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3. 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

5. 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

6. Systematic Review and Meta-analysis of Foodborne Tick-Borne Encephalitis, Europe, 1980-2021

Author

Elbaz, Meital, Gadoth, Avi, Shepshelovich, Daniel, Shasha, David, Rudoler, Nir, and Paran, Yael

Subjects
Epidemics -- Statistics -- Risk factors -- Causes of -- Europe, Tick-borne encephalitis -- Statistics -- Risk factors -- Causes of, Foodborne diseases -- Statistics -- Risk factors -- Causes of, Health
Abstract

Tick-borne encephalitis (TBE) is a viral infection of the central nervous system (CNS) caused by tick-borne encephalitis virus (TBEV). TBE occurs mainly in eastern, central, and northern Europe and in [...]

Published
2022
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8. Epidemiology and Characteristics of Autoimmune Encephalitis and Comparison to Infectious Causes – Experience from a Large Tertiary Center (P7-14.001)

Author

Segal, Yahel, primary, Rotschild, Ofer, additional, Mina, Yair, additional, Levison, Tal, additional, Paran, Yael, additional, Maayan, Gadi, additional, Aizenstein, Orna, additional, Halutz, Ora, additional, Dekel, Michal, additional, Cohen-Poradosu, Ronit, additional, Ashkenazi, Adi, additional, Moreno, Itamar, additional, Alcalay, Yifat, additional, and Gadoth, Avi, additional

Published
2024
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10. Area postrema syndrome: Frequency, criteria, and severity in AQP4-IgG-positive NMOSD

Author

Shosha, Eslam, Dubey, Divyanshu, Palace, Jacqueline, Nakashima, Ichiro, Jacob, Anu, Fujihara, Kazuo, Takahashi, Toshiyuki, Whittam, Daniel, Leite, Maria Isabel, Misu, Tatsuro, Yoshiki, Takai, Messina, Silvia, Elsone, Liene, Majed, Masoud, Flanagan, Eoin, Gadoth, Avi, Huebert, Carey, Sagen, Jessica, Greenberg, Benjamin M, Levy, Michael, Banerjee, Aditya, Weinshenker, Brian, and Pittock, Sean J

Subjects
Clinical Research, Adolescent, Adult, Aged, Aquaporin 4, Area Postrema, Cohort Studies, Female, Humans, Immunoglobulin G, Immunotherapy, International Cooperation, Magnetic Resonance Imaging, Male, Middle Aged, Nausea, Neuromyelitis Optica, Surveys and Questionnaires, Vomiting, Young Adult, Clinical Sciences, Neurosciences, Cognitive Sciences, Neurology & Neurosurgery
Abstract

ObjectiveTo define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4-immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS).MethodsAn International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG-positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) score. The diagnostic criteria, severity scale, and immunotherapy response was applied to a prospective validation cohort of patients from multiple centers.ResultsAnalysis of an international database for AQP4-IgG-seropositive NMOSD (n = 430) revealed a high prevalence of isolated APS attacks (onset 7.1%-10.3%; subsequent 9.4%-14.5%) across continents. For 100 patients with 157 episodes of APS, nausea (n = 127, 81%) lasted for a median of 14 days (range 2-365), vomiting (113, 72%) with a median of 5 episodes/d (2-40) lasted 1-20 minutes, and hiccups (102, 65%) lasted a median of 14 days (2-365). Symptoms consistently and completely resolved following immunotherapy. Data were used to propose APS diagnostic criteria and repurpose PUQE score (hiccups severity grade based on symptom duration). The clinical utility was demonstrated in a prospective validation cohort.ConclusionIsolated APS attacks are frequently encountered both at onset and during the NMOSD course. The diagnostic criteria proposed here will assist clinicians in recognizing APS. Diagnosis of an APS attack earlier than 48 hours is possible if a dorsal medulla lesion is detected. Accurate diagnosis and evaluation of APS attack severity will assist in outcome measurement in NMOSD clinical trials.

Published
2018

13. New Insights on DR and DQ Human Leukocyte Antigens in Anti-LGI1 Encephalitis

Author

Segal‬‏, Yahel, primary, Nisnboym, Michal, additional, Regev, Keren, additional, Arnon, Karni, additional, Kolb, Hadar, additional, Fahoum, Firas, additional, Aizenstein, Orna, additional, Paran, Yael, additional, Louzoun, Yoram, additional, Israeli, Sapir, additional, Loewenthal, Ron, additional, Svetlitzky, Nina, additional, Alcalay, Yifat, additional, Raphael, Itay, additional, and Gadoth, Avi, additional

Published
2023
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16. Posttransplant autoimmune encephalitis

Author

Cohen, Devon A., Lopez-Chiriboga, A. Sebastian, Pittock, Sean J., Gadoth, Avi, Zekeridou, Anastasia, Boilson, Barry A., Hogan, William J., Poterucha, John J., Wilton, Katelynn M., Lin, Yi, and Flanagan, Eoin P.

Published
2018
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19. Expanding Our Knowledge of the Immunogenetic Characteristics of Anti-LGI1 Encephalitis—A Study of an Israeli Cohort Suggests Additional Significant HLA Associations With DQ Alleles

Author

Segal, Yahel, primary, Nisnboym, Michal, additional, Regev, Keren, additional, Karni, Arnon, additional, Kolb, Hadar, additional, Fahoum, Firas, additional, Aizenstein, Orna, additional, Paran, Yael, additional, Louzoun, Yoram, additional, Israeli, Sapir, additional, Loewenthal, Ron, additional, Svetlicky, Nina, additional, Alcalay, Yifat, additional, and Gadoth, Avi, additional

Published
2022
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24. Case Report: Anti-LGI1 Encephalitis Following COVID-19 Vaccination

Author

Zlotnik, Yair, Gadoth, Avi, Abu-Salameh, Ibrahim, Horev, Anat, Novoa, Rosa, and Ifergane, Gal

Subjects
COVID-19 vaccination adverse effect, COVID-19 vaccination, anti-LGI1 autoimmune encephalitis, Immunology, Immunology and Allergy, case report, autoimmune encephalitis (AE), Immunologic diseases. Allergy, RC581-607
Abstract

Anti-leucine rich glioma inactivated 1 (LGI1) autoimmune encephalitis (AE) is characterized by cognitive impairment or rapid progressive dementia, psychiatric disorders, faciobrachial dystonic seizures (FBDS) and refractory hyponatremia. Since December 2020, millions of people worldwide have been vaccinated against COVID-19. Several soft neurological symptoms like pain, headache, dizziness, or muscle spasms are common and self-limited adverse effects after receiving the COVID-19 vaccine. However, several major neurological complications, despite the unproven causality, have been reported since the introduction of the COVID-19 vaccine. Herein, we describe a 48 years old man presenting with rapidly progressive cognitive decline and hyponatremia diagnosed with anti LGI1 AE, occurring shortly after the second dose of mRNA COVID -19 vaccine and possibly representing a severe adverse event related to the vaccination. Response to high dose steroid therapy was favorable. As millions of people worldwide are currently receiving COVID-19 vaccinations, this case should serve to increase the awareness for possible rare autoimmune reactions following this novel vaccination in general, and particularly of anti-LGI1 AE.

Published
2022

26. LGI1 antibody encephalitis: acute treatment comparisons and outcome

Author

Rodriguez, Andrew, primary, Klein, C J, additional, Sechi, Elia, additional, Alden, Eva, additional, Basso, Michael R, additional, Pudumjee, Shehroo, additional, Pittock, Sean J, additional, McKeon, Andrew, additional, Britton, Jeffrey W, additional, Lopez-Chiriboga, A Sebastian, additional, Zekeridou, Anastasia, additional, Zalewski, Nicholas L, additional, Boeve, B F, additional, Day, Gregory S, additional, Gadoth, Avi, additional, Burkholder, David, additional, Toledano, Michel, additional, Dubey, Divyanshu, additional, and Flanagan, Eoin P, additional

Published
2021
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33. Clinical spectrum of high-titre GAD65 antibodies

Author

Budhram, Adrian, primary, Sechi, Elia, additional, Flanagan, Eoin P, additional, Dubey, Divyanshu, additional, Zekeridou, Anastasia, additional, Shah, Shailee S, additional, Gadoth, Avi, additional, Naddaf, Elie, additional, McKeon, Andrew, additional, Pittock, Sean J, additional, and Zalewski, Nicholas L, additional

Published
2021
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35. Benefits of eculizumab in AQP4+ neuromyelitis optica spectrum disorder: Subgroup analyses of the randomized controlled phase 3 PREVENT trial

Author

Palace, Jacqueline, primary, Wingerchuk, Dean M., additional, Fujihara, Kazuo, additional, Berthele, Achim, additional, Oreja-Guevara, Celia, additional, Kim, Ho Jin, additional, Nakashima, Ichiro, additional, Levy, Michael, additional, Terzi, Murat, additional, Totolyan, Natalia, additional, Viswanathan, Shanthi, additional, Wang, Kai-Chen, additional, Pace, Amy, additional, Yountz, Marcus, additional, Miller, Larisa, additional, Armstrong, Róisín, additional, Pittock, Sean, additional, Muñoz, Daniel Julio, additional, Amor, Jorge David, additional, Bocchiardo, Carolina, additional, Danielle, Julieta Iourno, additional, Laffue, Alfredo, additional, Obregon, Carolina Daniela Diaz, additional, Paez, Maria Fernanda, additional, Perez, Roberto Martin, additional, Rocchi, Viviana Ana Maria, additional, Teijeiro, Loreley Deborah, additional, Gómez, Jesica, additional, Villa, Andres Maria, additional, Aguirre, Florencia, additional, Fernández, Victoria Carla, additional, Goicoechea, Ramon F., additional, Melamud, Luciana, additional, Stillman, Ana, additional, de Virgiliis, Mariana, additional, Cassara, Fatima Pagani, additional, Cordoba, Marta, additional, Gutierrez, Maria Teresa, additional, Ingolotti, Mariana, additional, Larripa, Natalia, additional, Lupinacci, Anahi, additional, Arroyo, Josefina, additional, Romano, Alejandra, additional, Torres, Mariana Foa, additional, Ballario, Carlos Héctor, additional, Chiesa, Ana Elisa, additional, Gómez, Hernán Gustavo, additional, Lattini, Hernán Gabriel, additional, Mainella, Carolina Natalia, additional, Bolner, Gisel Edith, additional, Eschoyez, María Soledad, additional, Broadley, Simon Andrew, additional, Heshmat, Saman, additional, Sabet, Arman, additional, Swayne, Andrew, additional, Freeman, Susan, additional, Sanchez, Sofia Jimenez, additional, Shuey, Neil, additional, Dalic, Linda, additional, French, Ann, additional, Kuma, Guru, additional, Laing, Joshua, additional, Law, Lai Yin, additional, MacIntyre, Jennifer, additional, Neal, Andrew, additional, Plummer, Christopher, additional, Ramachandran, Prashanth, additional, Sedal, Leslie, additional, Wilson, Ian, additional, Winkel, Antony, additional, Zhang, Wenwen, additional, Chen, Tina, additional, Watts, Rani, additional, Barnett, Michael, additional, Barton, Joshua, additional, Beadnall, Heidi, additional, Garber, Justin, additional, Hardy, Todd Andrew, additional, Trewin, Benjamin, additional, Taha, Marinda, additional, Walters, Deleni, additional, Sieger, Federico Arturo Silva, additional, Alfonso, Nhora Patricia Ruiz, additional, Camacho, Anna Maria Pinzon, additional, Moreno, Alexander Pabón, additional, Prad, Jorge Armando Castellanos, additional, Rueda, Adriana Paola Duarte, additional, Castillo, Tatiana, additional, Gonzalez, Karol Melissa Castillo, additional, Pico, Martha Yolanda Moreno, additional, Castill, Judith, additional, Habek, Mario, additional, Adamec, Ivan, additional, Barun, Barbara, additional, Crnosija, Luka, additional, Gabelic, Tereza, additional, Nytrova, Petra, additional, Krasulova, Eva, additional, Pavlickova, Jana, additional, Tyblova, Michaela, additional, Zubkova, Jana, additional, Petersen, Thor, additional, Dale, Gro Helen, additional, Rasmussen, Peter Vestergaard, additional, Stilund, Morten, additional, Svendsen, Kristina Bacher, additional, Brandt, Vivi, additional, Collongues, Nicolas, additional, Fleury, Marie-Celine, additional, Kremer, Laurent, additional, Bendele, Sandrine, additional, Neff, Valérie, additional, Diem, Ricarda, additional, Platten, Michael, additional, Berberich, Anne, additional, Jansen, Jonabelle, additional, Jaschoneck, Hannah, additional, Wildemann, Brigitte, additional, Aures, Ursula, additional, Brandenburger, Tanja, additional, Haut, Tanja, additional, Fernández, Maria-Lourdes Treceno, additional, Aly, Lilian, additional, Brinkhoff, Kirsten, additional, Buck, Dorothea, additional, Golkowski, Daniel, additional, Hermisson, Mirjam, additional, Hoshi, Muna-Miriam, additional, Kaminski, Miriam, additional, Kowarik, Markus Christian, additional, Kronsbein, Helena, additional, Lehmann-Horn, Klaus, additional, Pongratz, Viola Maria, additional, Schweiker, Andreas, additional, Leddy, Lisa-Ann, additional, Mueller, Silvia, additional, Obergfell, Kim, additional, Wanka, Marion, additional, Zettl, Uwe Klaus, additional, Klinke, Jan, additional, Loebermann, Micha, additional, Meister, Stefanie, additional, Rimmele, Florian, additional, Winkelmann, Alexander, additional, Schroeder, Ina, additional, Lau, Alexander Yuk-Lun, additional, Au, Lisa Wing-Chi, additional, Fan, Florence Sin-Ying, additional, Ip, Vincent Hing-Lung, additional, Ma, Karen Ka-Yan, additional, Ma, Sze-Ho, additional, Mok, Vincent Chung-Tong, additional, Au, Cheryl Chung-Kwan, additional, Kwan, Pauline Wing-Lam, additional, Patti, Francesco, additional, Caramma, Andrea Salvatore, additional, Chisari, Clara Grazia, additional, Fermo, Salvatore Lo, additional, Messina, Silvia, additional, Projetto, Maria, additional, Caserta, Cinzia, additional, Filla, Alessandro, additional, Costabile, Teresa, additional, Pane, Chiara, additional, Sacca, Francesco, additional, Marsili, Angela, additional, Puorro, Giorgia, additional, Bergamaschi, Roberto, additional, Berra, Eliana, additional, Mallucci, Giulia, additional, Fattore, Cinzia, additional, Gasperini, Claudio, additional, Galgani, Simonetta, additional, Haggiag, Shalom, additional, Ruggieri, Serena, additional, Vento, Claudio, additional, Quartuccio, Esmeralda Maria, additional, Pozzilli, Carlo, additional, Barletta, Valeria Teresa, additional, Borriello, Giovanna, additional, De Giglio, Laura, additional, Marinelli, Fabiana, additional, Tasillo, Miriam, additional, Amadori, Alessandra, additional, Fischetti, Mariano, additional, Gurreri, Flavia, additional, Mori, Masahiro, additional, Masuda, Hiroki, additional, Ohtani, Ryohei, additional, Sekiguchi, Yukari, additional, Uchida, Tomohiko, additional, Uzawa, Akiyuki, additional, Ito, Hiromi, additional, Kabasawa, Emi, additional, Kaneko, Yoko, additional, Matsushita, Takuya, additional, Matsuse, Dai, additional, Murai, Hiroyuki, additional, Hayashi, Shintaro, additional, Masak, Katsuhisa, additional, Ogata, Hidenori, additional, Shinoda, Koji, additional, Uehara, Taira, additional, Watanabe, Mitsuru, additional, Yamaguchi, Hiroo, additional, Yamasaki, Ryo, additional, Yonekawa, Tomomi, additional, Jingu, Maki, additional, Nagano, Makiko, additional, Nakamura, Yumiko, additional, Sano, Yoshiko, additional, Araki, Manabu, additional, Lin, Youwei, additional, Mori, Madoka, additional, Mukai, Yohei, additional, Sano, Terunori, additional, Sato, Wakiro, additional, Gogun, Naoya, additional, Maeda, Yuriko, additional, Nishimoto, Asami, additional, Tsukamoto, Sachiko, additional, Yanagi, Ritsuko, additional, Saida, Takahiko, additional, Nakamura, Shinichi, additional, Nasu, Tetsuya, additional, Saida, Kyoko, additional, Shikata, Yuko, additional, Kodani, Yoshimi, additional, Saeki, Megumi, additional, Sawada, Yukako, additional, Yoshikawa, Hiroo, additional, Kimura, Takashi, additional, Nishi, Masamitsu, additional, Sakamoto, Shun, additional, Ukon, Shinichiro, additional, Watanabe, Shohei, additional, Ebisuya, Saori, additional, Kimura, Nami, additional, Matsuura, Manami, additional, Morisaki, Yukie, additional, Muroi, Yoshiko, additional, Onishi, Kuniko, additional, Oshima, Ikuko, additional, Washino, Yuki, additional, Yamashita, Tomomi, additional, Misu, Tatsuro, additional, Kaneko, Kimihiko, additional, Kato, Masaaki, additional, Kuroda, Hiroshi, additional, Kurosawa, Kazuhiro, additional, Nishiyama, Shuhei, additional, Ono, Hirohiko, additional, Takai, Yoshiki, additional, Abe, Keiko, additional, Hoshi, Hitomi, additional, Jinushi, Mari, additional, Oyama, Azusa, additional, Sakuma, Motonari, additional, Sawada, Yuko, additional, Ishibashi, Satoru, additional, Yokota, Takanori, additional, Nishida, Yoichiro, additional, Ozaki, Kokoro, additional, Sanjo, Nobuo, additional, Sato, Nozomu, additional, Denno, Fuki, additional, Hiraki, Haruko, additional, Matsubara, Yumi, additional, Kanda, Takashi, additional, Abe, Masaaki, additional, Honda, Masaya, additional, Kawai, Motoharu, additional, Koga, Michiaki, additional, Maeda, Toshihiko, additional, Ogasawara, Junichi, additional, Omoto, Masatoshi, additional, Sano, Yasuteru, additional, Sato, Ryota, additional, Shimizu, Fumitaka, additional, Arima, Hideki, additional, Fukui, Sachie, additional, Ishikawa, Yoshiko, additional, Koyama, Tomoko, additional, Shimose, Shigemi, additional, Shinozaki, Hirokazu, additional, Watanabe, Masanori, additional, Yasuda, Sachi, additional, Yoshiwaka, Chieko, additional, Adenan, Suffian, additional, Aris, Mohd Azman M, additional, Mawardi, Ahmad Shahir bin, additional, Adnan, Muhammad Al Hafiz, additional, Munusamy, Nanthini, additional, Razali, Siti Nur Omaira, additional, Somasundram, Punitha, additional, Hyun, Jae Won, additional, Jeong, In Hye, additional, Kim, Su-Hyun, additional, Shin, Hyun-June, additional, Yoo, Ji Sung, additional, Jang, HyunMin, additional, Joung, AeRan, additional, Kim, Byung-Jo, additional, Baek, Seol-Hee, additional, Kim, Jung Bin, additional, Kim, Yoo Hwan, additional, Koo, Yong Seo, additional, Lee, Chan Nyoung, additional, Seok, Hung Youl, additional, Hwang, Jinhee, additional, Kim, Sung Min, additional, Ahn, So Hyun, additional, Choi, Kyomin, additional, Choi, Seok-Jin, additional, Kim, Jun-Soon, additional, Kwon, Young Nam, additional, Shin, Je-Young, additional, Kwon, Hyeonju, additional, Kim, Byoung Joon, additional, Cho, Eun Bin, additional, Cho, Hye-Jin, additional, Choi, Misong, additional, Kim, DongSun, additional, Kim, Ju Hyeon, additional, Ki, SeungJu, additional, Lee, Hye Lim, additional, Lee, Kwang-Ho, additional, Min, Ju-Hong, additional, Park, Ji-Hyung, additional, Seok, Jinmyoung, additional, Choi, Eunhwa, additional, Park, Sang Ae, additional, Kim, Seung Min, additional, Jeong, Ha-Neul, additional, Jeongbin, Bong, additional, Jung, Jin Woo, additional, Kim, Seung Woo, additional, Kim, Yool-hee, additional, Lee, Hyung Seok, additional, Shin, Ha Young, additional, Jung, Yeon, additional, Kim, Min Jung, additional, Lee, Nou Ri, additional, Shin, MiJu, additional, Khabirov, Farit A, additional, Averyanova, Lyudmila, additional, Babicheva, Natalya, additional, Granatov, Eugenii, additional, Kazarov, Sergey, additional, Khaybullin, Timur, additional, Rogozhin, Alexander, additional, Pokhabov, Dmitry V, additional, Abramov, Vladislav, additional, Amelina, Anastasia, additional, Nesteroca, Yulia, additional, Bozhenkina, Tatyana, additional, Boyko, Aleksey N, additional, Demyan, Elena G, additional, Khoroshilova, Inessa, additional, Melnikov, Mikhail, additional, Popova, Ekaterina V, additional, Sharanova, Svetlana N, additional, Shchur, Sergey G, additional, Sazonov, Denis V, additional, Babenko, Larisa, additional, Bayandina, Elena, additional, Yarmoschuk, Asya, additional, Baliazin, Victor A, additional, Baliazina, Elena, additional, Budaeva, Elena, additional, Chernikova, Irina, additional, Goncharova, Zoya, additional, Krasnov, Vladimir, additional, Myatleva, Marina, additional, Rodionova, Olga V., additional, Samulyzhko, Iuliana, additional, Timofeeva, Alla A., additional, Duran, Sabas Boyero, additional, Bilbao, Maria Mar Mendibe, additional, Cuervo, Irene Diaz, additional, Domingo, Jose Maria Losada, additional, Eizaguirre, Amaia Gonzalez, additional, Llona, Jose Eulalio Barcena, additional, Moyano, Roberto Valverde, additional, Bahamonde, Carmen, additional, Lopez, Fernando Sanchez, additional, Morales, Raquel Pinar, additional, Morales, Eduardo Agüera, additional, Roman, Carmen Bahamonde, additional, Sepulveda, Juan Jose Ochoa, additional, Valero, Maria del Carmen Blanco, additional, Viña, Nazaret Pelaez, additional, Gavilan, Cristina Conde, additional, Sanchez, Ana Maria Jover, additional, Bedmar, Sara Vila, additional, Garcia, Nuria Gonzalez, additional, Garcia, Aida Orviz, additional, Gonzalez-Suarez, Ines, additional, Guillamon, Elena Miñano, additional, Kawiorski, Miguel, additional, Schulz, Elena Guerra, additional, Alonso, Alba Garcia, additional, Perez, Francisco Jesus Lopez, additional, Sarmiento, Marta Palacios, additional, Ayuso, Guillermo Izquierdo, additional, Mascarell, Guillermo Navarro, additional, Camino, Cristina Paramo, additional, Garcia, Asuncion Varas, additional, Mendoza, Yaiza Montserrat, additional, Muñoz, Veronica Ines Vargas, additional, Tonda, Patricia Torres, additional, Tsai, Ching-Piao, additional, Yin, Jiu-Haw, additional, Chen, Mei-Jung, additional, Li, Shan-Ni, additional, Wang, Fei-Ti, additional, Srisuwannanukorn, Suwat, additional, Boonmongkol, Thanatat, additional, Borisutbuathip, Duangporn, additional, Singwicha, Duangkamol, additional, Siritanan, Krittika, additional, Thearapati, Chidchanoke, additional, Sornda, Kwanmuang, additional, Apiwattanakul, Metha, additional, Aungsumart, Saharat, additional, Suanprasert, Narupat, additional, Suksuchano, Kaona, additional, Parkinsee, Nittaya, additional, Kulkantrakorn, Kongkiat, additional, Lolekha, Praween, additional, Potigumjon, Artit, additional, Sukphulloprat, Puchit, additional, Suksasunee, Dararat, additional, Komaratat, Chankawee, additional, Luangtong, Sunattana, additional, Arayawichanont, Arkhom, additional, Konpan, Phanpaphon, additional, Riablershirun, Nathapol, additional, Wiroteurairuang, Thaddao, additional, Jantaweesirirat, Panadda, additional, Kurne, Aslı, additional, Erkent, Irem, additional, Kurt, Ebru Bekircan, additional, Saylam, Ezgi, additional, Caliskan, Yagmur, additional, Orsel, Gulsah, additional, Celik, Yahya, additional, Celebi, Canan, additional, Tekatas, Aslan, additional, Banbal, Tugce, additional, Demir, Gulsen Akman, additional, Altunrende, Burcu, additional, Matur, Zeliha, additional, Topcular, Baris, additional, Elmas, Tules, additional, Gulo, Aysenur, additional, Ozdemir, Selin, additional, Ozkoklesen, Cansu, additional, Ozturk, Mahinur, additional, Yanik, Mertkan, additional, Yildirim, Elif, additional, Tutuncu, Melih, additional, Altintas, Ayse, additional, Cam, Abdulsamet, additional, Elmali, Ayse Deniz, additional, Saip, Sabahattin, additional, Siva, Aksel, additional, Tanrıverdi, Uygur, additional, Uygunoglu, Ugur, additional, Caliskan, Sinem, additional, Gulo, Pinar, additional, Kozig, Esra, additional, Sakiz, Sakine, additional, Sengun, Ihsan Sukru, additional, Idiman, Egemen, additional, Ala, Rahmi Tumay, additional, Arslan, Duygu, additional, Bulut, Utku, additional, Karakaptan, Yasemin, additional, Kaya, Derya, additional, Mehdiyev, Zaur, additional, Balkan, Bengu, additional, Kuku, Berfu, additional, Ozhun, Mujgan, additional, Tuga, Celal, additional, Ugur, Muzeyyen, additional, Efendi, Husnu, additional, Bunul, Sena Destan, additional, Cavus, Hakan, additional, Gorke, Yunus Emre, additional, Kutlu, Ayse, additional, Ozturk, Seda, additional, Sarikaya, Cansu Egilmez, additional, Becerikli, Gulsah, additional, Semiz, Cansu, additional, Tun, Ozlem, additional, Ayer, Sehriban, additional, Onar, Musa Kazim, additional, Ozberk, Mehlika Berra, additional, Sen, Sedat, additional, Cavdar, Tugce Kirbas, additional, Veske, Adife, additional, Boz, Cavit, additional, Altiparmak, Didem, additional, Aydin, Cigdem Ozen, additional, Gazioglu, Sibel, additional, Bekircan, Duygu, additional, Jacob, Anu, additional, Arndt, Heike, additional, Elsone, Liene, additional, Hamid, Shahd Hassan Mahmoud, additional, Whittam, Daniel Hugh, additional, Wilson, Martin, additional, O'Brien, Imelda, additional, Leite, Maria Isabel da Silva, additional, Cruz, Pedro Maria Rodriguez, additional, Jenkins, Damian Robert, additional, Tackley, George, additional, Cavey, Ana, additional, Everett, Rosie, additional, Hodder, Joy, additional, Koelewyn, Abigail, additional, Mowry, Ellen, additional, Royal, Walter, additional, Shin, Robert, additional, Bever, Christopher, additional, Harrison, Daniel, additional, Rus, Horea, additional, Zheng, Wei, additional, Callison, Karen, additional, Naunton, Kerry, additional, Frishberg, Benjamin M, additional, Blumenfeld, Andrew M, additional, Inocelda, Andrew, additional, Korabathina, Kalyani, additional, Lobatz, Michael, additional, Mortin, Melissa M, additional, Oh, Irene J, additional, Rosenberg, Jay H, additional, Sadoff, Mark, additional, Sahagian, Gregory A, additional, Wang, Anchi, additional, Camberos, Yasmin, additional, Sanchez, Guadalupe, additional, Soto, Estela, additional, Nicholas, Jacqueline A, additional, Boster, Aaron, additional, Eubank, Geoffrey, additional, Groezinger, Katy, additional, Lauf, Meghan, additional, Okai, Annette F, additional, Hasan, Rashedul, additional, Khoury, Chaouki, additional, Stokes, Victoria, additional, Clardy, Stacey, additional, Cortez, Melissa, additional, Greenlee, John, additional, Rose, John, additional, Soldan, Mateo Paz, additional, Emett, Amanda, additional, Esquibel, Lawanda, additional, Fagatele, Lilly, additional, Wong, Ka-Ho, additional, Stevens, James C, additional, Banas, Thomas M, additional, Bultemeyer, Marlene C, additional, Haller, Andrea, additional, Manalo, Natalie, additional, Aeschliman, Keri, additional, Kocks, Debi, additional, Racke, Michael, additional, Boster, Aaron Lee, additional, Bowman, Michelle, additional, Imitola, Jaime, additional, Kisanuki, Yasushi, additional, Green, Misty, additional, Scarberry, Stephanie, additional, Lynch, Sharon G, additional, Anderson, Heather S, additional, Gronseth, Gary S, additional, Hammond, Nancy E, additional, Jassam, Yasir N, additional, Mittal, Manoj K, additional, Nashatizadeh, Muhammed M, additional, Levine, Nicholas, additional, Schmidt, Lisa, additional, Sibley, Jill, additional, Whitley, Vonda, additional, Winkley, James, additional, Coleman, Timothy, additional, Cooper, Gregory, additional, Sheffield, Stephanie, additional, Turner, Keri, additional, Galloway, Dana, additional, Tillett, Robert S, additional, Ganesh, Geeta A, additional, Plato, Brian M, additional, Seifert, Tad D, additional, Godwin, Diana, additional, Lockridge, Deborah, additional, Rammohan, Kottil W, additional, Sheremata, William A, additional, Delgado, Silvia, additional, Gonzalez, Jose, additional, Lizarraga, Alexis, additional, Maldonado, Janice, additional, Ortega, Melissa, additional, Tornes, Leticia, additional, Babcock, Yanet, additional, Cailam, Osmara, additional, Campos, Yesica, additional, Couvertier, Irlisse, additional, Daneri, Bettina, additional, Deni, Jeremy, additional, Hernandez, Jeffrey, additional, Jaramillo, Tatiana, additional, Morris, Tenita, additional, Nobel, Daniel, additional, Oliveira, Anjelis, additional, Richardson, Reshma, additional, Rodriguez, Gloria, additional, Romero, Ana, additional, Sandova, Carlos, additional, Sawant, Ruta, additional, Tueros, Lissett, additional, Zetka, Eric S, additional, Zheng, Chao, additional, Jacobs, Daniel H, additional, Easterling, Constance, additional, Fairbank, Jennifer, additional, Iyengar, Revathi, additional, Klafter, Mark, additional, Lindquist, Justin, additional, Sadek, Ahmed H, additional, Toro, Elizabeth Carmona, additional, Verma, Navin, additional, Castro, Brigith Patino, additional, Sukhraj-Singh, Nadia, additional, Berger, Joseph, additional, Williamson, Eric, additional, Chahin, Salim, additional, Jacobs, Dina, additional, Markowitz, Clyde, additional, Dobbins, Jessica, additional, Mace, Lauren, additional, Martin, Maria, additional, Pinckney, Ashley, additional, Roberts, Amber, additional, Zaydan, Islam, additional, Mitchell, Galen W, additional, Heyman, Rock A, additional, Orie, Ryan L, additional, Suski, Valerie R, additional, Oddis, Kerry, additional, Punjack, Darlene, additional, Flanagan, Eoin, additional, Gadoth, Avi, additional, McKeon, Andrew, additional, Tobin, W Oliver, additional, Zekeridou, Anastasia, additional, Dunlay, Katie, additional, Sagen, Jessica, additional, Carter, Jonathan L, additional, Estephan, Bachir, additional, Goodman, Brent P, additional, Snyder, Charlene R Hoffman, additional, Francone, Andrea, additional, Galasky, Irene, additional, Thomas, Martha, additional, Repovic, Pavle, additional, Bowen, James, additional, Mayadev, Angeli, additional, Qian, Peiqing, additional, Courtney, Yuriko, additional, Lennox, Lauren, additional, Naismith, Robert Thomas, additional, Cross, Anne Haney, additional, Evans, Emily, additional, Longbrake, Erin E, additional, Orchard, Megan E, additional, Wu, Gregory F, additional, Heinrich, Linda, additional, Sommers, Susan, additional, Amjad, Faria, additional, Mitchell, Erika, additional, Mora, Carlos, additional, Schreiber, Bethany, additional, Tornatore, Carlo, additional, Carlson, Alexis, additional, McCarthy, Sacha, additional, and Oliver, Alexandria, additional

Published
2021
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36. Area postrema syndrome

Author

Shosha, Eslam, Dubey, Divyanshu, Palace, Jacqueline, Nakashima, Ichiro, Jacob, Anu, Fujihara, Kazuo, Takahashi, Toshiyuki, Whittam, Daniel, Leite, Maria Isabel, Misu, Tatsuro, Yoshiki, Takai, Messina, Silvia, Elsone, Liene, Majed, Masoud, Flanagan, Eoin, Gadoth, Avi, Huebert, Carey, Sagen, Jessica, Greenberg, Benjamin M., Levy, Michael, Banerjee, Aditya, Weinshenker, Brian, and Pittock, Sean J.

Subjects
Article
Abstract

Objective To define the frequency, duration, and severity of intractable nausea, vomiting, or hiccups in aquaporin-4–immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and propose diagnostic criteria and a severity scale for area postrema syndrome (APS). Methods An International NMOSD database was interrogated for frequency of APS. Patients with AQP4-IgG–positive NMOSD completed an APS symptom questionnaire. Nausea and vomiting severity was derived from the Pregnancy-Unique Quantification of Emesis and Nausea (PUQE) score. The diagnostic criteria, severity scale, and immunotherapy response was applied to a prospective validation cohort of patients from multiple centers. Results Analysis of an international database for AQP4-IgG–seropositive NMOSD (n = 430) revealed a high prevalence of isolated APS attacks (onset 7.1%–10.3%; subsequent 9.4%–14.5%) across continents. For 100 patients with 157 episodes of APS, nausea (n = 127, 81%) lasted for a median of 14 days (range 2–365), vomiting (113, 72%) with a median of 5 episodes/d (2–40) lasted 1–20 minutes, and hiccups (102, 65%) lasted a median of 14 days (2–365). Symptoms consistently and completely resolved following immunotherapy. Data were used to propose APS diagnostic criteria and repurpose PUQE score (hiccups severity grade based on symptom duration). The clinical utility was demonstrated in a prospective validation cohort. Conclusion Isolated APS attacks are frequently encountered both at onset and during the NMOSD course. The diagnostic criteria proposed here will assist clinicians in recognizing APS. Diagnosis of an APS attack earlier than 48 hours is possible if a dorsal medulla lesion is detected. Accurate diagnosis and evaluation of APS attack severity will assist in outcome measurement in NMOSD clinical trials.

Published
2018

39. LGI1 antibody encephalitis: acute treatment comparisons and outcome.

Author

Rodriguez, Andrew, Klein, C. J., Sechi, Elia, Alden, Eva, Basso, Michael R., Pudumjee, Shehroo, Pittock, Sean J., McKeon, Andrew, Britton, Jeffrey W., Lopez-Chiriboga, A. Sebastian, Zekeridou, Anastasia, Zalewski, Nicholas L., Boeve, B. F., Day, Gregory S., Gadoth, Avi, Burkholder, David, Toledano, Michel, Dubey, Divyanshu, and Flanagan, Eoin P.

Subjects
ENCEPHALITIS, TREATMENT effectiveness, ELECTRONIC health records, CEREBROSPINAL fluid, SHORT-term memory, IMMUNOGLOBULINS
Abstract

Objective: To compare acute treatment responses and long-term outcome in leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis.Methods: Retrospective case series of 118 patients with LGI1 antibody encephalitis evaluated at Mayo Clinic across all US sites from 1 May 2008 to 31 March 2019. Patient clinical data were identified and analysed through the neuroimmunology laboratory and electronic medical record. LGI1 antibody detection was by cell-based indirect immunofluorescence assay of serum, cerebrospinal fluid or both. Clinical outcomes were faciobrachial dystonic seizure (FBDS) resolution, modified Rankin Scale (mRS) score, Kokmen Short Test of Mental Status (STMS) score (0-38 point scale) and neuropsychometric testing results.Results: Compared with intravenous immunoglobulin (IVIg) (n=21), patients treated with single-agent acute corticosteroids (intravenous, oral or both) (n=49) were more likely to experience resolution of FBDS (61% vs 7%, p=0.002) and improvements in mRS score (ΔmRS score 2 vs 0, p=0.008) and median Kokmen STMS scores (ΔKokmen STMS score 5 points vs 0 points, p=0.01). In 54 patients with long-term follow-up (≥2 years), the median mRS score was 1 (range 0-6) and the median Kokmen STMS score was 36 (range 24-38) after all combinations of immunotherapy. Neuropsychometric testing in 32 patients with long-term follow-up (≥2 years) demonstrated short-term memory impairments in 37%.Conclusions: Corticosteroids appeared more effective acutely than IVIg in improving LGI1 antibody encephalitis in this retrospective comparison of immunotherapies. While improvement with immunotherapy is typical and long-term outcome is favourable, short-term memory deficits are noted in approximately a third of the patients. [ABSTRACT FROM AUTHOR]

Published
2022
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44. Randomized Placebo‐Controlled Trial of Intravenous Immunoglobulin in Autoimmune LGI1/CASPR2 Epilepsy

Author

Dubey, Divyanshu, primary, Britton, Jeffrey, additional, McKeon, Andrew, additional, Gadoth, Avi, additional, Zekeridou, Anastasia, additional, Lopez Chiriboga, Sebastian A., additional, Devine, Michelle, additional, Cerhan, Jane H., additional, Dunlay, Katie, additional, Sagen, Jessica, additional, Ramberger, Melanie, additional, Waters, Patrick, additional, Irani, Sarosh R., additional, and Pittock, Sean J., additional

Published
2019
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46. Myelin Oligodendrocyte Glycoprotein Autoantibody Myelitis; Clinical characteristics, MRI clues and discriminators from other demyelinating myelitis etiologies (S21.005)

Author

Dubey, Divyanshu, primary, Pittock, Sean, additional, Krecke, Karl, additional, Morris, Padraig P., additional, Sechi, Elia, additional, Zalewski, Nicholas, additional, Weinshenker, Brian, additional, Shosha, Eslam, additional, Lucchinetti, Claudia, additional, Fryer, James, additional, Lopez Chiriboga, Alfonso S., additional, Chen, John, additional, Jitprapaikulsan, Jiraporn, additional, McKeon, Andrew, additional, Gadoth, Avi, additional, Keegan, Mark, additional, Tillema, Jan-Mendelt, additional, Naddaf, Elie, additional, Patterson, Marc, additional, Messacar, Kevin, additional, Tyler, Kenneth, additional, and Flanagan, Eoin, additional

Published
2019
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48. Clinical, Radiologic, and Prognostic Features of Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Autoantibody

Author

Dubey, Divyanshu, primary, Pittock, Sean J., additional, Krecke, Karl N., additional, Morris, Padraig P., additional, Sechi, Elia, additional, Zalewski, Nicholas L., additional, Weinshenker, Brian G., additional, Shosha, Eslam, additional, Lucchinetti, Claudia F., additional, Fryer, James P., additional, Lopez-Chiriboga, A. Sebastian, additional, Chen, John C., additional, Jitprapaikulsan, Jiraporn, additional, McKeon, Andrew, additional, Gadoth, Avi, additional, Keegan, B. Mark, additional, Tillema, Jan-Mendelt, additional, Naddaf, Elie, additional, Patterson, Marc C., additional, Messacar, Kevin, additional, Tyler, Kenneth L., additional, and Flanagan, Eoin P., additional

Published
2019
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50. Association of MOG-IgG Serostatus With Relapse After Acute Disseminated Encephalomyelitis and Proposed Diagnostic Criteria for MOG-IgG–Associated Disorders

Author

López-Chiriboga, A. Sebastian, primary, Majed, Masoud, additional, Fryer, James, additional, Dubey, Divyanshu, additional, McKeon, Andrew, additional, Flanagan, Eoin P., additional, Jitprapaikulsan, Jiraporn, additional, Kothapalli, Naga, additional, Tillema, Jan-Mendelt, additional, Chen, John, additional, Weinshenker, Brian, additional, Wingerchuk, Dean, additional, Sagen, Jessica, additional, Gadoth, Avi, additional, Lennon, Vanda A., additional, Keegan, B. Mark, additional, Lucchinetti, Claudia, additional, and Pittock, Sean J., additional

Published
2018
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