Journal article : Review
Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management
- Abstract:
- 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.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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(Preview, Version of record, pdf, 1.2MB, Terms of use)
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- Publisher copy:
- 10.1136/jnnp-2020-325300
Authors
+ Medical Research Council
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- Funder identifier:
- https://ror.org/03x94j517
- Grant:
- MR/V007173/1
- Publisher:
- BMJ Publishing Group
- Journal:
- Journal of Neurology, Neurosurgery and Psychiatry More from this journal
- Volume:
- 92
- Issue:
- 7
- Pages:
- 757-768
- Place of publication:
- England
- Publication date:
- 2021-03-01
- Acceptance date:
- 2021-01-10
- DOI:
- EISSN:
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1468-330X
- ISSN:
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0022-3050
- Pmid:
-
33649022
- Language:
-
English
- Keywords:
- Subtype:
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Review
- Pubs id:
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1166239
- Local pid:
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pubs:1166239
- Deposit date:
-
2025-02-07
- ARK identifier:
Terms of use
- Copyright holder:
- Abboud et al
- Copyright date:
- 2021
- Rights statement:
- © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
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