Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management.

Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management.
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DOI:
10.1136/jnnp-2020-325300
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发表时间:
2021-07
期刊:
Journal of neurology, neurosurgery, and psychiatry
影响因子:
--
通讯作者:
Autoimmune Encephalitis Alliance Clinicians Network
Autoimmune Encephalitis Alliance Clinicians Network
中科院分区:
其他
文献类型:
--
作者:
Abboud H;Probasco JC;Irani S;Ances B;Benavides DR;Bradshaw M;Christo PP;Dale RC;Fernandez-Fournier M;Flanagan EP;Gadoth A;George P;Grebenciucova E;Jammoul A;Lee ST;Li Y;Matiello M;Morse AM;Rae-Grant A;Rojas G;Rossman I;Schmitt S;Venkatesan A;Vernino S;Pittock SJ;Titulaer MJ;Autoimmune Encephalitis Alliance Clinicians Network

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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.
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