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Autoimmune Encephalitis: Proposed Best Practice Recommendations for Diagnosis and Acute Management

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Autoimmune Encephalitis: Proposed Best Practice Recommendations for Diagnosis and Acute Management Neuro- inflammation J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2020-325300 on 1 March 2021. Downloaded from Review Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management Hesham Abboud ,1,2 John C Probasco,3 Sarosh 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 J Titulaer ,25 Autoimmune Encephalitis Alliance Clinicians Network ► Additional material is ABSTRACT antibodies against intracellular onconeuronal anti- published online only. To view, The objective of this paper is to evaluate available gens such asANNA-1/anti-Hu. 5 6 These ‘classical’ please visit the journal online (http:// dx. doi. org/ 10. 1136/ evidence for each step in autoimmune encephalitis antibodies are non- pathogenic but represent markers jnnp- 2020- 325300). management and provide expert opinion when of T- cell-mediated immunity against the neoplasm evidence is lacking. The paper approaches autoimmune with secondary response against the nervous For numbered affiliations see encephalitis as a broad category rather than focusing on system. In recent years, an increasing number of end of article. individual antibody syndromes. Core authors from the antibodies targeting neuronal surface or synaptic Autoimmune Encephalitis Alliance Clinicians Network antigens have been recognised such as N-MethylD - Correspondence to copyright. Dr Hesham Abboud, Neurology, reviewed literature and developed the first draft. Where Aspartate Receptor (NMDAR)- antibody and 1 Case Western Reserve evidence was lacking or controversial, an electronic Leucine-richglioma inactivated (LGI1)- antibody. University, Cleveland, Ohio, survey was distributed to all members to solicit individual Most of these surface antibodies have been shown USA; hesham. abboud@ responses. Sixty-eight members from 17 countries to be likely pathogenic and are thought to mediate uhhospitals. org answered the survey. Corticosteroids alone or combined more immunotherapy- responsive forms of AE and SJP and MJT are joint senior with other agents (intravenous IG or plasmapheresis) have less association with tumours. Specific types of authors. were selected as a first- line therapy by 84% of encephalitis can occur in the setting of antibodies responders for patients with a general presentation, against oligodendrocytes (eg, anti- myelin oligoden- Received 2 October 2020 74% for patients presenting with faciobrachial dystonic drocyte glycoprotein (MOG) brainstem enceph- Revised 22 December 2020 Accepted 10 January 2021 seizures, 63% for NMDAR- IgG encephalitis and 48.5% alitis) or astrocytes (eg, anti-aquaporin-4 (AQP4) for classical paraneoplastic encephalitis. Half the diencephalic encephalitis, anti-glial fibrillary acidic http://jnnp.bmj.com/ responders indicated they would add a second- line protein (GFAP) meningoencephalitis). In addition, agent only if there was no response to more than one some AE patients do not have any identifiable anti- first- line agent, 32% indicated adding a second- line bodies (seronegative) representing a disease cate- agent if there was no response to one first- line agent, gory with novel, yet to be identified antibodies while only 15% indicated using a second- line agent in or T- cell mediated disease. Online supplemental all patients. As for the preferred second- line agent, 80% appendix S1 contains a list of the commercially of responders chose rituximab while only 10% chose available neuronal autoantibodies (NAAs). on September 26, 2021 by guest. Protected cyclophosphamide in a clinical scenario with unknown Recent epidemiological studies suggest that AE is antibodies. Detailed survey results are presented in possibly as common as infectious encephalitis with the manuscript and a summary of the diagnostic and an estimated prevalence rate of 13.7/100 000.7 The therapeutic recommendations is presented at the rapidly advancing knowledge of new antibodies conclusion. © Author(s) (or their and their associated syndromes has created a new 8 employer(s)) 2021. Re- use and growing field of autoimmune neurology. permitted under CC BY- NC. No However, advances from the laboratory bench have commercial re- use. See rights not been paralleled by advancement in clinical prac- and permissions. Published by BMJ. INTRODUCTION tice, leading to a large knowledge gap and many Autoimmune encephalitis (AE) comprises a group unanswered questions regarding the acute and To cite: Abboud H, Probasco of non- infectious immune- mediated inflammatory long- term management of AE. The heterogeneity JC, Irani S, et al. J Neurol disorders of the brain parenchyma often involving of AE presentation and findings on ancillary testing Neurosurg Psychiatry Epub ahead of print: [please the cortical or deep grey matter with or without hinder large-scale clinical trials and limit the quality include Day Month Year]. involvement of the white matter, meninges or of evidence behind AE management. doi:10.1136/jnnp-2020- the spinal cord.1–4 The original description of AE The objective of this paper is to evaluate avail- 325300 was based on paraneoplastic conditions related to able evidence for each step in AE management and Abboud H, et al. J Neurol Neurosurg Psychiatry 2021;0:1–12. doi:10.1136/jnnp-2020-325300 1 Neuro- inflammation J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2020-325300 on 1 March 2021. Downloaded from provide expert opinion when evidence is lacking. Although the of 7.3 AE subtypes (range 1–13 subtypes, median 8 subtypes). In turnaround time of commercial NAAs panels may improve in total, 9% of the participating members were affiliated with refer- the near future, currently these results are often unavailable at ence neuroimmunology laboratories with NAAs testing capabil- the time of early evaluation and management. Moreover, current ities. The results of individual survey questions are presented commercial NAAs panels are inherently limited in their ability under the corresponding sections of AE management. The final to diagnose AE, given the ever-growing numbers of antibodies draft was approved by all participating AEACN members after identified and the likelihood of T- cell mediated pathogenesis four rounds of revisions. The paper aimed to answer prespeci- in some cases. Consequently, clinicians have to approach AE fied clinical questions as detailed below. initially as a clinical entity when deciding on investigations and treatment.1 Long- term management can then be modified DATA AVAILABILITY STATEMENT according to the type of antibody identified, if any. Therefore, The results of the survey are partially summarised in figure 1 and the aim of this paper is to emphasise the practical acute and long- the detailed responses of all survey questions are published as term management of AE as a broad category rather than focusing online supplemental document 2. on individual antibody syndromes. Another important goal is to represent the practice of experienced clinicians from different clinical and geographical backgrounds. SECTION 1: DIAGNOSIS OF AE When to suspect AE clinically? A detailed history and examination is the first and most METHODS important step in AE diagnosis. The immune reaction in AE Core authors from the Autoimmune Encephalitis Alliance often results in acute or subacute presentation of a duration Clinicians Network (AEACN) developed the first draft of this less than 3 months.1 Chronic presentations are only seen paper (HA, JCP, SI, RCD, EPF, PG, AJ, YL, AR-G, IR, SJP and in some of these conditions, especially LGI1, Contactin- MJT). The AEACN is comprised of self- identified clinicians with associatedprotein- like 2 (CASPR2), Dipeptidyl- peptidase- interest and clinical expertise in AE management listed by the likeprotein 6 (DPPX) and Glutamicacid decarboxylase 65 AE Alliance, a non- profit organisation founded by AE patients (GAD65)- antibody encephalitis, and should otherwise raise and families to establish a supportive community for patients suspicion of a neurodegenerative disease or other etiolo- and caregivers, enhance clinical collaboration, and facilitate AE gies.9 Likewise, hyperacute presentations are also atypical scientific research. The AEACN includes a multidisciplinary and a vascular aetiology should be considered in those cases. international group of adult and paediatric neurologists, rheu- A recurrent course may point towards an autoimmune aeti- matologists, psychiatrists, neuropsychologists and other special- ology but unlike the typical relapsing-remitting course of ists with real-life experience in AE management. The authors of copyright. multiple sclerosis and systemic inflammatory disorders, AE the first draft reviewed available literature to compile existing relapses are rare and often result from insufficient treatment evidence for every step in AE management. Where evidence was or rapid interruption of immunotherapy. A monophasic lacking or controversial, an electronic survey was distributed to course is more common in idiopathic AE while a progres- all AEACN members to solicit individual responses. The survey sive course may be seen in some paraneoplastic syndromes questions were strategically
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