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

Author:

Dutra Lívia Almeida1ORCID,Silva Pedro Victor de Castro1ORCID,Ferreira João Henrique Fregadolli1ORCID,Marques Alexandre Coelho1ORCID,Toso Fabio Fieni1ORCID,Vasconcelos Claudia Cristina Ferreira2ORCID,Brum Doralina Guimarães3ORCID,Pereira Samira Luisa dos Apóstolos4ORCID,Adoni Tarso4ORCID,Rocha Leticia Januzi de Almeida5ORCID,Sampaio Leticia Pereira de Brito6ORCID,Sousa Nise Alessandra de Carvalho7ORCID,Paolilo Renata Barbosa6ORCID,Pizzol Angélica Dal8ORCID,Costa Bruna Klein daORCID,Disserol Caio César DinizORCID,Pupe Camila9ORCID,Valle Daniel Almeida do10ORCID,Diniz Denise Sisterolli11ORCID,Abrantes Fabiano Ferreira de12ORCID,Schmidt Felipe da Rocha13ORCID,Cendes Fernando14ORCID,Oliveira Francisco Tomaz Meneses de15ORCID,Martins Gabriela Joca16ORCID,Silva Guilherme Diogo4ORCID,Lin Katia17ORCID,Pinto Lécio Figueira4ORCID,Santos Mara Lúcia Schimtz Ferreira10ORCID,Gonçalves Marcus Vinícius Magno18ORCID,Krueger Mariana Braatz19ORCID,Haziot Michel Elyas Jung15ORCID,Barsottini Orlando Graziani Povoas12ORCID,Nascimento Osvaldo José Moreira doORCID,Nóbrega Paulo Ribeiro20ORCID,Proveti Priscilla Mara21ORCID,Castilhos Raphael Machado do22ORCID,Daccach Vanessa23ORCID,Glehn Felipe von20ORCID

Affiliation:

1. Hospital Israelita Albert Einstein, Instituto do Cérebro, São Paulo, São Paulo SP, Brazil.

2. Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Rio de Janeiro RJ, Brazil.

3. Universidade Estadual Paulista, Faculdade de Medicina de Botucatu, Departamento de Neurologia, Psicologia e Psiquiatria, Botucatu SP, Brazil.

4. Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

5. Universidade Federal de Alagoas, Hospital Universitário, Maceió AL, Brazil.

6. Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Instituto da Criança, São Paulo SP, Brazil.

7. Universidade Federal do Amazonas, Manaus AM, Brazil.

8. Hospital Moinhos de Vento, Departamento de Neurologia, Porto Alegre RS, Brazil.

9. Universidade Federal Fluminense, Niterói RJ, Brazil.

10. Hospital Pequeno Príncipe, Curitiba PR, Brazil.

11. Universidade Federal de Goiás, Goiânia GO, Brazil.

12. Universidade Federal de São Paulo, São Paulo SP, Brazil.

13. Universidade do Estado do Rio de Janeiro, Rio de Janeiro RJ, Brazil.

14. Universidade Estadual de Campinas, Campinas SP, Brazil.

15. Santa Casa de Misericórdia de São Paulo, São Paulo SP, Brazil.

16. Hospital Geral de Fortaleza, Fortaleza CE, Brazil.

17. Universidade Federal de Santa Catarina, Florianópolis SC, Brazil.

18. Universidade da Região de Joinville, Joinville SC, Brazil.

19. Hospital Infantil Albert Sabin, Fortaleza CE, Brazil.

20. Universidade Federal do Ceará, Fortaleza CE, Brazil.

21. Universidade de Brasília, Faculdade de Medicina, Brasília DF, Brazil.

22. Hospital de Clínicas de Porto Alegre, Porto Alegre RS, Brazil.

23. Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto SP, Brazil.

Abstract

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.

Publisher

Georg Thieme Verlag KG

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