Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management
2021/03/01 by Hesham Abboud, John C. Probasco, John C Probasco +35 · 26 citations
Medicine · #Autoimmune Neurological Disorders and Treatments #Peripheral Neuropathies and Disorders #Herpesvirus Infections and Treatments
paper · pdf · doi:10.1136/jnnp-2020-325300
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.
Cited by
- Guidelines on the Use of Therapeutic Apheresis in Clinical Practice – Evidence‐Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue
- Prüfer algebraic spaces
- Autoimmune Disorders of the Nervous System: Pathophysiology, Clinical Features, and Therapy. [europepmc]
- Anti-Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic Acid Receptor Encephalitis: A Review. [europepmc]
- Review and Updates on the Treatment of Refractory and Super Refractory Status Epilepticus. [europepmc]
- Update on Paraneoplastic Cerebellar Degeneration. [europepmc]
- Immunotherapy for Refractory Autoimmune Encephalitis. [europepmc]
- Antibody Therapies in Autoimmune Encephalitis. [europepmc]
- Cortical and Subcortical Dysmetabolism Are Dynamic Markers of Clinical Disability and Course in Anti-LGI1 Encephalitis. [europepmc]
- Paraneoplastic and Other Autoimmune Encephalitides: Antineuronal Antibodies, T Lymphocytes, and Questions of Pathogenesis. [europepmc]
- Recurrence of Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Cohort Study in Central China. [europepmc]
- Autoimmune and Paraneoplastic Chorea: A Review of the Literature. [europepmc]
- Immunity, Ion Channels and Epilepsy. [europepmc]
- Relapses of Anti-NMDAR, Anti-GABABR and Anti-LGI1 Encephalitis: A Retrospective Cohort Study. [europepmc]
- Treatment Options in Refractory Autoimmune Encephalitis. [europepmc]
- Autoimmune Encephalitis Misdiagnosis in Adults. [europepmc]
- Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management Strategies. [europepmc]
- Seizures, Epilepsy, and NORSE Secondary to Autoimmune Encephalitis: A Practical Guide for Clinicians. [europepmc]
- Immune Mechanisms in Epileptogenesis: Update on Diagnosis and Treatment of Autoimmune Epilepsy Syndromes. [europepmc]
- Comparison of IVIg and TPE efficacy in the treatment of neurological disorders: a systematic literature review. [europepmc]
- New insights into the treatment of meningoencephalomyelitis of unknown origin since 2009: A review of 671 cases. [europepmc]
- Autoimmune Encephalitis with Antibodies: Anti-NMDAR, Anti-AMPAR, Anti-GQ1b, Anti-DPPX, Anti-CASPR2, Anti-LGI1, Anti-RI, Anti-Yo, Anti-Hu, Anti-CV2 and Anti-GABAAR, in the Course of Psychoses, Neoplastic Diseases, and Paraneoplastic Syndromes. [europepmc]
- Interleukin 6: at the interface of human health and disease. [europepmc]
- Autoimmune encephalitis: what the radiologist needs to know. [europepmc]
- Case report: Rapid symptom relief in autoimmune encephalitis with efgartigimod: a three-patient case series. [europepmc]
- Targeting of Extracellular Vesicle-Based Therapeutics to the Brain. [europepmc]
- Autoimmune encephalitis-associated epilepsy. [europepmc]
Related