Autoimmune encephalitis: Proposed recommendations for symptomatic and long-term management

Hesham Abboud, John Probasco, Sarosh R. Irani, Beau Ances, David R. Benavides, Michael Bradshaw, Paulo Pereira Christo, Russell C. Dale, Mireya Fernandez-Fournier, Eoin P. Flanagan, Avi Gadoth, Pravin George, Elena Grebenciucova, Adham Jammoul, Soon Tae Lee, Yuebing Li, Marcelo Matiello, Anne Marie Morse, Alexander Rae-Grant, Galeno RojasIan Rossman, Sarah Schmitt, Arun Venkatesan, Steven Vernino, Sean J. Pittock, Maarten Titulaer

Research output: Contribution to journalReview articlepeer-review

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. 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.

Original languageEnglish (US)
Pages (from-to)897-907
Number of pages11
JournalJournal of Neurology, Neurosurgery and psychiatry
Volume92
Issue number8
DOIs
StatePublished - Aug 1 2021

Keywords

  • autoimmune encephalitis
  • neuroimmunology
  • paraneoplastic syndrome

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health

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