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DOI: 10.1590/0004-282X-ANP-2022-S122
A critical review and update on autoimmune encephalitis: understanding the alphabet soup
Revisão crítica e atualizações em encefalites autoimunes: entendendo a sopa de letrinhas
ABSTRACT
Autoimmune encephalitis (AE) comprises a group of diseases mediated by antibodies against neuronal cell surface or synaptic antigens, such as ion channels or neurotransmitter receptors. New clinical syndromes and their associated antibodies were and are still being characterized over the last two decades. The fact that their main clinical features are interdisciplinary, - encompassing neuropsychiatric symptoms, cognitive dysfunction, epileptic seizures, movement and sleep disorders - has led to a surge of interest in this field. Some of these diseases present with a well-defined syndrome, being recognizable on clinical grounds. Correct diagnosis is important since AE are potentially treatable diseases, despite their severity. On the other hand, an increasing number of neuronal antibodies being described casts doubt upon the way we should utilize antibody testing and interpret results. In this article we review, summarize and update the current knowledge on antibody mediated encephalitis.
RESUMO
As encefalites autoimunes compreendem um grupo de doenças mediadas por anticorpos contra antígenos de superfície neuronal ou sinapse, como canais iônicos ou receptores de neurotransmissores. Novas síndromes clínicas e os anticorpos a elas associados foram e ainda estão sendo caracterizados ao longo das últimas duas décadas. Dado que suas principais características clínicas são interdisciplinares, isto é, incluem -se sintomas neuropisquiátricos, disfunção cognitiva, crises epilépticas, distúrbio do movimento e do sono, há uma grande onda de interesse sobre esse campo de conhecimento. Algumas dessas doenças apresentam-se com uma síndrome bem definida, sendo possível reconhecê-las clinicamente. Diagnosticá-las corretamente é importante uma vez que se trata de doenças potencialmente tratáveis apesar da gravidade que lhes é característica. Por outro lado, o número crescente de anticorpos sendo descritos causam dúvida frequente sobre qual o melhor teste a solicitar e como interpretá-los. Nós aqui apresentamos uma revisão atualização resumida do conhecimento atual sobre as encefalites mediadas por anticorpos.
Keywords:
Encephalitis - Anti-N-Methyl-D-Aspartate Receptor Encephalitis - Limbic Encephalitis - Paraneoplastic Syndromes, Nervous System.Palavras-chave:
Encefalite - Encefalite Anti Receptor de N-Metil-D-Aspartato - Encefalite Límbica - Síndromes Paraneoplásicas do Sistema Nervoso.Authors’ contributions:
MMS: conceptualization, data curation, formal analysis, investigation, methodology, writing-original draft, writing-review & editing; GDS: conceptualization, formal analysis, methodology, validation, visualization; LHMC: conceptualization, formal analysis, methodology, validation, reviewing and editing; LTL: conceptualization, validation, visualization.
Publication History
Received: 30 March 2022
Accepted: 29 April 2022
Article published online:
06 February 2023
© 2022. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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