TY - JOUR
T1 - Autoimmune encephalitis
T2 - Proposed recommendations for symptomatic and long-term management
AU - Abboud, Hesham
AU - Probasco, John
AU - Irani, Sarosh R.
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
N1 - Publisher Copyright:
© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.
PY - 2021/8/1
Y1 - 2021/8/1
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. 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.
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. 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.
KW - autoimmune encephalitis
KW - neuroimmunology
KW - paraneoplastic syndrome
UR - http://www.scopus.com/inward/record.url?scp=85101884180&partnerID=8YFLogxK
U2 - 10.1136/jnnp-2020-325302
DO - 10.1136/jnnp-2020-325302
M3 - Review article
C2 - 33649021
AN - SCOPUS:85101884180
SN - 0022-3050
VL - 92
SP - 897
EP - 907
JO - Journal of Neurology, Neurosurgery and Psychiatry
JF - Journal of Neurology, Neurosurgery and Psychiatry
IS - 8
ER -