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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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Publisher copy:
10.1136/jnnp-2020-325300

Authors

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Role:
Author
ORCID:
0000-0001-5346-8254
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Role:
Author
ORCID:
0000-0003-2095-0786
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Institution:
University of Oxford
Division:
MSD
Department:
Clinical Neurosciences
Oxford college:
Corpus Christi College
Role:
Author
ORCID:
0000-0002-7667-9748

Contributors


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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:
1468-330X
ISSN:
0022-3050
Pmid:
33649022


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