RT Journal Article SR Electronic T1 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management JF Journal of Neurology, Neurosurgery & Psychiatry JO J Neurol Neurosurg Psychiatry FD BMJ Publishing Group Ltd SP 757 OP 768 DO 10.1136/jnnp-2020-325300 VO 92 IS 7 A1 Abboud, Hesham A1 Probasco, John C A1 Irani, Sarosh A1 Ances, Beau A1 Benavides, David R A1 Bradshaw, Michael A1 Christo, Paulo Pereira A1 Dale, Russell C A1 Fernandez-Fournier, Mireya A1 Flanagan, Eoin P A1 Gadoth, Avi A1 George, Pravin A1 Grebenciucova, Elena A1 Jammoul, Adham A1 Lee, Soon-Tae A1 Li, Yuebing A1 Matiello, Marcelo A1 Morse, Anne Marie A1 Rae-Grant, Alexander A1 Rojas, Galeno A1 Rossman, Ian A1 Schmitt, Sarah A1 Venkatesan, Arun A1 Vernino, Steven A1 Pittock, Sean J A1 Titulaer, Maarten J A1 , YR 2021 UL http://jnnp.bmj.com/content/92/7/757.abstract AB 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.