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CC BY 4.0 · Arq Neuropsiquiatr 2024; 82(07): s00441788586
DOI: 10.1055/s-0044-1788586
Brazilian Academy of Neurology

Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations

Consenso brasileiro sobre o diagnóstico e o tratamento de encefalites autoimunes nas populações adulta e pediátrica
Lívia Almeida Dutra 
1   Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.
,
Pedro Victor de Castro Silva 
1   Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.
,
João Henrique Fregadolli Ferreira 
1   Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.
,
Alexandre Coelho Marques 
1   Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.
,
Fabio Fieni Toso 
1   Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.
,
Claudia Cristina Ferreira Vasconcelos 
2   Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Rio de Janeiro RJ, Brazil.
,
Doralina Guimarães Brum 
3   Universidade Estadual Paulista, Faculdade de Medicina de Botucatu, Departamento de Neurologia, Psicologia e Psiquiatria, Botucatu SP, Brazil.
,
Samira Luisa dos Apóstolos Pereira 
4   Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.
,
Tarso Adoni 
4   Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.
,
Leticia Januzi de Almeida Rocha 
5   Universidade Federal de Alagoas, Hospital Universitário, Maceió AL, Brazil.
,
Leticia Pereira de Brito Sampaio 
6   Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Instituto da Criança, São Paulo SP, Brazil.
,
Nise Alessandra de Carvalho Sousa 
7   Universidade Federal do Amazonas, Manaus AM, Brazil.
,
Renata Barbosa Paolilo 
6   Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Instituto da Criança, São Paulo SP, Brazil.
,
Angélica Dal Pizzol 
8   Hospital Moinhos de Vento, Departamento de Neurologia, Porto Alegre RS, Brazil.
,
Bruna Klein da Costa 
9   Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre RS, Brazil.
10   Santa Casa de Misericórdia de Porto Alegre, Porto Alegre RS, Brazil.
,
Caio César Diniz Disserol 
11   Universidade Federal do Paraná, Hospital das Clínicas, Curitiba PR, Brazil.
12   Instituto de Neurologia de Curitiba, Curitiba PR, Brazil.
,
Camila Pupe 
13   Universidade Federal Fluminense, Niterói RJ, Brazil.
,
Daniel Almeida do Valle 
14   Hospital Pequeno Príncipe, Curitiba PR, Brazil.
,
Denise Sisterolli Diniz 
15   Universidade Federal de Goiás, Goiânia GO, Brazil.
,
Fabiano Ferreira de Abrantes 
16   Universidade Federal de São Paulo, São Paulo SP, Brazil.
,
Felipe da Rocha Schmidt 
17   Universidade do Estado do Rio de Janeiro, Rio de Janeiro RJ, Brazil.
,
Fernando Cendes 
18   Universidade Estadual de Campinas, Campinas SP, Brazil.
,
Francisco Tomaz Meneses de Oliveira 
19   Santa Casa de Misericórdia de São Paulo, São Paulo SP, Brazil.
,
Gabriela Joca Martins 
20   Hospital Geral de Fortaleza, Fortaleza CE, Brazil.
,
Guilherme Diogo Silva 
4   Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.
,
Katia Lin 
21   Universidade Federal de Santa Catarina, Florianópolis SC, Brazil.
,
Lécio Figueira Pinto 
4   Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.
,
Mara Lúcia Schimtz Ferreira Santos 
14   Hospital Pequeno Príncipe, Curitiba PR, Brazil.
,
Marcus Vinícius Magno Gonçalves 
22   Universidade da Região de Joinville, Joinville SC, Brazil.
,
Mariana Braatz Krueger 
23   Hospital Infantil Albert Sabin, Fortaleza CE, Brazil.
,
Michel Elyas Jung Haziot 
19   Santa Casa de Misericórdia de São Paulo, São Paulo SP, Brazil.
,
Orlando Graziani Povoas Barsottini 
16   Universidade Federal de São Paulo, São Paulo SP, Brazil.
,
Osvaldo José Moreira do Nascimento 
13   Universidade Federal Fluminense, Niterói RJ, Brazil.
24   Academia Nacional de Medicina, Rio de Janeiro RJ, Brazil.
,
Paulo Ribeiro Nóbrega 
25   Universidade Federal do Ceará, Fortaleza CE, Brazil.
,
Priscilla Mara Proveti 
26   Universidade de Brasília, Faculdade de Medicina, Brasília DF, Brazil.
,
Raphael Machado do Castilhos 
27   Hospital de Clínicas de Porto Alegre, Porto Alegre RS, Brazil.
,
Vanessa Daccach 
28   Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto SP, Brazil.
,
Felipe von Glehn 
25   Universidade Federal do Ceará, Fortaleza CE, Brazil.
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Abstract

Background Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. The patients are predominantly young, and delays in treatment are associated with worse prognosis.

Objective With the support of the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN) and the Brazilian Society of Child Neurology (Sociedade Brasileira de Neurologia Infantil, SBNI), a consensus on the diagnosis and treatment of AIE in Brazil was developed using the Delphi method.

Methods A total of 25 panelists, including adult and child neurologists, participated in the study.

Results The panelists agreed that patients fulfilling criteria for possible AIE should be screened for antineuronal antibodies in the serum and cerebrospinal fluid (CSF) using the tissue-based assay (TBA) and cell-based assay (CBA) techniques. Children should also be screened for anti-myelin oligodendrocyte glucoprotein antibodies (anti-MOG). Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. Most seizures in AIE are symptomatic, and antiseizure medications may be weaned after the acute stage. In anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the panelists have agreed that oral immunosuppressant agents should not be used. Patients should be evaluated at the acute and postacute stages using functional and cognitive scales, such as the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), the Modified Rankin Scale (mRS), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE).

Conclusion The present study provides tangible evidence for the effective management of AIE patients within the Brazilian healthcare system.

Resumo

Antecedentes Encefalites autoimunes (EAIs) são um grupo de doenças inflamatórias caracterizadas pela presença de anticorpos contra antígenos neuronais e gliais, que ocasionam sintomas psiquiátricos subagudos, queixas de memória e distúrbios anormais do movimento. A maioria dos pacientes é jovem, e o atraso no tratamento está associado a pior prognóstico.

Objetivo Com o apoio da Academia Brasileira de Neurologia (ABN) e da Sociedade Brasileira de Neurologia Infantil (SBNI), desenvolvemos um consenso sobre o diagnóstico e o tratamento da EAIs no Brasil utilizando a metodologia Delphi.

Métodos Um total de 25 especialistas, incluindo neurologistas e neurologistas infantis, foram convidados a participar.

Resultados Os especialistas concordaram que os pacientes com critérios de possíveis EAIs devem ser submetidos ao rastreio de anticorpos antineuronais no soro e no líquido cefalorraquidiano (LCR) por meio das técnicas de ensaio baseado em tecidos (tissue-based assay, TBA, em inglês) e ensaio baseado em células (cell-based assay, CBA, em inglês). As crianças também devem ser submetidas ao rastreio de de anticorpo contra a glicoproteína da mielina de oligodendrócitos (anti-myelin oligodendrocyte glycoprotein, anti-MOG, em inglês). O tratamento deve ser iniciado dentro das primeiras 4 semanas dos sintomas, sendo as opções de primeira linha metilprednisolona combinada com imunoglobulina intravenosa (IGIV) ou plasmaférese. O tratamento de segunda linha inclui rituximabe e ciclofosfamida. Bortezomib e tocilizumab são opções de tratamento de terceira linha. A maioria das crises epilépticas nas EAIs são sintomáticas, e os fármacos anticrise podem ser desmamadas após a fase aguda. Em relação à encefalite antirreceptor de N-metil-D-aspartato (anti-N-methyl-D-aspartate receptor, anti-NMDAR, em inglês), os especialistas concordaram que agentes imunossupressores orais não devem ser usados. Os pacientes devem ser avaliados na fase aguda e pós-aguda mediante escalas funcionais e cognitivas, como Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Modified Rankin Scale (mRS), e Clinical Assessment Scale in Autoimmune Encephalitis (CASE).

Conclusão Esta pesquisa oferece evidências tangíveis do manejo efetivo de pacientes com EAIs no sistema de saúde Brasileiro.

Keywords

Autoimmune Encephalitis - Anti-N-Methyl-D-Aspartate Receptor Encephalitis - Delphi Technique - Rituximab - Tocilizumab

Palavras-chave

Doenças Autoimunes do Sistema Nervoso - Encefalite Antirreceptor de N-Metil-D-Aspartato - Técnica Delphi - Rituximab - Tocilizumab

Authors' Contributions

LAD, PVCS: conceptualization, data curation, formal analysis, investigation, methodology, project administration, supervision, validation, writing of the original draft, and writing – review and editing. JHFF, ACM, FFT, CCFV, DGB, SLAP, TA, LJAR, LPBS, NACS, FVG: conceptualization, methodology, supervision, and writing – review and editing. ADP, BKDC, CCDD, CP, DADV, DSD, FFA, FRS, FTM, GJM, GDS, KL, LFP, MLSFS, MVMG, MBK, MEJH, OGPD, OJMN, PRN, PMP, RMPC, VD: writing – review and editing. All authors approved the final version of the manuscript and agree to be responsible for all aspects of the work.


Supplementary Material



Publication History

Received: 07 January 2024

Accepted: 27 April 2024

Article published online:
01 August 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

Thieme Revinter Publicações Ltda.
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Bibliographical Record
Lívia Almeida Dutra, Pedro Victor de Castro Silva, João Henrique Fregadolli Ferreira, Alexandre Coelho Marques, Fabio Fieni Toso, Claudia Cristina Ferreira Vasconcelos, Doralina Guimarães Brum, Samira Luisa dos Apóstolos Pereira, Tarso Adoni, Leticia Januzi de Almeida Rocha, Leticia Pereira de Brito Sampaio, Nise Alessandra de Carvalho Sousa, Renata Barbosa Paolilo, Angélica Dal Pizzol, Bruna Klein da Costa, Caio César Diniz Disserol, Camila Pupe, Daniel Almeida do Valle, Denise Sisterolli Diniz, Fabiano Ferreira de Abrantes, Felipe da Rocha Schmidt, Fernando Cendes, Francisco Tomaz Meneses de Oliveira, Gabriela Joca Martins, Guilherme Diogo Silva, Katia Lin, Lécio Figueira Pinto, Mara Lúcia Schimtz Ferreira Santos, Marcus Vinícius Magno Gonçalves, Mariana Braatz Krueger, Michel Elyas Jung Haziot, Orlando Graziani Povoas Barsottini, Osvaldo José Moreira do Nascimento, Paulo Ribeiro Nóbrega, Priscilla Mara Proveti, Raphael Machado do Castilhos, Vanessa Daccach, Felipe von Glehn. Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations. Arq Neuropsiquiatr 2024; 82: s00441788586.
DOI: 10.1055/s-0044-1788586
 

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