Publications by authors named "Jarius S"

Background: Recurrent attacks in neuromyelitis optica spectrum disorders (NMOSDs) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can lead to severe disability. We aimed to analyse the real-world use of immunotherapies in patients with NMOSD and MOGAD, focusing on changes in treatment strategies, effects on attack rates (ARR) and risk factors for attacks.

Methods: This longitudinal registry-based cohort study included 493 patients (320 with aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive NMOSD (65%), 44 with AQP4-IgG seronegative NMOSD (9%) and 129 MOGAD (26%)) with 1247 treatments from 19 German and one Austrian centre from the registry of the neuromyelitis optica study group (NEMOS).

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Objective: In this multicentric study, we were interested in the vision-related quality of life and its association with visual impairment in neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in comparison to multiple sclerosis (MS) and healthy controls.

Methods: We analysed extracted data from the German NEMOS registry including National Eye Institute Visual Function Questionnaire (NEI-VFQ) scores, high and low contrast visual acuity (HCVA, LCVA), visually evoked potentials (VEP) and the scores for the expanded disability status scale (EDSS) and other neurological tests which assessed their disease-related impairment. The mean follow-up time of our patients was 1.

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Article Synopsis
  • - The study investigates the relationship between the Epstein-Barr virus (EBV) and multiple sclerosis (MS) by analyzing the presence of antibodies against EBV and other common microbes in patients with MS, revealing a universal EBV seroprevalence among participants.
  • - A total of 50 MS patients were tested for antibodies in their cerebrospinal fluid (CSF) and serum, finding that while all patients were EBV positive, the production of specific antibodies in the CSF was significantly lower for EBV compared to other viruses like measles and VZV.
  • - The results indicate that even though almost all MS patients have been exposed to EBV, the actual production of antibodies against EBV in the central
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Background: Incomplete attack remission is the main cause of disability in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Apheresis therapies such as plasma exchange and immunoadsorption are widely used in neuroimmunology. Data on apheresis outcomes in MOGAD attacks remain limited.

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Article Synopsis
  • Eculizumab (ECU) has shown effectiveness in preventing attacks in patients with aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorders (NMOSDs) during a retrospective analysis in clinical settings between 2014 and 2022.
  • A total of 52 patients were studied, with 88% being attack-free during treatment, and the annualized attack rate significantly decreased from 1.0 to 0.
  • While common side effects included serious infections, five patients died from various complications, indicating a need for careful monitoring during long-term ECU therapy.
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Background: Data on cognition in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are limited to studies with small sample sizes. Therefore, we aimed to analyse the extent, characteristics and the longitudinal course of potential cognitive deficits in patients with MOGAD.

Methods: The CogniMOG-Study is a prospective, longitudinal and multicentre observational study of 113 patients with MOGAD.

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Epstein-Barr virus (EBV) infection has long been associated with the development of multiple sclerosis (MS). MS patients have elevated titers of EBV-specific antibodies in serum and show signs of CNS damage only after EBV infection. Regarding CD8+ T-cells, an elevated but ineffective response to EBV was suggested in MS patients, who present with a broader MHC-I-restricted EBV-specific T-cell receptor beta chain (TRB) repertoire compared to controls.

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Background: Myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) is an autoinflammatory disease of the central nervous system. MOGAD often follows a relapsing course that can lead to severe disability, but monophasic disease is possible as well. Currently, there is an unmet clinical need for disease activity biomarkers in MOGAD.

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Article Synopsis
  • Myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM) is an important condition to differentiate from multiple sclerosis and neuromyelitis optica, with new diagnostic criteria emphasizing specific antibody testing.
  • This study evaluated the effectiveness of using MOG-IgG3-specific testing in addition to standard tests for detecting MOG antibodies in patients previously identified as having MOG-EM.
  • Results showed that MOG-IgG3 could improve the sensitivity of tests, as 52% of patients still displayed positive MOG-IgG3 results, indicating it may be a crucial subclass in diagnosing MOG-EM, despite its absence in healthy controls or
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  • Recent studies suggest that myelin oligodendrocyte glycoprotein antibody-associated disease could be a rare complication linked to either SARS-CoV-2 infection or vaccination.
  • The research aims to determine if there's an immune response overlap between SARS-CoV-2 proteins and myelin oligodendrocyte glycoprotein, which could explain the condition's occurrence.
  • Serum samples from various groups with different histories of SARS-CoV-2 infection and neurological symptoms were analyzed to assess antibody responses against both myelin oligodendrocyte glycoprotein and SARS-CoV-2, among other common coronaviruses.
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Article Synopsis
  • The study focused on understanding how disability accumulates in patients with neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD), examining factors like disease duration, attack frequency, and age.* -
  • Data from 483 patients indicated that NMOSD patients reached significant disability milestones much sooner than MOGAD patients, despite similar attack rates; older age at symptom onset was linked to higher disability risk.* -
  • Overall, NMOSD and MOGAD exhibit different patterns of disability progression, with MOGAD being less severe, and recent research has enhanced awareness and treatment strategies, positively impacting patient outcomes.*
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Background: Information on cerebrospinal fluid (CSF) findings in patients with neurological manifestations in post-COVID-19 syndrome is scarce.

Methods: Retrospective evaluation of 84 CSF samples in patients fulfilling post-COVID-19 criteria in two neurological post-COVID-19 outpatient clinics.

Results: In 68% of samples, all CSF parameters were normal.

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Background: Aquaporin-4 immunoglobulin G (AQP4-IgG) antibody-positive neuromyelitis optica spectrum disorders (NMOSD) are frequently associated with other autoimmune disorders, including systemic lupus erythematosus (SLE). Eculizumab (ECU) is a highly effective long-term treatment for NMOSD. However, ECU is known to increase significantly the risk of infection with encapsulated bacteria and sepsis.

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Article Synopsis
  • - This manuscript offers practical advice for treating acute attacks and preventing future episodes of neuromyelitis optica spectrum disorders (NMOSD), an autoimmune disease that severely impacts the central nervous system, primarily affecting vision and mobility.
  • - The study highlights the importance of Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) as a key diagnostic marker and contributor to NMOSD, with recent advancements leading to four new approved preventive therapies: eculizumab, ravulizumab, inebilizumab, and satralizumab, which may replace older treatments.
  • - The Neuromyelitis Optica Study Group (NEMOS) presents a consensus on current NMOSD therapies and practical
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Article Synopsis
  • The term 'neuromyelitis optica spectrum disorders' (NMOSD) encompasses several related conditions, including aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and other syndromes lacking AQP4-IgG.
  • NMOSD is now recognized as a distinct disorder, separate from multiple sclerosis (MS), due to differences in their causes, clinical presentation, treatments, and outcomes.
  • The article series includes updated guidelines for diagnosing NMOSD and differentiating it from similar conditions, such as MOG antibody-associated diseases, as well as recommendations for the latest treatment options available.
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Background: Septins are cytoskeletal proteins with filament forming capabilities, which have multiple roles during cell division, cellular polarization, morphogenesis, and membrane trafficking. Autoantibodies against septin-5 are associated with non-paraneoplastic cerebellar ataxia, and autoantibodies against septin-7 with encephalopathy with prominent neuropsychiatric features. Here, we report on newly identified autoantibodies against septin-3 in patients with paraneoplastic cerebellar ataxia.

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Background: There is limited and inconsistent information on the prevalence of cognitive impairment in neuromyelitis optica spectrum disorders (NMOSD).

Objective: To assess cognitive performance and changes over time in NMOSD.

Methods: This study included data from 217 aquaporin-4-IgG-seropositive (80%) and double-seronegative NMOSD patients.

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Article Synopsis
  • This study examines the inflammatory response in the cerebrospinal fluid (CSF) of COVID-19 patients, finding that the changes are less severe compared to those with herpes simplex virus encephalitis (HSVE).
  • It highlights that inflammatory markers in COVID-19 patients may enter the CSF from the bloodstream rather than being produced in the central nervous system itself, with no significant levels of harmful neuronal autoantibodies detected.
  • Additionally, COVID-19 patients with bacterial superinfection exhibited significantly higher inflammatory markers, and genetic analysis identified 101 linear RNAs present in their CSF.
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Background: Data on the humoral vaccine response in patients on anti-interleukin-6 (IL-6) receptor therapy remain scarce.

Objective: The main objective of our study was to investigate the humoral response after vaccination against SARS-CoV-2 in neuromyelitis optica spectrum disorder (NMOSD)/myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) patients treated with anti-IL-6 receptor therapy. Secondarily, we analyzed relapse activity timely associated with vaccination.

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Background And Objectives: To evaluate the effects of the coronavirus disease 2019 (COVID-19) pandemic on the life of patients with neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated diseases (MOGAD).

Methods: This multicenter, cross-sectional study included data of 187 patients recruited from 19 different German and Austrian Neuromyelitis Optica Study Group (NEMOS) centers between July 2021 and March 2022. The effects of the pandemic on immunotherapeutic treatment and access to care, the possible severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, and the potential effect of vaccination against SARS-CoV-2 on disease incidence and relapse risk were assessed using a patient questionnaire.

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Article Synopsis
  • IgG antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) are linked to myelin oligodendrocyte glycoprotein associated disorders (MOGAD), which may flare up over time, but there's limited info on predicting these relapses.* -
  • This study analyzed 102 MOGAD patients by measuring MOG-IgG titres over time and found that higher titres during remission indicated a greater risk of future relapses.* -
  • The findings suggest that monitoring MOG-IgG levels could help identify patients who might need long-term treatment to prevent relapse, especially those with persistent positivity or high remission titres.*
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Aquaporin-4 antibody-seropositive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD; also termed MOG encephalomyelitis) are autoimmune diseases of the central nervous system. The typical initial manifestations in adult patients are optic neuritis and myelitis. Patients often present with additional involvement of the brain and brainstem, more so in the later stages of the disease.

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Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing, often debilitating neuroinflammatory disease, whose predominant clinical manifestations are longitudinally extensive transverse myelitis and optic neuritis. About 80% of the patients with an NMOSD phenotype have pathogenic autoantibodies against the astrocyte water channel aquaporin-4 (AQP4-IgG). While therapeutic options for NMOSD have greatly expanded in recent years, well-established biomarkers for prognosis or treatment response are still lacking.

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