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
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.