TY - JOUR
T1 - Autoimmune encephalitis
T2 - Proposed recommendations for symptomatic and long-term 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 - Funding Information:
manufactures rituximab and tocilizumab that were discussed in this paper. HA is a consultant for Bristol-Myers Squibb, which manufactures cyclophosphamide that is discussed in this paper. SRI is a coapplicant and receives royalties on patent application WO/210/046716 (UK patent No. PCT/GB2009/051441) licensed to Euroimmun for the development of assays for leucine-rich glioma inactivated protein 1 and other voltage-gated potassium channel complex antibodies discussed in this paper. SRI is supported by the BMA Research Grants-Vera Down grant (2013) and Margaret Temple (2017), Epilepsy Research UK (P1201), the Fulbright UK-US commission (MS-Society research award) and by the NIHR Oxford Biomedical Research Centre. The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health. AG has a patent for MAP1B autoantibodies as biomarkers of neurological autoimmunity and small cell lung cancer. S-TL is a consultant for GC Pharma, which manufactures IVIg that was discussed in this paper and for Advanced Neural Technologies which operates several neuronal autoantibody panels. SV receives research support from Quest Laboratories Diagnostics, which offers several commercial neuronal autoantibody panels, and from Genentech (rituximab) and Grifols (IVIG). SJP has a patent Patent# 8,889,102 (Application#12-678350)—Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia issued, and a patent Patent# 9891219B2 (Application#12-573942)—SJP has a patent pending for GFAP, Septin-5, Kelch11 and MAP1B autoantibodies as biomarkers of neurological autoimmunity. Some of these antibodies are discussed in this paper. MJT has filed a patent for methods for typing neurological disorders and cancer, and devices for use therein, and has received an unrestricted research grant from Euroimmun AG.
Funding Information:
Funding Open access funding was provided by Wellcome trust fund through Oxford University.
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 -