Autoimmune encephalitis: a review of diagnosis and treatment

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 copy

Autoimmune encephalitis: a review of diagnosis and treatment

Encefalites autoimunes: uma revisão sobre diagnóstico e tratamento

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.

encephalitis; antibodies, neoplasm; status epilepticus; anti-N-Methyl-D-Aspartate receptor encephalitis; immunoglobulin; 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