Encefalites autoimunes: uma revisão sobre diagnóstico e tratamento

Dutra, Lívia Almeida; Abrantes, Fabiano; Toso, Fabio Fieni; Pedroso, José Luiz; Barsottini, Orlando Graziani Povoas; Hoftberger, Romana

doi:10.1590/0004-282X20170176

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View and Review • Arq. Neuro-Psiquiatr. 76 (1) • Jan 2018 • https://doi.org/10.1590/0004-282X20170176 copiar

Encefalites autoimunes: uma revisão sobre diagnóstico e tratamento

Autoria SCIMAGO INSTITUTIONS RANKINGS

RESUMO

As encefalites autoimunes (EAI) são a principal causa de encefalite não-infecciosa. As manifestações neurológicas são variadas, incluindo alterações comportamentais ou psiquiátricas, disautonomia, transtornos do movimento e epilepsia. Habitualmente a instalação dos sintomas ocorre em até 6 semanas, de forma aguda ou subaguda. As EAI podem ser desencadeadas por tumores, quadros infecciosos virais ou ainda apresentar etiologia criptogênica. Este artigo revisa as principais EAI, estratégias de diagnóstico e tratamento.

encefalites; anticorpos antineoplásicos; status epiléptico; encefalite antirreceptor de N-Metil-D-Aspartato; imunoglobulinas; rituximab

Diagnosis can be made when all four of the following criteria have been met:
1 – Subacute onset (rapid progression of less than three months) of working memory deficits, seizures, or psychiatric symptoms suggesting involvement of the limbic system
2 – Bilateral brain abnormalities on MRI T2-weighted FLAIR sequence restricted to the medial temporal lobes
3 – At least one of the following:
EEG with epileptic or slow-wave activity involving the temporal lobes
CSF pleocytosis
4 – Reasonable exclusion of alternative causes

Diagnostic criteria for possible autoimmune encephalitis (all three of the following criteria met):
1 – Subacute onset (rapid progression of less than three months) of working memory deficits (short-term memory loss), altered mental status (decreased level of consciousness, lethargy or personality changes), or psychiatric symptoms
2 – At least one of the following:
New focal CNS findings
Seizures not explained by previously-known seizure disorder
CSF pleocytosis
MRI suggestive of encephalitis
3 – Reasonable exclusion of alternative causes

Criteria for autoantibody-negative but probable autoimmune encephalitis (all four criteria met):

1 - Subacute onset (rapid progression of less than three months) of working memory deficits (short-term memory loss), altered mental status (decreased level of consciousness, lethargy or personality changes), or psychiatric symptoms
2 – Exclusion of well-defined syndromes of autoimmune encephalitis (typical limbic encephalitis, Bickerstaff, brainstem encephalitis, acute disseminated encephalomyelitis)
3 – Absence of well-characterized autoantibodies in serum and CSF, and at least two of the following criteria:
MRI abnormalities suggestive of autoimmune encephalitis
CSF pleocytosis, oligoclonal bands or elevated CSF IgG index, or both
Brain biopsy showing inflammatory infiltrates and excluding other disorders
4 – Reasonable exclusion of alternative causes

Probable anti-NMDA receptor encephalitis
Diagnosis require all three of the following criteria
1 – Less than three months of at least four of the six following major group of symptoms:
Abnormal (psychiatric) behavior or cognitive dysfunction
Speech dysfunction (pressured speech, verbal reduction, mutism)
Seizures
Movement disorder, dyskinesias, or rigidity/abnormal postures
Decreased level of consciousness
Autonomic dysfunction or central hypoventilation
2 – At least one of the following laboratory study results:
Abnormal EEG (focal or diffuse slow or disorganized activity, epileptic activity, or extreme delta brush)
CSF with pleocytosis or oligoclonal bands
3 – Reasonable exclusion of other disorders
Diagnosis can also be made in the presence of three of the above groups of symptoms accompanied by a systemic teratoma
Definite anti-NMDA receptor encephalitis
Diagnosis can be made in the presence of one or more of the six major groups of symptoms and IgG anti-GluN1 antibodies, after reasonable exclusion of other disorders

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[1] Correspondence:Lívia Almeida Dutra; Rua Pedro de Toledo, 650; 04039-002 São Paulo SP, Brasil; E-mail: liviaadutra@hotmail.com