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1. Correction to: Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management

Author

Kümpfel, Tania, Giglhuber, Katrin, Aktas, Orhan, Ayzenberg, Ilya, Bellmann-Strobl, Judith, Häußler, Vivien, Havla, Joachim, Hellwig, Kerstin, Hümmert, Martin W., Jarius, Sven, Kleiter, Ingo, Klotz, Luisa, Krumbholz, Markus, Paul, Friedemann, Ringelstein, Marius, Ruprecht, Klemens, Senel, Makbule, Stellmann, Jan-Patrick, Bergh, Florian Then, Trebst, Corinna, Tumani, Hayrettin, Warnke, Clemens, Wildemann, Brigitte, and Berthele, Achim

Published
2024
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3. Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management

Author

Kümpfel, Tania, Giglhuber, Katrin, Aktas, Orhan, Ayzenberg, Ilya, Bellmann-Strobl, Judith, Häußler, Vivien, Havla, Joachim, Hellwig, Kerstin, Hümmert, Martin W., Jarius, Sven, Kleiter, Ingo, Klotz, Luisa, Krumbholz, Markus, Paul, Friedemann, Ringelstein, Marius, Ruprecht, Klemens, Senel, Makbule, Stellmann, Jan-Patrick, Bergh, Florian Then, Trebst, Corinna, Tumani, Hayrettin, Warnke, Clemens, Wildemann, Brigitte, and Berthele, Achim

Published
2024
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4. Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) – revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis

Author

Jarius, Sven, Aktas, Orhan, Ayzenberg, Ilya, Bellmann-Strobl, Judith, Berthele, Achim, Giglhuber, Katrin, Häußler, Vivien, Havla, Joachim, Hellwig, Kerstin, Hümmert, Martin W., Kleiter, Ingo, Klotz, Luisa, Krumbholz, Markus, Kümpfel, Tania, Paul, Friedemann, Ringelstein, Marius, Ruprecht, Klemens, Senel, Makbule, Stellmann, Jan-Patrick, Bergh, Florian Then, Tumani, Hayrettin, Wildemann, Brigitte, and Trebst, Corinna

Published
2023
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5. The brain reacting to COVID-19: analysis of the cerebrospinal fluid proteome, RNA and inflammation

Author

Reinhold, Dirk, Farztdinov, Vadim, Yan, Yan, Meisel, Christian, Sadlowski, Henrik, Kühn, Joachim, Perschel, Frank H., Endres, Matthias, Düzel, Emrah, Vielhaber, Stefan, Guttek, Karina, Goihl, Alexander, Venø, Morten, Teegen, Bianca, Stöcker, Winfried, Stubbemann, Paula, Kurth, Florian, Sander, Leif E., Ralser, Markus, Otto, Carolin, Streit, Simon, Jarius, Sven, Ruprecht, Klemens, Radbruch, Helena, Kjems, Jørgen, Mülleder, Michael, Heppner, Frank, and Körtvelyessy, Peter

Published
2023
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7. Two new cases of anti-Ca (anti-ARHGAP26/GRAF) autoantibody-associated cerebellar ataxia

Author

Jarius Sven, Martínez-García Pedro, Hernandez Adelaida León, Brase Jan Christoph, Borowski Kathrin, Regula Jens Ulrich, Meinck Hans Michael, Stöcker Winfried, Wildemann Brigitte, and Wandinger Klaus-Peter

Subjects
Autoimmune cerebellar ataxia, Purkinje cells, Autoimmunity, Autoantibodies, RhoGTPase-activating protein 26 (ARHGAP26), GTPase regulator associated with focal adhesion kinase pp125 (GRAF), Oligophrenin-1-like protein, Paraneoplastic, Ovarian cancer, Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract Recently, we discovered a novel serum and cerebrospinal fluid (CSF) autoantibody (anti-Ca) to Purkinje cells in a patient with autoimmune cerebellar ataxia (ACA) and identified the RhoGTPase-activating protein 26 (ARHGAP26; alternative designations include GTPase regulator associated with focal adhesion kinase pp125, GRAF, and oligophrenin-1-like protein, OPHN1L) as the target antigen. Here, we report on two new cases of ARHGAP26 autoantibody-positive ACA that were first diagnosed after publication of the index case study. While the index patient developed ACA following an episode of respiratory infection with still no evidence for malignancy 52 months after onset, neurological symptoms heralded ovarian cancer in one of the patients described here. Our finding of anti-Ca/anti-ARHGAP26 antibodies in two additional patients supports a role of autoimmunity against ARHGAP26 in the pathogenesis of ACA. Moreover, the finding of ovarian cancer in one of our patients suggests that anti-Ca/anti-ARHGAP26-positive ACA might be of paraneoplastic aetiology in some cases. In conclusion, testing for anti-Ca/anti-ARHGAP26 should be included in the diagnostic work-up of patients with ACA, and an underlying tumour should be considered in patients presenting with anti-Ca/ARHGAP26 antibody-positive ACA.

Published
2013
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8. Usefulness of antibody index assessment in cerebrospinal fluid from patients negative for total-IgG oligoclonal bands

Author

Jarius Sven, Eichhorn Peter, Wildemann Brigitte, and Wick Manfred

Subjects
Intrathecal IgG synthesis, Antibody index, Cerebrospinal fluid, Oligoclonal bands, Herpes simplex virus, Cytomegalovirus, Varicella zoster virus, Measles virus, Rubella virus, Borrelia burgdorferi, Multiple sclerosis, MRZ reaction, Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract Background Testing for cerebrospinal fluid (CSF)-restricted oligoclonal bands (OCB) by isoelectric focusing is used to detect intrathecally produced total IgG. By contrast, antibody indices (AI) are assessed to test for intrathecally produced antigen-specific IgG. A number of previous cases reports have suggested that AI testing might be more sensitive than OCB testing in detecting intrathecal IgG synthesis. Findings Here we report on 21 patients with positive AI for either herpes simplex virus, varicella zoster virus, cytomegalovirus, measles virus, rubella virus, or Borrelia burgdorferi in the absence of total-IgG OCB and, accordingly, in the presence of a normal total-IgG CSF/serum ratio. Conclusion Our findings indicate that AI testing should not generally be omitted in OCB-negative patients and provide a rationale for systematic and prospective studies on the comparative sensitivity and specificity of AI and total-IgG OCB testing in infectious and other diseases of the CNS.

Published
2012
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9. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients

Author

Jarius Sven, Ruprecht Klemens, Wildemann Brigitte, Kuempfel Tania, Ringelstein Marius, Geis Christian, Kleiter Ingo, Kleinschnitz Christoph, Berthele Achim, Brettschneider Johannes, Hellwig Kerstin, Hemmer Bernhard, Linker Ralf A, Lauda Florian, Mayer Christoph A, Tumani Hayrettin, Melms Arthur, Trebst Corinna, Stangel Martin, Marziniak Martin, Hoffmann Frank, Schippling Sven, Faiss Jürgen H, Neuhaus Oliver, Ettrich Barbara, Zentner Christian, Guthke Kersten, Hofstadt-van Oy Ulrich, Reuss Reinhard, Pellkofer Hannah, Ziemann Ulf, Kern Peter, Wandinger Klaus P, Bergh Florian, Boettcher Tobias, Langel Stefan, Liebetrau Martin, Rommer Paulus S, Niehaus Sabine, Münch Christoph, Winkelmann Alexander, Zettl U Uwe K, Metz Imke, Veauthier Christian, Sieb Jörn P, Wilke Christian, Hartung Hans P, Aktas Orhan, and Paul Friedemann

Subjects
Neuromyelitis optica, Devic disease, Devic syndrome, longitudinally extensive transverse myelitis, recurrent optic neuritis, NMO-IgG, aquaporin-4 (AQP4) antibody, epidemiology, clinical features, magnetic resonance imaging, cerebrospinal fluid, Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract Background The diagnostic and pathophysiological relevance of antibodies to aquaporin-4 (AQP4-Ab) in patients with neuromyelitis optica spectrum disorders (NMOSD) has been intensively studied. However, little is known so far about the clinical impact of AQP4-Ab seropositivity. Objective To analyse systematically the clinical and paraclinical features associated with NMO spectrum disorders in Caucasians in a stratified fashion according to the patients' AQP4-Ab serostatus. Methods Retrospective study of 175 Caucasian patients (AQP4-Ab positive in 78.3%). Results Seropositive patients were found to be predominantly female (p < 0.0003), to more often have signs of co-existing autoimmunity (p < 0.00001), and to experience more severe clinical attacks. A visual acuity of ≤ 0.1 during acute optic neuritis (ON) attacks was more frequent among seropositives (p < 0.002). Similarly, motor symptoms were more common in seropositive patients, the median Medical Research Council scale (MRC) grade worse, and MRC grades ≤ 2 more frequent, in particular if patients met the 2006 revised criteria (p < 0.005, p < 0.006 and p < 0.01, respectively), the total spinal cord lesion load was higher (p < 0.006), and lesions ≥ 6 vertebral segments as well as entire spinal cord involvement more frequent (p < 0.003 and p < 0.043). By contrast, bilateral ON at onset was more common in seronegatives (p < 0.007), as was simultaneous ON and myelitis (p < 0.001); accordingly, the time to diagnosis of NMO was shorter in the seronegative group (p < 0.029). The course of disease was more often monophasic in seronegatives (p < 0.008). Seropositives and seronegatives did not differ significantly with regard to age at onset, time to relapse, annualized relapse rates, outcome from relapse (complete, partial, no recovery), annualized EDSS increase, mortality rate, supratentorial brain lesions, brainstem lesions, history of carcinoma, frequency of preceding infections, oligoclonal bands, or CSF pleocytosis. Both the time to relapse and the time to diagnosis was longer if the disease started with ON (p < 0.002 and p < 0.013). Motor symptoms or tetraparesis at first myelitis and > 1 myelitis attacks in the first year were identified as possible predictors of a worse outcome. Conclusion This study provides an overview of the clinical and paraclinical features of NMOSD in Caucasians and demonstrates a number of distinct disease characteristics in seropositive and seronegative patients.

Published
2012
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10. Complement activating antibodies to myelin oligodendrocyte glycoprotein in neuromyelitis optica and related disorders

Author

Mader Simone, Gredler Viktoria, Schanda Kathrin, Rostasy Kevin, Dujmovic Irena, Pfaller Kristian, Lutterotti Andreas, Jarius Sven, Di Pauli Franziska, Kuenz Bettina, Ehling Rainer, Hegen Harald, Deisenhammer Florian, Aboul-Enein Fahmy, Storch Maria K, Koson Peter, Drulovic Jelena, Kristoferitsch Wolfgang, Berger Thomas, and Reindl Markus

Subjects
Neuromyelitis optica, autoantibodies, myelin oligodendrocyte glycoprotein, aquaporin-4, complement mediated cytotoxicity, biomarker, Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract Background Serum autoantibodies against the water channel aquaporin-4 (AQP4) are important diagnostic biomarkers and pathogenic factors for neuromyelitis optica (NMO). However, AQP4-IgG are absent in 5-40% of all NMO patients and the target of the autoimmune response in these patients is unknown. Since recent studies indicate that autoimmune responses to myelin oligodendrocyte glycoprotein (MOG) can induce an NMO-like disease in experimental animal models, we speculate that MOG might be an autoantigen in AQP4-IgG seronegative NMO. Although high-titer autoantibodies to human native MOG were mainly detected in a subgroup of pediatric acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) patients, their role in NMO and High-risk NMO (HR-NMO; recurrent optic neuritis-rON or longitudinally extensive transverse myelitis-LETM) remains unresolved. Results We analyzed patients with definite NMO (n = 45), HR-NMO (n = 53), ADEM (n = 33), clinically isolated syndromes presenting with myelitis or optic neuritis (CIS, n = 32), MS (n = 71) and controls (n = 101; 24 other neurological diseases-OND, 27 systemic lupus erythematosus-SLE and 50 healthy subjects) for serum IgG to MOG and AQP4. Furthermore, we investigated whether these antibodies can mediate complement dependent cytotoxicity (CDC). AQP4-IgG was found in patients with NMO (n = 43, 96%), HR-NMO (n = 32, 60%) and in one CIS patient (3%), but was absent in ADEM, MS and controls. High-titer MOG-IgG was found in patients with ADEM (n = 14, 42%), NMO (n = 3, 7%), HR-NMO (n = 7, 13%, 5 rON and 2 LETM), CIS (n = 2, 6%), MS (n = 2, 3%) and controls (n = 3, 3%, two SLE and one OND). Two of the three MOG-IgG positive NMO patients and all seven MOG-IgG positive HR-NMO patients were negative for AQP4-IgG. Thus, MOG-IgG were found in both AQP4-IgG seronegative NMO patients and seven of 21 (33%) AQP4-IgG negative HR-NMO patients. Antibodies to MOG and AQP4 were predominantly of the IgG1 subtype, and were able to mediate CDC at high-titer levels. Conclusions We could show for the first time that a subset of AQP4-IgG seronegative patients with NMO and HR-NMO exhibit a MOG-IgG mediated immune response, whereas MOG is not a target antigen in cases with an AQP4-directed humoral immune response.

Published
2011
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11. Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance

Author

Jarius Sven, Franciotta Diego, Paul Friedemann, Ruprecht Klemens, Bergamaschi Roberto, Rommer Paulus S, Reuss Reinhard, Probst Christian, Kristoferitsch Wolfgang, Wandinger Klaus, and Wildemann Brigitte

Subjects
Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract Background In 70-80% of cases, neuromyelitis optica (NMO) is associated with highly specific serum auto-antibodies to aquaporin-4 (termed AQP4-Ab or NMO-IgG). Recent evidence strongly suggests that AQP4-Ab are directly involved in the immunopathogenesis of NMO. Objective To assess the frequency, syndrome specificity, diagnostic relevance, and origin of cerebrospinal fluid (CSF) AQP4-Ab in patients with NMO spectrum disorders (NMOSD). Methods 87 CSF samples from 37 patients with NMOSD and 42 controls with other neurological diseases were tested for AQP4-Ab in a cell based assay using recombinant human AQP4. Twenty-three paired CSF and serum samples from AQP4-Ab seropositive NMOSD patients were further analysed for intrathecal IgG synthesis to AQP4. Results AQP4-Ab were detectable in 68% of CSF samples from AQP4-Ab seropositive patients with NMOSD, but in none of the CSF samples from AQP4-Ab seronegative patients with NMOSD and in none of the control samples. Acute disease relapse within 30 days prior to lumbar puncture, AQP4-Ab serum titres >1:250, and blood-CSF barrier dysfunction, but not treatment status, predicted CSF AQP4-Ab positivity. A positive AQP4-specific antibody index was present in 1/23 samples analysed. Conclusions AQP4-Ab are detectable in the CSF of most patients with NMOSD, mainly during relapse, and are highly specific for this condition. In the cohort analysed in this study, testing for CSF AQP4-Ab did not improve the sensitivity and specificity of the current diagnostic criteria for NMO. The substantial lack of intrathecal AQP4-Ab synthesis in patients with NMOSD may reflect the unique localisation of the target antigen at the blood brain barrier, and is important for our understanding of the immunopathogenesis of the disease.

Published
2010
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12. A new Purkinje cell antibody (anti-Ca) associated with subacute cerebellar ataxia: immunological characterization

Author

Horn Sigrun, Wandinger Klaus P, Jarius Sven, Heuer Heike, and Wildemann Brigitte

Subjects
Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract We report on a newly discovered serum and cerebrospinal fluid (CSF) reactivity to Purkinje cells (PCs) associated with subacute inflammatory cerebellar ataxia. The patient, a previously healthy 33-year-old lady, presented with severe limb and gait ataxia, dysarthria, and diplopia two weeks after she had recovered from a common cold. Immunohistochemical studies on mouse, rat, and monkey brain sections revealed binding of a high-titer (up to 1:10,000) IgG antibody to the cerebellar molecular layer, Purkinje cell (PC) layer, and white matter. The antibody is highly specific for PCs and binds to the cytoplasm as well as to the inner side of the membrane of PC somata, dendrites and axons. It is produced by B cell clones within the CNS, belongs to the IgG1 subclass, and activates complement in vitro. Western blotting of primate cerebellum extract revealed binding of CSF and serum IgG to an 80-97 kDa protein. Extensive control studies were performed to rule out a broad panel of previously described paraneoplastic and non-paraneoplastic antibodies known to be associated with cerebellar ataxia. Screening of >9000 human full length proteins by means of a protein array and additional confirmatory experiments revealed Rho GTPase activating protein 26 (ARHGAP26, GRAF, oligophrenin-1-like protein) as the target antigen. Preadsorption of the patient's serum with human ARHGAP26 but not preadsorption with other proteins resulted in complete loss of PC staining. Our findings suggest a role of autoimmunity against ARHGAP26 in the pathogenesis of subacute inflammatory cerebellar ataxia, and extend the panel of diagnostic markers for this devastating disease.

Published
2010
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15. Serum glial fibrillary acidic protein correlates with retinal structural damage in aquaporin-4 antibody positive neuromyelitis optica spectrum disorder

Author

Lin, Ting-Yi, Schindler, Patrick, Grittner, Ulrike, Oertel, Frederike C., Lu, Angelo, Motamedi, Seyedamirhosein, Yadav, Sunil Kumar, Duchow, Ankelien S., Jarius, Sven, Kuhle, Jens, Benkert, Pascal, Brandt, Alexander U., Bellmann-Strobl, Judith, Schmitz-Hübsch, Tanja, Paul, Friedemann, Ruprecht, Klemens, and Zimmermann, Hanna G.

Published
2022
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19. Impaired response of blood neutrophils to cell-death stimulus differentiates AQP4-IgG-seropositive NMOSD from MOGAD

Author

Schroeder-Castagno, Maria, Del Rio-Serrato, Alba, Wilhelm, Andreas, Romero-Suárez, Silvina, Schindler, Patrick, Alvarez-González, Cesar, Duchow, Ankelien-Solveig, Bellmann-Strobl, Judith, Ruprecht, Klemens, Hastermann, Maria, Grütz, Gerald, Wildemann, Brigitte, Jarius, Sven, Schmitz-Hübsch, Tanja, Paul, Friedemann, and Infante-Duarte, Carmen

Published
2022
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20. Cerebrospinal fluid findings in COVID-19: a multicenter study of 150 lumbar punctures in 127 patients

Author

Jarius, Sven, Pache, Florence, Körtvelyessy, Peter, Jelčić, Ilijas, Stettner, Mark, Franciotta, Diego, Keller, Emanuela, Neumann, Bernhard, Ringelstein, Marius, Senel, Makbule, Regeniter, Axel, Kalantzis, Rea, Willms, Jan F., Berthele, Achim, Busch, Markus, Capobianco, Marco, Eisele, Amanda, Reichen, Ina, Dersch, Rick, Rauer, Sebastian, Sandner, Katharina, Ayzenberg, Ilya, Gross, Catharina C., Hegen, Harald, Khalil, Michael, Kleiter, Ingo, Lenhard, Thorsten, Haas, Jürgen, Aktas, Orhan, Angstwurm, Klemens, Kleinschnitz, Christoph, Lewerenz, Jan, Tumani, Hayrettin, Paul, Friedemann, Stangel, Martin, Ruprecht, Klemens, and Wildemann, Brigitte

Published
2022
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22. Treatment of MOG-IgG-associated disorder with rituximab: An international study of 121 patients

Author

Whittam, Daniel H, Cobo-Calvo, Alvaro, Lopez-Chiriboga, A Sebastian, Pardo, Santiago, Gornall, Matthew, Cicconi, Silvia, Brandt, Alexander, Berek, Klaus, Berger, Thomas, Jelcic, Ilijas, Gombolay, Grace, Oliveira, Luana Micheli, Callegaro, Dagoberto, Kaneko, Kimihiko, Misu, Tatsuro, Capobianco, Marco, Gibbons, Emily, Karthikeayan, Venkatraman, Brochet, Bruno, Audoin, Bertrand, Mathey, Guillaume, Laplaud, David, Thouvenot, Eric, Cohen, Mikaël, Tourbah, Ayman, Maillart, Elisabeth, Ciron, Jonathan, Deschamps, Romain, Biotti, Damien, Rostasy, Kevin, Neuteboom, Rinze, Hemingway, Cheryl, Forsyth, Rob, Matiello, Marcelo, Webb, Stewart, Hunt, David, Murray, Katy, Hacohen, Yael, Lim, Ming, Leite, M Isabel, Palace, Jacqueline, Solomon, Tom, Lutterotti, Andreas, Fujihara, Kazuo, Nakashima, Ichiro, Bennett, Jeffrey L, Pandit, Lekha, Chitnis, Tanuja, Weinshenker, Brian G, Wildemann, Brigitte, Sato, Douglas Kazutoshi, Kim, Su-Hyun, Huda, Saif, Kim, Ho Jin, Reindl, Markus, Levy, Michael, Jarius, Sven, Tenembaum, Silvia, Paul, Friedemann, Pittock, Sean, Marignier, Romain, and Jacob, Anu

Published
2020
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25. Restoring immune tolerance in neuromyelitis optica

Author

Steinman, Larry, Bar-Or, Amit, Behne, Jacinta M, Benitez-Ribas, Daniel, Chin, Peter S, Clare-Salzler, Michael, Healey, Donald, Kim, James I, Kranz, David M, Lutterotti, Andreas, Martin, Roland, Schippling, Sven, Villoslada, Pablo, Wei, Cheng-Hong, Weiner, Howard L, Zamvil, Scott S, Yeaman, Michael R, Smith, Terry J, Aktas, Orhan, Amezcua, Lilyana, Appiwatanakul, Metha, Asgari, Nasrin, Banwell, Brenda, Bennett, Jeffrey, Bowen, James, Cabre, Philippe, Chitnis, Tanuja, Cohen, Jeffrey, De Seze, Jerome, Fujihara, Kazuo, Han, May, Hellwig, Kerstin, Hintzen, Rogier, Hooper, D Craig, Iorio, Raffaele, Jacob, Anu, Jarius, Sven, Kim, Ho Jin, Kissani, Najib, Klawiter, Eric C, Kleiter, Ingo, Lana-Peixoto, Marco, Leite, Maria Isabel, Levy, Michael, Lublin, Fred, Draayer, Yang Mao, Marignier, Romain, Matiello, Marcelo, Nakashima, Ichiro, O’Connor, Kevin C, Palace, Jacqueline, Pandit, Lekha, Paul, Friedemann, Prayoonwiwat, Naraporn, Riley, Claire, Ruprecht, Klemens, Saiz, Albert, Siritho, Sasitorn, Tenembaum, Silvia, Weinshenker, Brian, Wingerchuk, Dean, and Würfel, Jens

Subjects
Biomedical and Clinical Sciences, Immunology, Neurosciences, Neurodegenerative, Eye Disease and Disorders of Vision, Multiple Sclerosis, Clinical Research, Brain Disorders, Autoimmune Disease, Aetiology, 2.1 Biological and endogenous factors
Abstract

Neuromyelitis optica (NMO) and spectrum disorder (NMO/SD) represent a vexing process and its clinical variants appear to have at their pathogenic core the loss of immune tolerance to the aquaporin-4 water channel protein. This process results in a characteristic pattern of astrocyte dysfunction, loss, and demyelination that predominantly affects the spinal cord and optic nerves. Although several empirical therapies are currently used in the treatment of NMO/SD, none has been proven effective in prospective, adequately powered, randomized trials. Furthermore, most of the current therapies subject patients to long-term immunologic suppression that can cause serious infections and development of cancers. The following is the first of a 2-part description of several key immune mechanisms in NMO/SD that might be amenable to therapeutic restoration of immune tolerance. It is intended to provide a roadmap for how potential immune tolerance restorative techniques might be applied to patients with NMO/SD. This initial installment provides a background rationale underlying attempts at immune tolerization. It provides specific examples of innovative approaches that have emerged recently as a consequence of technical advances. In several autoimmune diseases, these strategies have been reduced to practice. Therefore, in theory, the identification of aquaporin-4 as the dominant autoantigen makes NMO/SD an ideal candidate for the development of tolerizing therapies or cures for this increasingly recognized disease.

Published
2016

26. Restoring immune tolerance in neuromyelitis optica

Author

Bar-Or, Amit, Steinman, Larry, Behne, Jacinta M, Benitez-Ribas, Daniel, Chin, Peter S, Clare-Salzler, Michael, Healey, Donald, Kim, James I, Kranz, David M, Lutterotti, Andreas, Martin, Roland, Schippling, Sven, Villoslada, Pablo, Wei, Cheng-Hong, Weiner, Howard L, Zamvil, Scott S, Smith, Terry J, Yeaman, Michael R, Aktas, Orhan, Amezcua, Lilyana, Appiwatanakul, Metha, Asgari, Nasrin, Banwell, Brenda, Bennett, Jeffrey, Bowen, James, Cabre, Philippe, Chitnis, Tanuja, Cohen, Jeffrey, De Seze, Jerome, Fujihara, Kazuo, Han, May, Hellwig, Kerstin, Hintzen, Rogier, Hooper, D Craig, Iorio, Raffaele, Jacob, Anu, Jarius, Sven, Kim, Ho Jin, Kissani, Najib, Klawiter, Eric C, Kleiter, Ingo, Lana-Peixoto, Marco, Leite, Maria Isabel, Levy, Michael, Lublin, Fred, Draayer, Yang Mao, Marignier, Romain, Matiello, Marcelo, Nakashima, Ichiro, O’Connor, Kevin C, Palace, Jacqueline, Pandit, Lekha, Paul, Friedemann, Prayoonwiwat, Naraporn, Riley, Claire, Ruprecht, Klemens, Saiz, Albert, Siritho, Sasitorn, Tenembaum, Silvia, Weinshenker, Brian, Wingerchuk, Dean, and Würfel, Jens

Subjects
Biomedical and Clinical Sciences, Immunology, Multiple Sclerosis, Autoimmune Disease, Neurosciences, Brain Disorders, Neurodegenerative, Eye Disease and Disorders of Vision, Aetiology, 2.1 Biological and endogenous factors
Abstract

Neuromyelitis optica spectrum disorder (NMO/SD) and its clinical variants have at their core the loss of immune tolerance to aquaporin-4 and perhaps other autoantigens. The characteristic phenotype is disruption of astrocyte function and demyelination of spinal cord, optic nerves, and particular brain regions. In this second of a 2-part article, we present further perspectives regarding the pathogenesis of NMO/SD and how this disease might be amenable to emerging technologies aimed at restoring immune tolerance to disease-implicated self-antigens. NMO/SD appears to be particularly well-suited for these strategies since aquaporin-4 has already been identified as the dominant autoantigen. The recent technical advances in reintroducing immune tolerance in experimental models of disease as well as in humans should encourage quantum leaps in this area that may prove productive for novel therapy. In this part of the article series, the potential for regulatory T and B cells is brought into focus, as are new approaches to oral tolerization. Finally, a roadmap is provided to help identify potential issues in clinical development and guide applications in tolerization therapy to solving NMO/SD through the use of emerging technologies. Each of these perspectives is intended to shine new light on potential cures for NMO/SD and other autoimmune diseases, while sparing normal host defense mechanisms.

Published
2016

27. Cell-based assays for the detection of MOG antibodies: a comparative study

Author

Gastaldi, Matteo, Scaranzin, Silvia, Jarius, Sven, Wildeman, Brigitte, Zardini, Elisabetta, Mallucci, Giulia, Rigoni, Eleonora, Vegezzi, Elisa, Foiadelli, Thomas, Savasta, Salvatore, Banfi, Paola, Versino, Maurizio, Benedetti, Luana, Novi, Giovanni, Mancardi, Margherita Maria, Giacomini, Thea, Annovazzi, Pietro, Baroncini, Damiano, Ferraro, Diana, Lampasona, Vito, Reindl, Markus, Waters, Patrick, and Franciotta, Diego

Published
2020
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32. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders

Author

Wingerchuk, Dean M, Banwell, Brenda, Bennett, Jeffrey L, Cabre, Philippe, Carroll, William, Chitnis, Tanuja, de Seze, Jérôme, Fujihara, Kazuo, Greenberg, Benjamin, Jacob, Anu, Jarius, Sven, Lana-Peixoto, Marco, Levy, Michael, Simon, Jack H, Tenembaum, Silvia, Traboulsee, Anthony L, Waters, Patrick, Wellik, Kay E, and Weinshenker, Brian G

Subjects
Eye Disease and Disorders of Vision, Brain Disorders, Autoimmune Disease, Clinical Research, Neurodegenerative, Multiple Sclerosis, Neurosciences, Neurological, Humans, Neuromyelitis Optica, International Panel for NMO Diagnosis, Clinical Sciences, Cognitive Sciences, Neurology & Neurosurgery
Abstract

Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS.

Published
2015

33. Serum GFAP and NfL as disease severity and prognostic biomarkers in patients with aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder

Author

Schindler, Patrick, Grittner, Ulrike, Oechtering, Johanna, Leppert, David, Siebert, Nadja, Duchow, Ankelien S., Oertel, Frederike C., Asseyer, Susanna, Kuchling, Joseph, Zimmermann, Hanna G., Brandt, Alexander U., Benkert, Pascal, Reindl, Markus, Jarius, Sven, Paul, Friedemann, Bellmann-Strobl, Judith, Kuhle, Jens, and Ruprecht, Klemens

Published
2021
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34. Interleukin-6 Receptor Blockade in Treatment-Refractory MOG-IgG–Associated Disease and Neuromyelitis Optica Spectrum Disorders

Author

Ringelstein, Marius, Ayzenberg, Ilya, Lindenblatt, Gero, Fischer, Katinka, Gahlen, Anna, Novi, Giovanni, Hayward-Könnecke, Helen, Schippling, Sven, Rommer, Paulus S., Kornek, Barbara, Zrzavy, Tobias, Biotti, Damien, Ciron, Jonathan, Audoin, Bertrand, Berthele, Achim, Giglhuber, Katrin, Zephir, Helene, Kümpfel, Tania, Berger, Robert, Röther, Joachim, Häußler, Vivien, Stellmann, Jan-Patrick, Whittam, Daniel, Jacob, Anu, Kraemer, Markus, Gueguen, Antoine, Deschamps, Romain, Bayas, Antonios, Hümmert, Martin W., Trebst, Corinna, Haarmann, Axel, Jarius, Sven, Wildemann, Brigitte, Grothe, Matthias, Siebert, Nadja, Ruprecht, Klemens, Paul, Friedemann, Collongues, Nicolas, Marignier, Romain, Levy, Michael, Karenfort, Michael, Deppe, Michael, Albrecht, Philipp, Hellwig, Kerstin, Gold, Ralf, Hartung, Hans-Peter, Meuth, Sven G., Kleiter, Ingo, and Aktas, Orhan

Published
2022
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35. Time to Disability Milestones and Annualized Relapse Rates in NMOSD and MOGAD.

Author

Duchow, Ankelien, Bellmann‐Strobl, Judith, Friede, Tim, Aktas, Orhan, Angstwurm, Klemens, Ayzenberg, Ilya, Berthele, Achim, Dawin, Eva, Engels, Daniel, Fischer, Katinka, Flaskamp, Martina, Giglhuber, Katrin, Grothe, Matthias, Havla, Joachim, Hümmert, Martin W., Jarius, Sven, Kaste, Matthias, Kern, Peter, Kleiter, Ingo, and Klotz, Luisa

Subjects
NEUROMYELITIS optica
Abstract

Objective: To investigate accumulation of disability in neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein‐antibody‐associated disease (MOGAD) in a changing treatment landscape. We aimed to identify risk factors for the development of disability milestones in relation to disease duration, number of attacks, and age. Methods: We analyzed data from individuals with NMOSD and MOGAD from the German Neuromyelitis Optica Study Group registry. Applying survival analyses, we estimated risk factors and computed time to disability milestones as defined by the Expanded Disability Status Score (EDSS). Results: We included 483 patients: 298 AQP4‐IgG+ NMOSD, 52 AQP4‐IgG−/MOG‐IgG− NMOSD patients, and 133 patients with MOGAD. Despite comparable annualized attack rates, disability milestones occurred earlier and after less attacks in NMOSD patients than MOGAD patients (median time to EDSS 3: AQP4‐IgG+ NMOSD 7.7 (95% CI 6.6–9.6) years, AQP4‐IgG−/MOG‐IgG− NMOSD 8.7) years, MOGAD 14.1 (95% CI 10.4–27.6) years; EDSS 4: 11.9 (95% CI 9.7–14.7), 11.6 (95% lower CI 7.6) and 20.4 (95% lower CI 14.1) years; EDSS 6: 20.1 (95% CI 16.5–32.1), 20.7 (95% lower CI 11.6), and 37.3 (95% lower CI 29.4) years; and EDSS 7: 34.2 (95% lower CI 31.1) for AQP4‐IgG+ NMOSD). Higher age at onset increased the risk for all disability milestones, while risk of disability decreased over time. Interpretation: AQP4‐IgG+ NMOSD, AQP4‐IgG−/MOG‐IgG− NMOSD, and MOGAD patients show distinctive relapse‐associated disability progression, with MOGAD having a less severe disease course. Investigator‐initiated research has led to increasing awareness and improved treatment strategies appearing to ameliorate disease outcomes for NMOSD and MOGAD. ANN NEUROL 2024;95:720–732 [ABSTRACT FROM AUTHOR]

Published
2024
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36. Cerebrospinal fluid findings in patients with neurological manifestations in post-COVID-19 syndrome

Author

Boesl, Fabian, primary, Goereci, Yasemin, additional, Gerhard, Ameli, additional, Bremer, Benno, additional, Raeder, Vanessa, additional, Schweitzer, Finja, additional, Hoppmann, Uta, additional, Behrens, Janina, additional, Bellmann-Strobl, Judith, additional, Paul, Friedemann, additional, Wildemann, Brigitte, additional, Jarius, Sven, additional, Prüss, Harald, additional, Audebert, Heinrich J., additional, Warnke, Clemens, additional, and Franke, Christiana, additional

Published
2023
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41. Cerebrospinal fluid findings in patients with myelin oligodendrocyte glycoprotein (MOG) antibodies. Part 1: Results from 163 lumbar punctures in 100 adult patients

Author

Jarius, Sven, Pellkofer, Hannah, Siebert, Nadja, Korporal-Kuhnke, Mirjam, Hümmert, Martin W., Ringelstein, Marius, Rommer, Paulus S., Ayzenberg, Ilya, Ruprecht, Klemens, Klotz, Luisa, Asgari, Nasrin, Zrzavy, Tobias, Höftberger, Romana, Tobia, Rafik, Buttmann, Mathias, Fechner, Kai, Schanda, Kathrin, Weber, Martin, Asseyer, Susanna, Haas, Jürgen, Lechner, Christian, Kleiter, Ingo, Aktas, Orhan, Trebst, Corinna, Rostasy, Kevin, Reindl, Markus, Kümpfel, Tania, Paul, Friedemann, and Wildemann, Brigitte

Published
2020
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44. Longitudinal optic neuritis-unrelated visual evoked potential changes in NMO spectrum disorders

Author

Ringelstein, Marius, Harmel, Jens, Zimmermann, Hanna, Brandt, Alexander U., Paul, Friedemann, Haarmann, Axel, Buttmann, Mathias, Hümmert, Martin W., Trebst, Corinna, Schroeder, Christoph, Ayzenberg, Ilya, Kleiter, Ingo, Hellwig, Kerstin, Havla, Joachim, Kümpfel, Tania, Jarius, Sven, Wildemann, Brigitte, Rommer, Paulus, Weber, Martin S., Pellkofer, Hannah, Röpke, Luise, Geis, Christian, Retzlaff, Nele, Zettl, Uwe, Deppe, Michael, Klotz, Luisa, Young, Kim, Stellmann, Jan-Patrick, Kaste, Matthias, Kermer, Pawel, Marouf, Wael, Lauda, Florian, Tumani, Hayrettin, Graf, Jonas, Klistorner, Alexander, Hartung, Hans-Peter, Aktas, Orhan, and Albrecht, Philipp

Published
2020
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46. Real-world data on eculizumab treatment in NMOSD: high efficacy and potential challenges (P13-5.017)

Author

Ayzenberg, Ilya, primary, Asseyer, Susanna, additional, Lindenblatt, Gero, additional, Fischer, Katinka, additional, Pul, Refik, additional, Özalp, Sinem-Hilal, additional, Lohmann, Lisa, additional, Giglhuber, Katrin, additional, Häussler, Vivien, additional, Karenfort, Michael, additional, Hellwig, Kerstin, additional, Paul, Friedemann, additional, Bellmann-Strobl, Judith, additional, Otto, Carolin, additional, Ruprecht, Klemens, additional, Ziemssen, Tjalf, additional, Emmer, Alexander, additional, Rothhammer, Veit, additional, Nickel, Florian, additional, Angstwurm, Klemens, additional, Linker, Ralf, additional, Warnke, Clemens, additional, Jarius, Sven, additional, Korporal-Kuhnke, Mirjam, additional, Wildemann, Brigitte, additional, Wolff, Stephanie, additional, Seipelt, Maria, additional, Yalachkov, Yavor, additional, Retzlaff, Nele, additional, Zettl, Uwe, additional, Rommer, Paulus, additional, Kowarik, Markus, additional, Wickel, Jonathan, additional, Geis, Christian, additional, Hümmert, Martin W., additional, Trebst, Corinna, additional, Senel, Makbule, additional, Klotz, Luisa, additional, Gold, Ralf, additional, Kleinschnitz, Christoph, additional, Meuth, Sven, additional, Aktas, Orhan, additional, Berthele, Achim, additional, and Ringelstein, Marius, additional

Published
2023
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47. Prognostic relevance of quantitative and longitudinal MOG antibody testing in patients with MOGAD: a multicentre retrospective study

Author

Gastaldi, Matteo, Foiadelli, Thoma, Greco, Giacomo, Scaranzin, Silvia, Rigoni, Eleonora, Masciocchi, Stefano, Ferrari, Sergio, Mancinelli, Chiara, Brambilla, Laura, Mancardi, Margherita, Giacomini, Thea, Ferraro, Diana, Della Corte, Marida, Gallo, Antonio, Di Filippo, Massimiliano, Benedetti, Luana, Novi, Giovanni, Versino, Maurizio, Banfi, Paola, Iorio, Raffaele, Moiola, Lucia, Turco, Emanuela, Sartori, Stefano, Nosadini, Margherita, Ruggieri, Martino, Savasta, Salvatore, Colombo, Elena, Ballante, Elena, Jarius, Sven, Mariotto, Sara, Franciotta, Diego, Iorio, Raffaele (ORCID:0000-0002-6270-0956), Gastaldi, Matteo, Foiadelli, Thoma, Greco, Giacomo, Scaranzin, Silvia, Rigoni, Eleonora, Masciocchi, Stefano, Ferrari, Sergio, Mancinelli, Chiara, Brambilla, Laura, Mancardi, Margherita, Giacomini, Thea, Ferraro, Diana, Della Corte, Marida, Gallo, Antonio, Di Filippo, Massimiliano, Benedetti, Luana, Novi, Giovanni, Versino, Maurizio, Banfi, Paola, Iorio, Raffaele, Moiola, Lucia, Turco, Emanuela, Sartori, Stefano, Nosadini, Margherita, Ruggieri, Martino, Savasta, Salvatore, Colombo, Elena, Ballante, Elena, Jarius, Sven, Mariotto, Sara, Franciotta, Diego, and Iorio, Raffaele (ORCID:0000-0002-6270-0956)

Abstract

Background IgG antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) define a subset of associated disorders (myelin oligodendrocyte glycoprotein associated disorders (MOGAD)) that can have a relapsing course. However, information on relapse predictors is scarce. The utility of retesting MOG-IgG over time and measuring their titres is uncertain. We aimed to evaluate the clinical relevance of longitudinal MOG-IgG titre measurement to predict relapses in patients with MOGAD. Methods In this retrospective multicentre Italian cohort study, we recruited patients with MOGAD and available longitudinal samples (at least one >3 months after disease onset) and tested them with a live cell-based assay with endpoint titration (1:160 cut-off). Samples were classified as € attack' (within 30 days since a disease attack (n=59, 17%)) and € remission' (≥31 days after attack (n=295, 83%)). Results We included 102 patients with MOGAD (57% adult and 43% paediatric) with a total of 354 samples (83% from remission and 17% from attack). Median titres were higher during attacks (1:1280 vs 1:640, p=0.001). Median onset titres did not correlate with attack-related disability, age or relapses. Remission titres were higher in relapsing patients (p=0.02). When considering the first remission sample available for each patient, titres >1:2560 were predictors of relapsing course in survival (log rank, p<0.001) and multivariate analysis (p<0.001, HR: 10.9, 95% CI 3.4 to 35.2). MOG-IgG seroconversion to negative was associated with a 95% relapse incidence rate reduction (incidence rate ratio: 0.05, p<0.001). Conclusions Persistent MOG-IgG positivity and high remission titres are associated with an increased relapse risk. Longitudinal MOG-IgG titres could be useful to stratify patients to be treated with long term immunosuppression. © 2023 Authors. All rights reserved

Published
2023

50. Apheresis therapies for NMOSD attacks: A retrospective study of 207 therapeutic interventions

Author

Kleiter, Ingo, Gahlen, Anna, Borisow, Nadja, Fischer, Katrin, Wernecke, Klaus-Dieter, Hellwig, Kerstin, Pache, Florence, Ruprecht, Klemens, Havla, Joachim, Kümpfel, Tania, Aktas, Orhan, Hartung, Hans-Peter, Ringelstein, Marius, Geis, Christian, Kleinschnitz, Christoph, Berthele, Achim, Hemmer, Bernhard, Angstwurm, Klemens, Stellmann, Jan-Patrick, Schuster, Simon, Stangel, Martin, Lauda, Florian, Tumani, Hayrettin, Mayer, Christoph, Krumbholz, Markus, Zeltner, Lena, Ziemann, Ulf, Linker, Ralf, Schwab, Matthias, Marziniak, Martin, Then Bergh, Florian, Hofstadt-van Oy, Ulrich, Neuhaus, Oliver, Zettl, Uwe K., Faiss, Jürgen, Wildemann, Brigitte, Paul, Friedemann, Jarius, Sven, Trebst, Corinna, Albrecht, P., Ayzenberg, I., Bayas, A., Bellmann-Strobl, J., Bischof, F., Bittner, S., Böttcher, T., Brettschneider, J., Buttmann, M., DSouza, M., Ettrich, B., Frank, B., Gass, A., Grothe, M., Guthke, K., Haarmann, A., Habedank, E., Hoffmann, F., Hoffmann, O., Hümmert, M.W., Junghans, J., Kaste, M., Kaulen, B., Kermer, P., Kern, P., Klotz, L., Köhler, W., Kolesilova, E., Korsen, M., Kowarik, M., Langel, S., Lee, D.H., Liebetrau, M., Luessi, F., Marouf, W., Meister, S., Melms, A., Metz, I., Münch, C., Niehaus, S., Pawlitzki, M., Pellkofer, H., Puhlmann, H.U., Pul, R., Retzlaf, N., Riedlinger, A., Rommer, P., Röpke, L., Rostásy, K., Rückriem, L., Ruschil, C., Schippling, S., Senel, M., Sieb, J.P., Sommer, C., Spreer, A., Steinbrecher, A., Stephanik, H., Stoppe, M., Süße, M., Tackenberg, B., Tünnerhoff, J., Veauthier, C., Walter, A., Wandinger, K.P., Warnke, C., Weber, M.S., Weissert, R., Wiendl, H., Wilke, C., Winkelmann, A., Yalachkov, Y., Young, K., Zentner, C., and Zipp, F.

Published
2018
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