TY - JOUR
T1 - Autoimmune encephalitis
T2 - Proposed best practice recommendations for diagnosis and acute management
AU - Autoimmune Encephalitis Alliance Clinicians Network
AU - Abboud, Hesham
AU - Probasco, John C.
AU - Irani, Sarosh
AU - Ances, Beau
AU - Benavides, David R.
AU - Bradshaw, Michael
AU - Christo, Paulo Pereira
AU - Dale, Russell C.
AU - Fernandez-Fournier, Mireya
AU - Flanagan, Eoin P.
AU - Gadoth, Avi
AU - George, Pravin
AU - Grebenciucova, Elena
AU - Jammoul, Adham
AU - Lee, Soon Tae
AU - Li, Yuebing
AU - Matiello, Marcelo
AU - Morse, Anne Marie
AU - Rae-Grant, Alexander
AU - Rojas, Galeno
AU - Rossman, Ian
AU - Schmitt, Sarah
AU - Venkatesan, Arun
AU - Vernino, Steven
AU - Pittock, Sean J.
AU - Titulaer, Maarten J.
AU - Tarawneh, Rawan
AU - Van Mater, Heather
AU - Muscal, Eyal
AU - Ruhoy, Ilene
AU - Anziska, Yaacov
AU - Longbrake, Erin
AU - Benseler, Susa
AU - Wang, Cynthia
AU - Apperson, Michelle
AU - Iorio, Raffaele
AU - Simabukuro, Mateus Mistieri
AU - Zhong, Ning
AU - Rüegg, Stephan
AU - Piquet, Amanda
AU - Kuo, Jonathan
AU - Konuskan, Bahadir
AU - Frid, Elena
AU - Deng, Joseph
AU - Mitchell, Wendy
AU - Mooneyham, Gena Lynne
AU - Estiasari, Riwanti
AU - Chiba, Yuhei
AU - Alarcio, Melanie
AU - Han, Velda
N1 - Publisher Copyright:
© 2021 BMJ Publishing Group. All rights reserved.
PY - 2021/7/1
Y1 - 2021/7/1
N2 - 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.
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.
KW - autoimmune encephalitis
KW - neuroimmunology
KW - paraneoplastic syndrome
UR - http://www.scopus.com/inward/record.url?scp=85101872216&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85101872216&partnerID=8YFLogxK
U2 - 10.1136/jnnp-2020-325300
DO - 10.1136/jnnp-2020-325300
M3 - Review article
C2 - 33649022
AN - SCOPUS:85101872216
SN - 0022-3050
VL - 92
SP - 757
EP - 768
JO - Journal of Neurology, Neurosurgery and Psychiatry
JF - Journal of Neurology, Neurosurgery and Psychiatry
IS - 7
ER -