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Review
. 2021 Mar 1;92(8):897-907.
doi: 10.1136/jnnp-2020-325302. Online ahead of print.

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

Hesham Abboud  1   2 , John Probasco  3 , Sarosh R Irani  4 , Beau Ances  5 , David R Benavides  6 , Michael Bradshaw  7   8 , Paulo Pereira Christo  9 , Russell C Dale  10 , Mireya Fernandez-Fournier  11 , Eoin P Flanagan  12 , Avi Gadoth  13 , Pravin George  14 , Elena Grebenciucova  15 , Adham Jammoul  14 , Soon-Tae Lee  16 , Yuebing Li  14 , Marcelo Matiello  17   18 , Anne Marie Morse  19 , Alexander Rae-Grant  14 , Galeno Rojas  20   21 , Ian Rossman  22 , Sarah Schmitt  23 , Arun Venkatesan  3 , Steven Vernino  24 , Sean J Pittock  12 , Maarten Titulaer  25 ; Autoimmune Encephalitis Alliance Clinicians Network
Collaborators, Affiliations
Review

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

Hesham Abboud et al. J Neurol Neurosurg Psychiatry. .

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.

Keywords: autoimmune encephalitis; neuroimmunology; paraneoplastic syndrome.

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Conflict of interest statement

Competing interests: HA is a consultant for Roche/Genentech, which manufactures rituximab and tocilizumab that were discussed in this paper. HA is a consultant for Bristol-Myers Squibb, which manufactures cyclophosphamide that is discussed in this paper. SRI is a coapplicant and receives royalties on patent application WO/210/046716 (UK patent No. PCT/GB2009/051441) licensed to Euroimmun for the development of assays for leucine-rich glioma inactivated protein 1 and other voltage-gated potassium channel complex antibodies discussed in this paper. SRI is supported by the BMA Research Grants- Vera Down grant (2013) and Margaret Temple (2017), Epilepsy Research UK (P1201), the Fulbright UK-US commission (MS-Society research award) and by the NIHR Oxford Biomedical Research Centre. The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health. AG has a patent for MAP1B autoantibodies as biomarkers of neurological autoimmunity and small cell lung cancer. S-TL is a consultant for GC Pharma, which manufactures IVIg that was discussed in this paper and for Advanced Neural Technologies which operates several neuronal autoantibody panels. SV receives research support from Quest Laboratories Diagnostics, which offers several commercial neuronal autoantibody panels, and from Genentech (rituximab) and Grifols (IVIG). SJP has a patent Patent# 8,889,102 (Application#12-678350)—Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia issued, and a patent Patent# 9891219B2 (Application#12-573942)—SJP has a patent pending for GFAP, Septin-5, Kelch11 and MAP1B autoantibodies as biomarkers of neurological autoimmunity. Some of these antibodies are discussed in this paper. MJT has filed a patent for methods for typing neurological disorders and cancer, and devices for use therein, and has received an unrestricted research grant from Euroimmun AG.

Figures

Figure 1
Figure 1
Interpretation of the neuronal autoantibody panel. *Anti-Hu (ANNA-1), anti-Ri (ANNA-2), ANNA-3, anti-SOX1 (AGNA), anti-amphiphysin, anti-CRMP-5 (anti-CV2), anti-Yo (PCA-1), PCA-2, high-titre anti-GAD65. **Anti-NMDA-R, anti-LGI1, anti-CASPR2, anti-AMPA-R, anti-GABA-A/B, PCA-Tr, anti-DPPX, anti-mGluR1, anti-mGluR2, anti-mGluR5, anti-IgLON5, anti-AQP4, anti-MOG. ***Non-LGI1 non-CASPR2 anti-VGKC, anti-P/Q VGCC, anti-N VGCC, Ach-b, Ach-M, Ach-G, Striational. Low-titre anti-GAD65 is an antibody against cytoplasmic antigen but is of questionable clinical significance. Adapted with permission from George et al.
Figure 2
Figure 2
Autoimmune Encephalitis Alliance Clinicians Network survey results for periodic cancer screening. AE, autoimmune encephalitis. *Excluding immune checkpoint inhibitors.
See this image and copyright information in PMC

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