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1. Neuropathological spectrum of anti-IgLON5 disease and stages of brainstem tau pathology: updated neuropathological research criteria of the disease-related tauopathy

Author

Gelpi, Ellen, Reinecke, Raphael, Gaig, Carles, Iranzo, Alex, Sabater, Lidia, Molina-Porcel, Laura, Aldecoa, Iban, Endmayr, Verena, Högl, Birgit, Schmutzhard, Erich, Poewe, Werner, Pfausler, Bettina, Popovic, Mara, Pretnar-Oblak, Janja, Leypoldt, Frank, Matschke, Jakob, Glatzel, Markus, Erro, Elena Maria, Jerico, Ivonne, Caballero, Maria Cristina, Zelaya, Maria Victoria, Mariotto, Sara, Heidbreder, Anna, Kalev, Ognian, Weis, Serge, Macher, Stefan, Berger-Sieczkowski, Evelyn, Ferrari, Julia, Reisinger, Christoph, Klupp, Nikolaus, Tienari, Pentti, Rautila, Osma, Niemelä, Marja, Yilmazer-Hanke, Deniz, Guasp, Mar, Bloem, Bas, Van Gaalen, Judith, Kusters, Benno, Titulaer, Maarten, Fransen, Nina L., Santamaria, Joan, Dawson, Thimoty, Holton, Janice L., Ling, Helen, Revesz, Tamas, Myllykangas, Liisa, Budka, Herbert, Kovacs, Gabor G., Lewerenz, Jan, Dalmau, Josep, Graus, Francesc, Koneczny, Inga, and Höftberger, Romana

Published
2024
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3. SARS-CoV-2 infection causes dopaminergic neuron senescence

Author

Yang, Liuliu, Kim, Tae Wan, Han, Yuling, Nair, Manoj S., Harschnitz, Oliver, Zhu, Jiajun, Wang, Pengfei, Koo, So Yeon, Lacko, Lauretta A., Chandar, Vasuretha, Bram, Yaron, Zhang, Tuo, Zhang, Wei, He, Feng, Pan, Chendong, Wu, Junjie, Huang, Yaoxing, Evans, Todd, van der Valk, Paul, Titulaer, Maarten J., Spoor, Jochem K.H., Furler O’Brien, Robert L., Bugiani, Marianna, D.J. Van de Berg, Wilma, Schwartz, Robert E., Ho, David D., Studer, Lorenz, and Chen, Shuibing

Published
2024
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5. Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management.

Author

Abboud, Hesham, Probasco, John C, Irani, Sarosh, Ances, Beau, Benavides, David R, Bradshaw, Michael, Christo, Paulo Pereira, Dale, Russell C, Fernandez-Fournier, Mireya, Flanagan, Eoin P, Gadoth, Avi, George, Pravin, Grebenciucova, Elena, Jammoul, Adham, Lee, Soon-Tae, Li, Yuebing, Matiello, Marcelo, Morse, Anne Marie, Rae-Grant, Alexander, Rojas, Galeno, Rossman, Ian, Schmitt, Sarah, Venkatesan, Arun, Vernino, Steven, Pittock, Sean J, Titulaer, Maarten J, and Autoimmune Encephalitis Alliance Clinicians Network

Subjects
Autoimmune Encephalitis Alliance Clinicians Network, Humans, Encephalitis, Autoimmune Diseases, Adrenal Cortex Hormones, Immunoglobulins, Intravenous, Treatment Outcome, Plasmapheresis, autoimmune encephalitis, neuroimmunology, paraneoplastic syndrome, Clinical Research, Autoimmune Disease, Brain Disorders, Medical and Health Sciences, Psychology and Cognitive Sciences, Neurology & Neurosurgery
Abstract

The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.

Published
2021

7. Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management.

Author

Abboud, Hesham, Probasco, John, Irani, Sarosh R, Ances, Beau, Benavides, David R, Bradshaw, Michael, Christo, Paulo Pereira, Dale, Russell C, Fernandez-Fournier, Mireya, Flanagan, Eoin P, Gadoth, Avi, George, Pravin, Grebenciucova, Elena, Jammoul, Adham, Lee, Soon-Tae, Li, Yuebing, Matiello, Marcelo, Morse, Anne Marie, Rae-Grant, Alexander, Rojas, Galeno, Rossman, Ian, Schmitt, Sarah, Venkatesan, Arun, Vernino, Steven, Pittock, Sean J, Titulaer, Maarten, and Autoimmune Encephalitis Alliance Clinicians Network

Subjects
Autoimmune Encephalitis Alliance Clinicians Network, autoimmune encephalitis, neuroimmunology, paraneoplastic syndrome, Biotechnology, Prevention, Autoimmune Disease, Clinical Research, Brain Disorders, Neurosciences, Medical and Health Sciences, Psychology and Cognitive Sciences, Neurology & Neurosurgery
Abstract

The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. The most popular bridging therapy was oral prednisone taper chosen by 38% of responders while rituximab was the most popular maintenance therapy chosen by 46%. Most responders considered maintenance immunosuppression after a second relapse in patients with neuronal surface antibodies (70%) or seronegative autoimmune encephalitis (61%) as opposed to those with onconeuronal antibodies (29%). Most responders opted to cancer screening for 4 years in patients with neuronal surface antibodies (49%) or limbic encephalitis (46%) as opposed to non-limbic seronegative autoimmune encephalitis (36%). Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.

Published
2021

8. Viral, Bacterial, Metabolic, and Autoimmune Causes of Severe Acute Encephalopathy in Sub-Saharan Africa: A Multicenter Cohort Study

Author

Edridge, Arthur, Namazzi, Ruth, Tebulo, Andrew, Mfizi, Anan, Deijs, Martin, Koekkoek, Sylvie, de Wever, Bob, van der Ende, Arie, Umiwana, Jeanine, de Jong, Menno D., Jans, Judith, Verhoeven-Duif, Nanda, Titulaer, Maarten, van Karnebeek, Clara, Seydel, Karl, Taylor, Terrie, Asiimwe-Kateera, Brenda, van der Hoek, Lia, Kabayiza, Jean-Claude, Mallewa, Macpherson, Idro, Richard, Boele van Hensbroek, Michael, and van Woensel, Job B.M.

Published
2023
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9. Cerebrospinal fluid proteomics indicates immune dysregulation and neuronal dysfunction in antibody associated autoimmune encephalitis

Author

Räuber, Saskia, Schroeter, Christina B., Strippel, Christine, Nelke, Christopher, Ruland, Tillmann, Dik, Andre, Golombeck, Kristin S., Regner-Nelke, Liesa, Paunovic, Manuela, Esser, Daniela, Münch, Christian, Rosenow, Felix, van Duijn, Martijn, Henes, Antonia, Ruck, Tobias, Amit, Ido, Leypoldt, Frank, Titulaer, Maarten J., Wiendl, Heinz, Meuth, Sven G., Meyer zu Hörste, Gerd, and Melzer, Nico

Published
2023
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10. Review and meta-analysis of neuropsychological findings in autoimmune limbic encephalitis with autoantibodies against LGI1, CASPR2, and GAD65 and their response to immunotherapy

Author

Mueller, Christoph, Elben, Saskia, Day, Gregory S., Alves, Pedro, Hebert, Julien, Tang-Wai, David F., Holtmann, Olga, Iorio, Raffaele, Perani, Daniela, Titulaer, Maarten J., Hansen, Niels, Bartsch, Thorsten, Johnen, Andreas, Illes, Zsolt, Borm, Leah, Willison, Alice G., Wiendl, Heinz, Meuth, Sven G., Kovac, Stjepana, Bölte, Jens, and Melzer, Nico

Published
2023
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13. Risk of Seizure Recurrence Due to Autoimmune Encephalitis With NMDAR, LGI1, CASPR2, and GABA B R Antibodies

Author

Rada, Anna, primary, Hagemann, Anne, additional, Aaberg Poulsen, Charlotte, additional, Baumgartner, Tobias, additional, Berki, Timea, additional, Blaabjerg, Morten, additional, Brenner, Juliette, additional, Britton, Jeffrey W., additional, Christiana, Andrew, additional, Ciano-Petersen, Nicolás L., additional, Crijnen, Yvette, additional, Elišák, Martin, additional, Farina, Antonio, additional, Friedman, Alec R., additional, Hayden, Zsófia, additional, Hébert, Julien, additional, Holtkamp, Martin, additional, Hong, Zhen, additional, Honnorat, Jerome, additional, Ilyas-Feldmann, Maria, additional, Irani, Sarosh R., additional, Kovac, Stjepana, additional, Marusic, Petr, additional, Muñiz-Castrillo, Sergio, additional, Ramanathan, Sudarshini, additional, Smith, Kelsey M., additional, Steriade, Claude, additional, Strippel, Christine, additional, Surges, Rainer, additional, Titulaer, Maarten J., additional, Uy, Christopher E., additional, de Vries, Juna M., additional, Bien, Christian G., additional, and Specht, Ulrich, additional

Published
2024
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15. Comparative Study of Paraneoplastic and Nonparaneoplastic Autoimmune Encephalitis With GABA B R Antibodies

Author

Lamblin, Florian, primary, Kerstens, Jeroen, additional, Muñiz-Castrillo, Sergio, additional, Vogrig, Alberto, additional, Goncalves, David, additional, Rogemond, Veronique, additional, Picard, Geraldine, additional, Villard, Marine, additional, Pinto, Anne-Laurie, additional, Van Coevorden-Hameete, Marleen H., additional, De Bruijn, Marienke A., additional, De Vries, Juna M., additional, Schreurs, Marco, additional, Tyvaert, Louise, additional, Hopes, Lucie, additional, Aupy, Jerome, additional, Marchal, Cecile, additional, Psimaras, Dimitri, additional, Kremer, Laurent, additional, Bourg, Veronique, additional, Antoine, Jean-Christophe G., additional, Wang, Adrien, additional, Kahane, Philippe, additional, Demeret, Sophie, additional, Ahle, Guido, additional, Sempere, Vicente Peris, additional, Timestit, Noemie, additional, Nourredine, Mikail, additional, Maureille, Aurelien, additional, Benaiteau, Marie, additional, Joubert, Bastien, additional, Mignot, Emmanuel, additional, Titulaer, Maarten J., additional, and Honnorat, Jerome, additional

Published
2024
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17. A clinical approach to diagnosis of autoimmune encephalitis

Author

Graus, Francesc, Titulaer, Maarten J, Balu, Ramani, Benseler, Susanne, Bien, Christian G, Cellucci, Tania, Cortese, Irene, Dale, Russell C, Gelfand, Jeffrey M, Geschwind, Michael, Glaser, Carol A, Honnorat, Jerome, Höftberger, Romana, Iizuka, Takahiro, Irani, Sarosh R, Lancaster, Eric, Leypoldt, Frank, Prüss, Harald, Rae-Grant, Alexander, Reindl, Markus, Rosenfeld, Myrna R, Rostásy, Kevin, Saiz, Albert, Venkatesan, Arun, Vincent, Angela, Wandinger, Klaus-Peter, Waters, Patrick, and Dalmau, Josep

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Immunization, Prevention, Clinical Research, Autoimmune Disease, Brain Disorders, Vaccine Related, Neurosciences, Neurodegenerative, 4.1 Discovery and preclinical testing of markers and technologies, 4.2 Evaluation of markers and technologies, Detection, screening and diagnosis, Metabolic and endocrine, Inflammatory and immune system, Autoimmune Diseases of the Nervous System, Encephalitis, Humans, Neurology & Neurosurgery, Clinical sciences
Abstract

Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the literature and gathered the experience of a team of experts with the aims of developing a practical, syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines to navigate through the differential diagnosis. Because autoantibody test results and response to therapy are not available at disease onset, we based the initial diagnostic approach on neurological assessment and conventional tests that are accessible to most clinicians. Through logical differential diagnosis, levels of evidence for autoimmune encephalitis (possible, probable, or definite) are achieved, which can lead to prompt immunotherapy.

Published
2016

18. Risk of Seizure Recurrence Due to Autoimmune Encephalitis With NMDAR, LGI1, CASPR2, and GABABR Antibodies:Implications for Return to Driving

Author

Rada, Anna, Hagemann, Anne, Aaberg Poulsen, Charlotte, Baumgartner, Tobias, Berki, Timea, Blaabjerg, Morten, Brenner, Juliette, Britton, Jeffrey W., Christiana, Andrew, Ciano-Petersen, Nicolás L., Crijnen, Yvette, Elišák, Martin, Farina, Antonio, Friedman, Alec R., Hayden, Zsófia, Hébert, Julien, Holtkamp, Martin, Hong, Zhen, Honnorat, Jerome, Ilyas-Feldmann, Maria, Irani, Sarosh R., Kovac, Stjepana, Marusic, Petr, Muñiz-Castrillo, Sergio, Ramanathan, Sudarshini, Smith, Kelsey M., Steriade, Claude, Strippel, Christine, Surges, Rainer, Titulaer, Maarten J., Uy, Christopher E., De Vries, Juna M., Bien, Christian G., Specht, Ulrich, Rada, Anna, Hagemann, Anne, Aaberg Poulsen, Charlotte, Baumgartner, Tobias, Berki, Timea, Blaabjerg, Morten, Brenner, Juliette, Britton, Jeffrey W., Christiana, Andrew, Ciano-Petersen, Nicolás L., Crijnen, Yvette, Elišák, Martin, Farina, Antonio, Friedman, Alec R., Hayden, Zsófia, Hébert, Julien, Holtkamp, Martin, Hong, Zhen, Honnorat, Jerome, Ilyas-Feldmann, Maria, Irani, Sarosh R., Kovac, Stjepana, Marusic, Petr, Muñiz-Castrillo, Sergio, Ramanathan, Sudarshini, Smith, Kelsey M., Steriade, Claude, Strippel, Christine, Surges, Rainer, Titulaer, Maarten J., Uy, Christopher E., De Vries, Juna M., Bien, Christian G., and Specht, Ulrich

Abstract

Background and Objectives:Patients with ongoing seizures are usually not allowed to drive. The prognosis for seizure freedom is favorable in patients with autoimmune encephalitis (AIE) with antibodies against NMDA receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), and the gamma-aminobutyric-acid B receptor (GABABR). We hypothesized that after a seizure-free period of 3 months, patients with AIE have a seizure recurrence risk of <20% during the subsequent 12 months. This would render them eligible for noncommercial driving according to driving regulations in several countries.Methods:This retrospective multicenter cohort study analyzed follow-up data from patients aged 15 years or older with seizures resulting from NMDAR-, LGI1-, CASPR2-, or GABABR-AIE, who had been seizure-free for ≥3 months. We used Kaplan-Meier (KM) estimates for the seizure recurrence risk at 12 months for each antibody group and tested for the effects of potential covariates with regression models.Results:We included 383 patients with NMDAR-, 440 with LGI1-, 114 with CASPR2-, and 44 with GABABR-AIE from 14 international centers. After being seizure-free for 3 months after an initial seizure period, we calculated the probability of remaining seizure-free for another 12 months (KM estimate) as 0.89 (95% confidence interval [CI] 0.85-0.92) for NMDAR, 0.84 (CI 0.80-0.88) for LGI1, 0.82 (CI 0.75-0.90) for CASPR2, and 0.76 (CI 0.62-0.93) for GABABR.Discussion:Taking a <20% recurrence risk within 12 months as sufficient, patients with NMDAR-AIE and LGI1-AIE could be considered eligible for noncommercial driving after having been seizure-free for 3 months.

Published
2024

19. Autoimmune-associated seizure disorders

Author

Smith, Kelsey M., Budhram, Adrian, Geis, Christian, Mckeon, Andrew, Steriade, Claude, Stredny, Coral M., Titulaer, Maarten J., Britton, Jeffrey W., Smith, Kelsey M., Budhram, Adrian, Geis, Christian, Mckeon, Andrew, Steriade, Claude, Stredny, Coral M., Titulaer, Maarten J., and Britton, Jeffrey W.

Abstract

With the discovery of an expanding number of neural autoantibodies, autoimmune etiologies of seizures have been increasingly recognized. Clinical phenotypes have been identified in association with specific underlying antibodies, allowing an earlier diagnosis. These phenotypes include faciobrachial dystonic seizures with LGI1 encephalitis, neuropsychiatric presentations associated with movement disorders and seizures in NMDA-receptor encephalitis, and chronic temporal lobe epilepsy in GAD65 neurologic autoimmunity. Prompt recognition of these disorders is important, as some of them are highly responsive to immunotherapy. The response to immunotherapy is highest in patients with encephalitis secondary to antibodies targeting cell surface synaptic antigens. However, the response is less effective in conditions involving antibodies binding intracellular antigens or in Rasmussen syndrome, which are predominantly mediated by cytotoxic T-cell processes that are associated with irreversible cellular destruction. Autoimmune encephalitides also may have a paraneoplastic etiology, further emphasizing the importance of recognizing these disorders. Finally, autoimmune processes and responses to novel immunotherapies have been reported in new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES), warranting their inclusion in any current review of autoimmune-associated seizure disorders.

Published
2024

20. Clinical Outcome Assessments in Encephalitis

Author

Brenner, Juliette, Olijslagers, Sammy H.C., Crijnen, Yvette S., de Vries, Juna M., Mandarakas, Melissa R., Titulaer, Maarten J., Brenner, Juliette, Olijslagers, Sammy H.C., Crijnen, Yvette S., de Vries, Juna M., Mandarakas, Melissa R., and Titulaer, Maarten J.

Abstract

Background and Objectives Most patients with encephalitis experience persisting neurocognitive and neuropsychiatric sequelae in the years following this acute illness. Reported outcomes are often based on generic clinical outcome assessments that rarely capture the patient perspective. This may result in an underestimation of disease-specific sequelae. Disease-specific clinical outcome assessments can improve clinical relevance of reported outcomes and increase the power of research and trials. There are no patient-reported outcome measures (PROMs) developed or validated specifically for patients with encephalitis. The primary objective of this systematic literature review was to identify PROMs that have been developed for or validated in patients with encephalitis. Methods We performed a systematic review of the literature published from inception until May 2023 in 3 large international databases (MEDLINE, EMBASE and Cochrane libraries). Eligible studies should have developed or validated a PROM in patients with encephalitis or encephalopathy. Methodologic quality was evaluated using the Consensus-based Standards for the selection of health status Measurement Instruments study design checklist for PROMs. Results We identified no disease-specific PROMs developed or validated for patients with encephalitis. We identified one study on the development and validation of a disease-specific PROM for hepatic encephalopathy, although this disease course is substantially different to that of patients with encephalitis. The methodologic quality of the included study was generally rated as "doubtful." We identified 30 PROMs that have been applied in 46 studies on encephalitis or encephalopathy, although not validated in these populations. The most commonly applied PROMs for measuring Health-Related Quality of Life were the Medical Outcomes Study Short Form-36 and the Sickness Impact Profile. Emotional well-being was often

Published
2024

21. Neuropsychological Testing in Autoimmune Encephalitis:A Scoping Review

Author

Galioto, Rachel, Grezmak, Tiffany, Swetlik, Carol, Abbatemarco, Justin R., Titulaer, Maarten J., Finke, Carsten, Kunchok, Amy, Galioto, Rachel, Grezmak, Tiffany, Swetlik, Carol, Abbatemarco, Justin R., Titulaer, Maarten J., Finke, Carsten, and Kunchok, Amy

Abstract

BACKGROUND AND OBJECTIVES: Identifying optimal methods for evaluation and monitoring of cognitive outcomes in AE is important for clinical care and research. This scoping review aimed to evaluate neuropsychological tests (NPT) that are most frequently impaired in AE cohorts to provide recommendations for a standardized NPT battery for AE outcome. METHODS: PubMed search for studies examining NPT in patients with AE was conducted on June 9, 2023. Studies were screened for inclusion/exclusion criteria as follows: at least 1 NPT, individual NPT test scores with comparison with healthy controls or normative data and neural-IgG status, total sample size ≥5, and English manuscript available. RESULTS: The search yielded 5,393 studies, of which 3,359 were screened, 107 were full text reviewed, and 32 met inclusion/exclusion criteria, anti-NMDA-R (k = 18), anti-LGI1 (k = 10), anti-GABAB-R (k = 2), anti-GAD-65 (k = 4), and anti-CASPR2 (k = 3). The cognitive domains most frequently impaired were visual and verbal episodic memory, attention/working memory, processing speed, and aspects of executive functions. DISCUSSION: Given the dearth of literature examining NPT in AE in combination with small sample sizes and methodological differences, more research in this area is needed. However, we provide recommendations for a test battery to be used in future studies, with the aim of standardizing research in this area. Based on the available literature, we recommend the use of comprehensive NPT batteries, spanning all cognitive domains. The highest yield measures may include the tests of (1) visual and verbal learning/memory, (2) basic and sustained attention, (3) processing speed, and (4) executive functions.

Published
2024

22. Comparative Study of Paraneoplastic and Nonparaneoplastic Autoimmune Encephalitis With GABABR Antibodies

Author

Lamblin, Florian, Kerstens, Jeroen, Muñiz-Castrillo, Sergio, Vogrig, Alberto, Goncalves, David, Rogemond, Veronique, Picard, Geraldine, Villard, Marine, Pinto, Anne Laurie, Van Coevorden-Hameete, Marleen H., De Bruijn, Marienke A., De Vries, Juna M., Schreurs, Marco, Tyvaert, Louise, Hopes, Lucie, Aupy, Jerome, Marchal, Cecile, Psimaras, Dimitri, Kremer, Laurent, Bourg, Veronique, Antoine, Jean Christophe G., Wang, Adrien, Kahane, Philippe, Demeret, Sophie, Ahle, Guido, Sempere, Vicente Peris, Timestit, Noemie, Nourredine, Mikail, Maureille, Aurelien, Benaiteau, Marie, Joubert, Bastien, Mignot, Emmanuel, Titulaer, Maarten J., Honnorat, Jerome, Lamblin, Florian, Kerstens, Jeroen, Muñiz-Castrillo, Sergio, Vogrig, Alberto, Goncalves, David, Rogemond, Veronique, Picard, Geraldine, Villard, Marine, Pinto, Anne Laurie, Van Coevorden-Hameete, Marleen H., De Bruijn, Marienke A., De Vries, Juna M., Schreurs, Marco, Tyvaert, Louise, Hopes, Lucie, Aupy, Jerome, Marchal, Cecile, Psimaras, Dimitri, Kremer, Laurent, Bourg, Veronique, Antoine, Jean Christophe G., Wang, Adrien, Kahane, Philippe, Demeret, Sophie, Ahle, Guido, Sempere, Vicente Peris, Timestit, Noemie, Nourredine, Mikail, Maureille, Aurelien, Benaiteau, Marie, Joubert, Bastien, Mignot, Emmanuel, Titulaer, Maarten J., and Honnorat, Jerome

Abstract

BACKGROUND AND OBJECTIVES: While patients with paraneoplastic autoimmune encephalitis (AE) with gamma-aminobutyric-acid B receptor antibodies (GABABR-AE) have poor functional outcomes and high mortality, the prognosis of nonparaneoplastic cases has not been well studied. METHODS: Patients with GABABR-AE from the French and the Dutch Paraneoplastic Neurologic Syndromes Reference Centers databases were retrospectively included and their data collected; the neurologic outcomes of paraneoplastic and nonparaneoplastic cases were compared. Immunoglobulin G (IgG) isotyping and human leukocyte antigen (HLA) genotyping were performed in patients with available samples. RESULTS: A total of 111 patients (44/111 [40%] women) were enrolled, including 84 of 111 (76%) paraneoplastic and 18 of 111 (16%) nonparaneoplastic cases (cancer status was undetermined for 9 patients). Patients presented with seizures (88/111 [79%]), cognitive impairment (54/111 [49%]), and/or behavioral disorders (34/111 [31%]), and 54 of 111 (50%) were admitted in intensive care unit (ICU). Nonparaneoplastic patients were significantly younger (median age 54 years [range 19-88] vs 67 years [range 50-85] for paraneoplastic cases, p < 0.001) and showed a different demographic distribution. Nonparaneoplastic patients more often had CSF pleocytosis (17/17 [100%] vs 58/78 [74%], p = 0.02), were almost never associated with KTCD16-abs (1/16 [6%] vs 61/70 [87%], p < 0.001), and were more frequently treated with second-line immunotherapy (11/18 [61%] vs 18/82 [22%], p = 0.003). However, no difference of IgG subclass or HLA association was observed, although sample size was small (10 and 26 patients, respectively). After treatment, neurologic outcome was favorable (mRS ≤2) for 13 of 16 (81%) nonparaneoplastic and 37 of 84 (48%) paraneoplastic cases (p = 0.03), while 3 of 18 (17%) and 42 of 83 (51%) patients had died at last follow-up (p = 0.008), respectively. Neurologic outcome no longer differed after adj

Published
2024

24. Encephalitis and AMPA receptor antibodies

Author

Höftberger, Romana, van Sonderen, Agnes, Leypoldt, Frank, Houghton, David, Geschwind, Michael, Gelfand, Jeffrey, Paredes, Mercedes, Sabater, Lidia, Saiz, Albert, Titulaer, Maarten J, Graus, Francesc, and Dalmau, Josep

Subjects
Immunization, Rare Diseases, Neurosciences, Biotechnology, Cancer, Clinical Research, Brain Disorders, Lung, Aetiology, 2.1 Biological and endogenous factors, Adult, Aged, Aged, 80 and over, Autoantibodies, Brain, Comorbidity, Encephalitis, Female, Follow-Up Studies, HEK293 Cells, Humans, Kaplan-Meier Estimate, Male, Middle Aged, Receptors, AMPA, Retrospective Studies, Young Adult, Clinical Sciences, Cognitive Sciences, Neurology & Neurosurgery
Abstract

ObjectiveWe report the clinical features, comorbidities, and outcome of 22 newly identified patients with antibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR).MethodsThis was a retrospective review of patients diagnosed between May 2009 and March 2014. Immunologic techniques have been reported previously.ResultsPatients' median age was 62 years (range 23-81; 14 female). Four syndromes were identified: 12 (55%) patients presented with distinctive limbic encephalitis (LE), 8 (36%) with limbic dysfunction along with multifocal/diffuse encephalopathy, one with LE preceded by motor deficits, and one with psychosis with bipolar features. Fourteen patients (64%) had a tumor demonstrated pathologically (5 lung, 4 thymoma, 2 breast, 2 ovarian teratoma) or radiologically (1 lung). Additional antibodies occurred in 7 patients (3 onconeuronal, 1 tumor-related, 2 cell surface, and 1 tumor-related and cell surface), all with neurologic symptoms or tumor reflecting the concurrent autoimmunity. Treatment and outcome were available from 21 patients (median follow-up 72 weeks, range 5-266): 5 had good response to immunotherapy and tumor therapy, 10 partial response, and 6 did not improve. Eventually 5 patients died; all had a tumor or additional paraneoplastic symptoms related to onconeuronal antibodies. Coexistence of onconeuronal antibodies predicted a poor outcome (p = 0.009).ConclusionAnti-AMPAR encephalitis usually manifests as LE, can present with other symptoms or psychosis, and is paraneoplastic in 64% of cases. Complete and impressive neurologic improvement can occur, but most patients have partial recovery. Screening for a tumor and onconeuronal antibodies is important because their detection influences outcome.

Published
2015

27. Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial

Author

Cutter, Gary, Aban, Inmaculada, Minisman, Greg, Feese, Michelle, Kuo, Hui-Chien, Newsom-Davis, John, Wolfe, Gil, Kaminski, Henry, Jaretzki, Alfred, Sonett, Joshua, Mazia, Claudio, Saluto, Valeria, Rosenberg, Moises, Alvarez, Valeria, Rey, Lisa, King, John, Butzkueven, Helmut, Goldblatt, John, Carey, John, Pollard, John, Reddel, Stephen, Handel, Nicholas, McCaughan, Brian, Pallot, Linda, Waddington-Cruz, Márcia, Novis, Ricardo, Boasquevisque, Carlos, Dias-Tosta, Elza, Morato-Fernandez, Rubens, Ximenes, Manoel, Werneck, Lineu, Scola, Rosana, Soltoski, Paulo, Chalk, Colin, Moore, Fraser, Mulder, David, Wadup, Lisa, Oger, Joel, Mezei, Michele, Evans, Kenneth, Jiwa, Theresa, Schaffar, Anne, White, Chris, Toth, Cory, Gelfand, Gary, Wood, Susan, Pringle, Elizabeth, Zwicker, Jocelyn, Maziak, Donna, Shamji, Farid, Sundaresan, Sudhir, Seely, Andrew, Cea, Gabriel, Verduga, Renato, Aguayo, Alberto, Jander, Sebastian, Zickler, Philipp, Klein, Michael, Marx, Alexander, Ströbel, Philipp, Weis, Cleo-Aron, Melms, Arthur, Bischof, Felix, Aebert, Hermann, Ziemer, Gerhard, Nix, Wilfred, Thümler, Björn, Wilhem-Schwenkmezger, Thomas, Mayer, Eckhard, Schalke, Berthold, Pöschel, Peter, Hieber, Gisela, Wiebe, Karsten, Antonini, Giovanni, Clemenzi, Alessandro, Ceschin, Vanessa, Rendina, Erino, Venuta, Federico, Morino, Stefania, Bucci, Elisabetta, Durelli, Luca, Tavella, Alessia, Clerico, Marinella, Contessa, Giulia, Borasio, Piero, Evoli, Amelia, Servidei, Serenella, Granone, Pierluigi, Mantegazza, Renato, Berta, Emilia, Novellino, Lorenzo, Spinelli, Luisa, Motomura, Masakatsu, Matsuo, Hidenori, Nagayasu, Takeshi, Yoshikawa, Hiroaki, Takamori, Masaharu, Oda, Makoto, Matsumoto, Isao, Furukawa, Yutaka, Noto, Daisuke, Motozaki, Yuko, Iwasa, Kazuo, Yanase, Daisuke, Garcia Ramos, Guillermo, Cacho, Bernardo, de la Garza, Lorenzo, Kostera-Pruszczyk, Anne, Lipowska, Marta, Kwiecinski, Hubert, Potulska-Chromik, Anna, Orlowski, Tadeusz, Silva, Ana, Feijo, Marta, Freitas, António, Heckmann, Jeannine, Frost, Andrew, Pan, Edward, Tucker, Lawrence, Rossouw, Johan, Drummond, Fiona, Illa, Isabel, Diaz, Jorge, Leon, Carlos, Yeh, Jiann-Horng, Chiu, Hou-Chang, Hsieh, Yei-San, Witoonpanich, Rawiphan, Tunlayadechanont, Supoch, Attanavanich, Sukasom, Verschuuren, Jan, Straathof, Chiara, Titulaer, Maarten, Versteegh, Michel, Pels, Arda, Krum, Yvonne, Buckley, Camilla, Leite, M. Isabel, Vincent, Angela, Hilton-Jones, David, Ratnatunga, Chandi, Farrugia, Maria, Petty, Richard, Overell, James, Kirk, Alan, Gibson, Andrew, McDermott, Chris, Hopkinson, David, Lecky, Bryan, Watling, David, Marshall, Dot, Saminaden, Sam, Davies, Deborah, Dougan, Charlotte, Sathasivam, Siva, Page, Richard, Sussman, Jon, Ealing, John, Krysiak, Peter, Amato, Anthony, Salajegheh, Mohammad, Jaklitsch, Michael, Roe, Kristen, Ashizawa, Tetsuo, Smith, Robert Glenn, Zwischenberg, Joseph, Stanton, Penny, Barboi, Alexandru, Jaradeh, Safwan, Tisol, William, Gasparri, Mario, Haasler, George, Yellick, Mary, Dennis, Cedric, Barohn, Richard, Pasnoor, Mamatha, Dimachkie, Mazen, McVey, April, Gronseth, Gary, Dick, Arthur, Kramer, Jeffrey, Currence, Melissa, Herbelin, Laura, Belsh, Jerry, Li, George, Langenfeld, John, Mertz, Mary Ann, Benatar, Michael, Harrison, Taylor, Force, Seth, Usher, Sharon, Beydoun, Said, Lin, Frank, DeMeester, Steve, Akhter, Salem, Malekniazi, Ali, Avenido, Gina, Crum, Brian, Milone, Margherita, Cassivi, Stephen, Fisher, Janet, Ciafaloni, Emma, Heatwole, Chad, Watson, Thomas, Hilbert, James, Smirnow, Alexis, Distad, B. Jane, Weiss, Michael, Wood, Douglas, Haug, Joanna, Ernstoff, Raina, Cao, Jingyang, Chmielewski, Gary, Welsh, Robert, Duris, Robin, Gutmann, Laurie, Pawar, Gauri, Graeber, Geoffrey Marc, Altemus, Patricia, Nance, Christopher, Gutmann, Ludwig, Jackson, Carlayne, Grogan, Patrick, Calhoon, John, Kittrell, Pamela, Myers, Deborah, Hayat, Ghazala, Naunheim, Keith, Eller, Susan, Holzemer, Eve, Katirji, Bashar, Alshekhlee, Amer, Robke, Jason, Karlinchak, Brenda, Katz, Jonathan, Miller, Robert, Roan, Ralph, Forshew, Dallas, Kissel, John, Elsheikh, Bakri, Ross, Patrick, Chelnick, Sharon, Lewis, Richard, Acsadi, Agnes, Baciewicz, Frank, Masse, Stacey, Massey, Janice, Juel, Vern, Onaitis, Mark, Lowe, James, Lipscomb, Bernadette, Mozaffar, Tahseen, Thai, Gaby, Milliken, Jeffrey, Martin, Veronica, Karayan, Ronnie, Muley, Suraj, Parry, Gareth, Shumway, Sara, Oh, Shin, Claussen, Gwen, Lu, Liang, Cerfolio, Robert, Young, Angela, Morgan, Marla, Pascuzzi, Robert, Kincaid, John, Kesler, Kenneth, Guingrich, Sandy, Michaels, Angi, Phillips, Lawrence, Burns, Ted, Jones, David, Fischer, Cindy, Pulley, Michael, Berger, Alan, D'Agostino, Harry, Smith, Lisa, Rivner, Michael, Pruitt, Jerry, Landolfo, Kevin, Hillman, Demetric, Shaibani, Aziz, Sermas, Angelo, Ruel, Ross, Ismail, Farah, Sivak, Mark, Goldstein, Martin, Camunas, Jorge, Bratton, Joan, Tandan, Rup, Panitch, Hill, Leavitt, Bruce, Jones, Marilee, Muppidi, Srikanth, Vernino, Steven, Nations, Sharon, Meyer, Dan, Gorham, Nina, Wolfe, Gil I, Kaminski, Henry J, Aban, Inmaculada B, Cea, J Gabriel, Heckmann, Jeannine M, King, John O, Beydoun, Said R, Chalk, Colin H, Barboi, Alexandru C, Amato, Anthony A, Shaibani, Aziz I, Lecky, Bryan R F, Pulley, Michael T, Rivner, Michael H, Kostera-Pruszczyk, Anna, Pascuzzi, Robert M, Jackson, Carlayne E, Verschuuren, Jan J G M, Massey, Janice M, Kissel, John T, Werneck, Lineu C, Barohn, Richard J, Silvestri, Nicholas J, Conwit, Robin, Sonett, Joshua R, Jaretzki, Alfred, III, and Cutter, Gary R

Published
2019
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30. HLA and KIR genetic association and NK cells in anti-NMDAR encephalitis.

Author

Peris Sempere, Vicente, Guo Luo, Muñiz-Castrillo, Sergio, Pinto, Anne-Laurie, Picard, Géraldine, Rogemond, Véronique, Titulaer, Maarten J., Finke, Carsten, Leypoldt, Frank, Kuhlenbäumer, Gregor, Jones, Hannah F., Dale, Russell C., Binks, Sophie, Irani, Sarosh R., Bastiaansen, Anna E., de Vries, Juna M., de Bruijn, Marienke A. A. M., Roelen, Dave L., Tae-Joon Kim, and Kon Chu

Subjects
ANTI-NMDA receptor encephalitis, KILLER cells, MONONUCLEAR leukocytes, GENOME-wide association studies, HLA histocompatibility antigens, METHYL aspartate receptors
Abstract

Introduction: Genetic predisposition to autoimmune encephalitis with antibodies against N-methyl-D-aspartate receptor (NMDAR) is poorly understood. Given the diversity of associated environmental factors (tumors, infections), we hypothesized that human leukocyte antigen (HLA) and killer-cell immunoglobulin-like receptors (KIR), two extremely polymorphic gene complexes key to the immune system, might be relevant for the genetic predisposition to anti-NMDAR encephalitis. Notably, KIR are chiefly expressed by Natural Killer (NK) cells, recognize distinct HLA class I allotypes and play a major role in anti-tumor and anti-infection responses. Methods: We conducted a Genome Wide Association Study (GWAS) with subsequent control-matching using Principal Component Analysis (PCA) and HLA imputation, in a multi-ethnic cohort of anti-NMDAR encephalitis (n=479); KIR and HLA were further sequenced in a large subsample (n=323). PCAcontrolled logistic regression was then conducted for carrier frequencies (HLA and KIR) and copy number variation (KIR). HLA-KIR interaction associations were also modeled. Additionally, single cell sequencing was conducted in peripheral blood mononuclear cells from 16 cases and 16 controls, NK cells were sorted and phenotyped. Results: Anti-NMDAR encephalitis showed a weak HLA association with DRB1*01:01~DQA1*01:01~DQB1*05:01 (OR=1.57, 1.51, 1.45; respectively), and DRB1*11:01 (OR=1.60); these effects were stronger in European descendants and in patients without an underlying ovarian teratoma. More interestingly, we found increased copy number variation of KIR2DL5B (OR=1.72), principally due to an overrepresentation of KIR2DL5B*00201. Further, we identified two allele associations in framework genes, KIR2DL4*00103 (25.4% vs. 12.5% in controls, OR=1.98) and KIR3DL3*00302 (5.3% vs. 1.3%, OR=4.44). Notably, the ligands of these KIR2DL4 and KIR3DL3, respectively, HLA-G and HHLA2, are known to act as immune checkpoint with immunosuppressive functions. However, we did not find differences in specific KIR-HLA ligand interactions or HLA-G polymorphisms between cases and controls. Similarly, gene expression of CD56dim or CD56bright NK cells did not differ between cases and controls. Discussion: Our observations for the first time suggest that the HLA-KIR axis might be involved in anti-NMDAR encephalitis. While the genetic risk conferred by the identified polymorphisms appears small, a role of this axis in the pathophysiology of this disease appears highly plausible and should be analyzed in future studies. [ABSTRACT FROM AUTHOR]

Published
2024
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33. The extinguish trial: A phase-2B randomized placebo-controlled trial of inebilizumab in anti-NMDA receptor encephalitis

Author

Wong, Ka-Ho, primary, Day, Gregory, additional, Torner, James, additional, Cudkowicz, Merit, additional, Coffey, Christopher, additional, Cho, Sophie, additional, Utz, Ursula, additional, Klawiter, Eric, additional, Singleton, J., additional, Mitchell, Dana, additional, Cahalan, Susannah, additional, Fedler, Janel, additional, Ecklund, Dixie, additional, Klements, David, additional, Costigan, Michele, additional, Pearson, Brenda, additional, Steinhart, Erin, additional, Desir, Christina, additional, Titulaer, Maarten, additional, and Clardy, Stacey, additional

Published
2023
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35. Autoimmune Encephalitis Resembling Dementia Syndromes

Author

Bastiaansen, Anna E.M., van Steenhoven, Robin W., de Bruijn, Marienke A.A.M., Crijnen, Yvette S., van Sonderen, Agnes, van Coevorden-Hameete, Marleen H., Nühn, Marieke M., Verbeek, Marcel M., Schreurs, Marco W.J., Sillevis Smitt, Peter A.E., de Vries, Juna M., Jan de Jong, Frank, and Titulaer, Maarten J.

Published
2021
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37. Clinical relevance of distinguishing autoimmune nodopathies from CIDP: longitudinal assessment in a large cohort

Author

Broers, Merel C, primary, Wieske, Luuk, additional, Erdag, Ece, additional, Gürlek, Cemre, additional, Bunschoten, Carina, additional, van Doorn, Pieter A, additional, Eftimov, Filip, additional, Kuitwaard, Krista, additional, de Vries, Juna M, additional, de Wit, Marie-Claire Y, additional, Nagtzaam, Mariska MP, additional, Franken, Suzanne C, additional, Zhu, Louisa, additional, Paunovic, Manuela, additional, de Wit, Maurice, additional, Schreurs, Marco WJ, additional, Lleixà, Cinta, additional, Martín-Aguilar, Lorena, additional, Pascual-Goñi, Elba, additional, Querol, Luis, additional, Jacobs, Bart C, additional, Huizinga, Ruth, additional, and Titulaer, Maarten J, additional

Published
2023
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38. Antibodies Associated With Autoimmune Encephalitis in Patients With Presumed Neurodegenerative Dementia

Author

Bastiaansen, Anna E.M., primary, van Steenhoven, Robin W., additional, te Vaarwerk, Esmee S., additional, van der Flier, Wiesje M., additional, Teunissen, Charlotte, additional, de Graaff, Esther, additional, Nagtzaam, Mariska M.P., additional, Paunovic, Manuela, additional, Franken, Suzanne C., additional, Schreurs, Marco W.J., additional, Leypoldt, Frank, additional, Smitt, Peter A.E., additional, de Vries, Juna M., additional, Seelaar, Harro, additional, van Swieten, John, additional, Jan de Jong, Frank, additional, Pijnenburg, Yolande A.L., additional, and Titulaer, Maarten J., additional

Published
2023
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39. Clinical features, prognostic factors, and antibody effects in anti-mGluR1 encephalitis

Author

Spatola, Marianna, Petit Pedrol, Mar, Maudes, Estibaliz, Simabukuro, Mateus, Muñiz-Castrillo, Sergio, Pinto, Anne-Laurie, Wandinger, Klaus-Peter, Spiegler, Juliane, Schramm, Peter, Dutra, Lívia Almeida, Iorio, Raffaele, Kornblum, Cornelia, Bien, Christian G., Höftberger, Romana, Leypoldt, Frank, Titulaer, Maarten J., Sillevis Smitt, Peter, Honnorat, Jérôme, Rosenfeld, Myrna R., Graus, Francesc, and Dalmau, Josep

Published
2020
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44. Mimics of Autoimmune Encephalitis

Author

Van Steenhoven, Robin W., primary, de Vries, Juna M., additional, Bruijstens, Arlette L., additional, Paunovic, Manuela, additional, Nagtzaam, Mariska M., additional, Franken, Suzanne C., additional, Bastiaansen, Anna E., additional, De Bruijn, Marienke A., additional, Van Sonderen, Agnes, additional, Schreurs, Marco W.J., additional, Gardeniers, Mayke, additional, Verdijk, Robert M., additional, Balvers, Rutger K., additional, Sillevis Smitt, Peter A., additional, Neuteboom, Rinze F., additional, and Titulaer, Maarten J., additional

Published
2023
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45. Autoantibody subclass predominance is not driven by aberrant class switching or impaired B cell development

Author

Paardekooper, Laurent M., primary, Fillié-Grijpma, Yvonne E., additional, van der Sluijs-Gelling, Alita J., additional, Zlei, Mihaela, additional, van Doorn, Remco, additional, Vermeer, Maarten H., additional, Paunovic, Manuela, additional, Titulaer, Maarten J., additional, van der Maarel, Silvère M., additional, van Dongen, Jacques J.M., additional, Verschuuren, Jan J., additional, and Huijbers, Maartje G., additional

Published
2023
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48. Autoimmune Encephalitis

Author

van Sonderen, Agnes, Titulaer, Maarten J., Deisenhammer, Florian, editor, Sellebjerg, Finn, editor, Teunissen, Charlotte E, editor, and Tumani, Hayrettin, editor

Published
2015
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