of EMRN in the pediatric population. [ABSTRACT FROM AUTHOR]

Introduction: Deep brain stimulation (DBS) is an established and effective therapy for movement disorders. Here, we present a case of secondary myoclonus-dystonia syndrome following acute disseminated encephalomyelitis (ADEM) in childhood, which was alleviated by DBS. Using a patient-specific connectome analysis, we sought to characterise the fibres and circuits affected by stimulation. Case report: We report a case of a 20-year-old man with progressive dystonia, myoclonic jerks, and impaired concentration following childhood ADEM. Motor assessments utilising the Unified Myoclonus Rating Scale (UMRS) and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) revealed a greater improvement in dystonia compared to myoclonus following adjustments of DBS parameters. These adjustments were based on visualisation of electrode position and volume of tissue activated (VTA) 3 years after surgery. A patient-specific connectome analysis using the VTA as a region of interest revealed fibre tracts connecting to the cerebello-thalamo-cortical network and the superior frontal gyrus in addition to basal ganglia circuits as particularly effective. Conclusion: Globus pallidus internus (GPi) DBS shows promise as a treatment for secondary myoclonus-dystonia syndromes. Personalised structural considerations, tailored to individual symptoms and clinical characteristics, can provide significant benefits. Patient-specific connectome analysis, specifically, offers insights into the structures involved and may enable a favourable treatment response. [ABSTRACT FROM AUTHOR]

Diagnosis of lymphomatosis cerebri (LC) is usually delayed because of its rarity and the need for pathological confirmation. The association of LC with humoral immunity has scarcely been reported. Herein, we present a woman with a 2‐week history of dizziness and gait ataxia, followed by diplopia, altered mental status, and spasticity of all limbs. Magnetic resonance imaging (MRI) of the brain showed multifocal lesions involving bilateral subcortical white matter, deep gray structures, and brainstem. Oligoclonal bands and anti‐N‐methyl‐D‐aspartate receptor (NMDAR) antibodies were present in cerebrospinal fluid (CSF) twice. She was initially treated with methylprednisolone but still worsening. A stereotactic brain biopsy confirmed the diagnosis of LC. This is a report on the distinctive coexistence of the rare CNS lymphoma variant and the anti‐NMDAR antibody. [ABSTRACT FROM AUTHOR]

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder of the central nervous system that is usually triggered by infections. We aimed to determine the temporal trends in the incidence of ADEM before and during the pandemic and their correlation with viral epidemiology. We conducted a nationwide, population-based, retrospective, ADEM cohort study by using the Health Insurance Review and Assessment Service database. New-onset ADEM was defined as a patient (age <19 years) who was hospitalized with a diagnostic code of G04.0, G36.8, and G36.9 and a prescription of intravenous methylprednisolone. The National Infectious Disease Surveillance System was used to collect the nationwide viral epidemics. A total of 185 new-onset pediatric ADEM cases were included. The mean patient age was 7.0 ± 4.9 years. The incidence of ADEM was 0.34 to 0.48 of 100,000 persons per year before the pandemic, which dropped to 0.22 of 100,000 persons per year during the first pandemic year. The risk of ADEM occurrence was approximately 1.74% higher during the prepandemic years compared with the first pandemic year (odds ratio = 1.017, P = 0.009). There was a weak positive correlation between acute respiratory viral infection and ADEM incidence (r = 0.28, P = 0.03). This study demonstrates how infection control during the early coronavirus disease 2019 (COVID-19) pandemic influenced the incidence of ADEM. The low incidence of ADEM in the early COVID-19 pandemic may be related to the decline in acute respiratory viral infections, which are potential triggers of ADEM. [ABSTRACT FROM AUTHOR]

The involvement of the central nervous system in COVID-19 is now widely known. Acute disseminated encephalomyelitis (ADEM) as a presentation of COVID-19 is sparse. We would like to present clinical and radiological features of a COVID-19 adult patient and evocative of virus-associated ADEM. During the early stages of the COVID-19 epidemic, the patient succumbs to his illness despite all available treatments.

Various vaccines have been developed in response to the SARS-CoV-2 pandemic, and the safety of vaccines has become an important issue. COVID-19 vaccine-related central nervous system inflammatory demyelinating diseases (CNS IDDs) have been reported recently. We present one case of AstraZeneca vaccine-related myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease and a literature review of another 78 patients published from January 2020 to October 2022. Patients were divided into three vaccine types (viral vector, mRNA, and inactivated vaccines) for further analyses. Among 79 patients with COVID-19 vaccine-related CNS IDDs, 49 (62%) cases received viral vector vaccines, 20 (25.3%) received mRNA vaccines, and 10 (12.7%) received inactivated vaccines. Twenty-seven cases (34.2%) were confirmed with autoantibodies, including fifteen patients (19%) with anti-MOG, eleven (13.9%) with anti-aquaporin 4 (AQP4), and one (1.3%) with both antibodies. Significantly, more males developed CNS IDDs post viral vector vaccines compared to mRNA and inactivated vaccines. Patients receiving mRNA vaccines were older than those receiving other types. Furthermore, mRNA and inactivated vaccines correlated more with anti-AQP4 antibodies, while viral vector vaccines showed higher MOG positivity. This research suggests potential associations between COVID-19 vaccine-related CNS IDDs and gender, age, and autoantibodies, contingent on vaccine types. Protein sequence analysis implies similarities between the S protein and AQP4/MOG. Further studies may elucidate the mechanisms of CNS IDDs, aiding vaccine selection for specific types.

The neurological manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection are underrecognized. Ischemic stroke and thrombotic complications have been documented in patients with SARS-CoV-2 infection. Acute disseminated encephalomyelitis (ADEM) associated with coronavirus disease 2019 (COVID-19) is rare but can occur; the incidence of COVID-19-associated ADEM is still not clear due to the lack of reporting of cases. ADEM may have atypical stroke-like manifestations, such as hemiparesis, hemiparesthesia and dysarthria. The treatment strategies for ADEM and acute stroke are different. Early identification and prompt management may prevent further potentially life-threatening complications. We report a patient with SARS-CoV-2 infection presenting with stroke-like manifestations. We also make a comparison between demyelinating diseases, COVID-19-associated ADEM and acute stroke. This case can prompt physicians to learn about the clinical manifestations of SARS-CoV-2-associated ADEM.