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

Hesham Abboud, John C Probasco, Sarosh 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 Rojas, Ian Rossman, Sarah Schmitt, Arun Venkatesan, Steven Vernino, Sean J Pittock, Maarten J Titulaer

Journal: Journal of neurology, neurosurgery, and psychiatry 2022;92(7):757-768

PMID: 33649022

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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.

Address: Neurology, Case Western Reserve University, Cleveland, Ohio, USA [email protected].; Multiple Sclerosis and Neuroimmunology Program, University Hospitals of Cleveland, Cleveland, Ohio, USA.; Neurology, Johns Hopkins Medicine, Baltimore, Maryland, USA.; Oxford Autoimmune Neurology Group, John Radcliffe Hospital, Oxford, UK.; Neurology, Washington University in St Louis, St Louis, Missouri, USA.; Neurology, University of Maryland School of Medicine, Baltimore, Maryland, USA.; Neurology, Rosalind Franklin University of Medicine and Science, North Chicago, Illinois, USA.; Billings Clinic, Billings, Montana, USA.; Neurology, Minas Gerais Federal University Risoleta Tolentino Neves Hospital, Belo Horizonte, MG, Brazil.; Neuroimmunology Group, The University of Sydney Faculty of Medicine and Health, Sydney, New South Wales, Australia.; Neurology, La Paz University Hospital General Hospital Department of Neurology, Madrid, Madrid, Spain.; Neurology, Mayo Clinic, Rochester, Minnesota, USA.; Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.; Neurology, Cleveland Clinic, Cleveland, Ohio, USA.; Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.; Neurology, Seoul National University College of Medicine, Seoul, Republic of Korea.; Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.; Neurology, Harvard Medical School, Boston, Massachusetts, USA.; Pediatric Neurology, Geisinger Commonwealth School of Medicine, Scranton, Pennsylvania, USA.; Neurology, Sanatorio de La Trinidad Mitre, Buenos Aires, Argentina.; Neurology, Favaloro Foundation, Buenos Aires, Argentina.; Neuro-developmental Science Center, Akron Children's Hospital, Akron, Ohio, USA.; Neurology, MUSC, Charleston, South Carolina, USA.; Neurology, UT Southwestern, Dallas, Texas, USA.; Neurology, Erasmus Medical Center, Rotterdam, Zuid-Holland, The Netherlands.

DOI: 10.1136/jnnp-2020-325300