Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. Issue 7 (1st March 2021)

• Record Type: Journal Article
• Authors: 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
• Other Names: author non-byline.; Tarawneh Rawan author non-byline.; Van Mater Heather author non-byline.; Muscal Eyal author non-byline.; Ruhoy Ilene author non-byline.; Anziska Yaacov author non-byline.; Longbrake Erin author non-byline.; Benseler Susa author non-byline.; Wang Cynthia author non-byline.; Apperson Michelle author non-byline.; Iorio Raffaele author non-byline.; Simabukuro Mateus Mistieri author non-byline.; Zhong Ning author non-byline.; Rüegg Stephan author non-byline.; Piquet Amanda author non-byline.; Kuo Jonathan author non-byline.; Konuskan Bahadir author non-byline.; Frid Elena author non-byline.; Deng Joseph author non-byline.; Mitchell Wendy author non-byline.; Mooneyham GenaLynne author non-byline.; Estiasari Riwanti author non-byline.; Chiba Yuhei author non-byline.; Alarcio Melanie author non-byline.; Han Velda author non-byline.; Williams Jon author non-byline.; Sweeney Michael author non-byline.; Cellucci Tania author non-byline.; Blackburn Kyle author non-byline.; Klein-Gitelman Marisa author non-byline.; Santoro Jonathan D author non-byline.; Suarez Raymond author non-byline.; Irazuzta Jose author non-byline.; Brod Staley author non-byline.; Hyslop Ann author non-byline.; Manibog Katrina author non-byline.; Escudero Domingo author non-byline.; Noland William author non-byline.; Clardy Stacey author non-byline.; Mar Soe author non-byline.; Kilgo William author non-byline.;
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 92:Issue 7(2021)
• Page Start: 757
• Page End: 768
• Publication Date: 2021-03-01
• Subjects: autoimmune encephalitis -- paraneoplastic syndrome -- neuroimmunology; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2020-325300
• Languages: English
• ISSNs: 0022-3050

Abstract

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.