November 2021; 8 (6) ArticleOpen Access
Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis
Real-world Evidence From the GENERATE Registry
Franziska S. Thaler, Luise Zimmermann, Stefan Kammermeier, Christine Strippel, Marius Ringelstein, Andrea Kraft, Kurt-Wolfram Sühs, View ORCID ProfileJonathan Wickel, Christian Geis, Robert Markewitz, Christian Urbanek, Claudia Sommer, Kathrin Doppler, Loana Penner, View ORCID ProfileJan Lewerenz, Rosa Rößling, Carsten Finke, View ORCID ProfileHarald Prüss, View ORCID ProfileNico Melzer, Klaus-Peter Wandinger, Frank Leypoldt, Tania Kümpfel, on behalf of the German Network for Research on Autoimmune Encephalitis (GENERATE)
First published October 1, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001088
Franziska S. Thaler
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDLuise Zimmermann
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDStefan Kammermeier
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDChristine Strippel
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDMarius Ringelstein
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDAndrea Kraft
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDKurt-Wolfram Sühs
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDJonathan Wickel
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDChristian Geis
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDRobert Markewitz
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDChristian Urbanek
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDClaudia Sommer
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDKathrin Doppler
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDLoana Penner
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
Jan Lewerenz
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDRosa Rößling
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDCarsten Finke
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDHarald Prüss
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDNico Melzer
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDKlaus-Peter Wandinger
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDFrank Leypoldt
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDTania Kümpfel
From the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
MDFrom the Institute of Clinical Neuroimmunology (F.S.T., T.K.), University Hospital and Biomedical Center, Ludwig-Maximilians-Universität, Munich; Section of Translational Neuroimmunology (L.Z., J.W., C.G.), Department of Neurology, Jena University Hospital, Jena; Department of Neurology (S.K.), University Hospital, Ludwig-Maximilians-University, Munich; Department of Neurology with Institute of Translational Neurology (Christine Strippel, N.M.), University of Muenster; Department of Neurology (M.R., N.M.), Medical Faculty, Heinrich Heine University Düsseldorf; Department of Neurology (M.R.), Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf; Department of Neurology Martha-Maria Hospital (A.K.), Halle/Saale, Academic Hospital of University Halle-Wittenberg; Department of Neurology (K.-W.S.), Hannover Medical School; Department of Neurology (R.M., K.-P.W.), University Hospital Schleswig-Holstein, Lübeck; Department of Neurology (C.U.), Klinikum der Stadt Ludwigshafen a. Rh. gGmbH, Ludwigshafen; Department of Neurology (C. Sommer, K.D.), University Hospital Würzburg; Department of Neurology (L.P., J.L.), Ulm University; Department of Neurology and Experimental Neurology (R.R., C.F., H.P.), Charité - Universitätsmedizin Berlin; German Center for Neurodegenerative Diseases (DZNE) Berlin (R.R., H.P.); Neuroimmunology Section (K.-P.W., F.L.), Institute of Clinical Chemistry, University Hospital Schleswig-Holstein Kiel, Lübeck; and Department of Neurology (F.L.), Christian-Albrechts-Universität Kiel, Germany.
Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis
Real-world Evidence From the GENERATE Registry
Franziska S. Thaler, Luise Zimmermann, Stefan Kammermeier, Christine Strippel, Marius Ringelstein, Andrea Kraft, Kurt-Wolfram Sühs, Jonathan Wickel, Christian Geis, Robert Markewitz, Christian Urbanek, Claudia Sommer, Kathrin Doppler, Loana Penner, Jan Lewerenz, Rosa Rößling, Carsten Finke, Harald Prüss, Nico Melzer, Klaus-Peter Wandinger, Frank Leypoldt, Tania Kümpfel, on behalf of the German Network for Research on Autoimmune Encephalitis (GENERATE)
Neurol Neuroimmunol Neuroinflamm Nov 2021, 8 (6) e1088; DOI: 10.1212/NXI.0000000000001088
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Abstract
Background and Objectives To determine the real-world use of rituximab in autoimmune encephalitis (AE) and to correlate rituximab treatment with the long-term outcome.
Methods Patients with NMDA receptor (NMDAR)-AE, leucine-rich glioma-inactivated-1 (LGI1)- AE, contactin-associated protein-like-2 (CASPR2)-AE, or glutamic acid decarboxylase 65 (GAD65) disease from the GErman Network for Research on AuToimmune Encephalitis who had received at least 1 rituximab dose and a control cohort of non–rituximab-treated patients were analyzed retrospectively.
Results Of the 358 patients, 163 (46%) received rituximab (NMDAR-AE: 57%, CASPR2-AE: 44%, LGI1-AE: 43%, and GAD65 disease: 37%). Rituximab treatment was initiated significantly earlier in NMDAR- and LGI1-AE (median: 54 and 155 days from disease onset) compared with CASPR2-AE or GAD65 disease (median: 632 and 1,209 days). Modified Rankin Scale (mRS) scores improved significantly in patients with NMDAR-AE, both with and without rituximab treatment. Although being more severely affected at baseline, rituximab-treated patients with NMDAR-AE more frequently reached independent living (mRS score ≤2) (94% vs 88%). In LGI1-AE, rituximab-treated and nontreated patients improved, whereas in CASPR2-AE, only rituximab-treated patients improved significantly. No improvement was observed in patients with GAD65 disease. A significant reduction of the relapse rate was observed in rituximab-treated patients (5% vs 13%). Detection of NMDAR antibodies was significantly associated with mRS score improvement. A favorable outcome was also observed with early treatment initiation.
Discussion We provide real-world data on immunosuppressive treatments with a focus on rituximab treatment for patients with AE in Germany. We suggest that early and short-term rituximab therapy might be an effective and safe treatment option in most patients with NMDAR-, LGI1-, and CASPR2-AE.
Class of Evidence This study provides Class IV evidence that rituximab is an effective treatment for some types of AE.
Glossary
- abs=
- antibodies;
- AE=
- autoimmune encephalitis;
- CA=
- cerebellar ataxia;
- CASPR2=
- contactin-associated protein-like-2;
- CBA=
- cell-based assay;
- GAD65=
- glutamic acid decarboxylase 65;
- GENERATE=
- GErman Network for Research on AuToimmune Encephalitis;
- IHC=
- immunohistochemistry;
- IVIG=
- IV immunoglobulin;
- LE=
- limbic/autoimmune encephalitis;
- LGI1=
- leucine-rich glioma-inactivated-1;
- mRS=
- Modified Rankin Scale;
- NMDAR=
- NMDA receptor;
- RIA=
- radioimmunoassay;
- SPS=
- stiff-person syndrome
Footnotes
Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.
F. Leypoldt and T. Kümpfel contributed equally to this work as co–senior authors.
German Network for Research on Autoimmune Encephalitis (GENERATE) coinvestigators are listed in Appendix 2 at the end of the article.
The Article Processing Charge was funded by the authors.
Class of Evidence: NPub.org/coe
- Received February 12, 2021.
- Accepted in final form August 23, 2021.
- Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
Letters: Rapid online correspondence
- Author Response: Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis: Real-world Evidence From the GENERATE Registry
- Frank Leypoldt, Neuroimmunologist, Neuroimmunology, Inst. of Clinical Chemistry and Dep. of Neurology, University Hospital Schleswig-Holstein
- Franziska Thaler, Neuroimmunologist, Institute of Clinical Neuroimmunology, Ludwig-Maximilians-University Munich
- Tania Kümpfel, Neuroimmunologist, Institute of Clinical Neuroimmunology, Ludwig-Maximilians-University Munich
Submitted November 17, 2021 - Reader Response: Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis
- Maria Andreina Vera Silva, Neurology Resident, Cleveland Clinic Florida
- Nestor Galvez-Jimenez, Neurologist, Cleveland Clinic Florida
Submitted November 03, 2021
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