Abstract
Autoimmune encephalitis are one of the emergent causes of subacute changes in the level of consciousness, behavior, cognitive impairment and seizures, mainly in young people. They are a consequence of inflammation or dysfunction of parts of the brain caused by antibodies against specific brain antigens, usually located in the limbic system, resulting in clinical presentation as a limbic encephalitis. The objectives of this article are to show the clinical presentation, complementary studies and treatment of this entity, considering that the patient's prognostic depends on a high level of clinical suspicion, and on an early initiation of immunosuppressive therapy. We did a nonsystematic review of the literature on autoimmune encephalitis between 2005 and 2017. We conclude that the prevalence of autoimmune encephalitis is increasing, even surpassing infectious causes of encephalitis in developed countries. Clinical presentation includes sub-acute cognitive and behavioral impairment, with or without alterations in consciousness and seizures. Fever and inflammation of the cerebrospinal fluid are less common than in the infectious causes but psychiatric symptoms are more frequent. There are specific clinical presentations according to the particular type of antigen/antibody present, which also determines the association with cancer, constituting a paraneoplastic syndrome only in some cases. Immunosuppressive therapy has been standardized in steps, and should be initiated early to improve prognosis.
Highlights
Durante la realización de este trabajo Carolina Delgado recibió financiamiento del proyecto anillo ACT1403 PIA CONICYT Ninguno de los autores tiene otro conflicto de interés
En una cohorte entre el 2007-2011 del “Proyecto de encefalitis de California”, la encefalitis por AC anti receptor de N-metil-D aspartato (R-NMDA) fue la primera causa de encefalitis (41% del total), superando inclusive a las causas virales individuales en los menores de 30 años (38% enterovirus, 9% virus Herpes Simplex tipo 1 (VHS-1))[8], estimándose que la encefalitis por AC anti R-NMDA representa al menos 1% de los pacientes jóvenes en cuidados intensivos[6]
Erazo R, González J, Quintanilla C, Devaud C, Gayoso C, Toledo X, et al Encefalitis subaguda por anticuerpos anti receptor de N-metil-D-aspartato: Serie de 13 casos pediátricos
Summary
Autoimmune encephalitis are one of the emergent causes of subacute changes in the level of consciousness, behavior, cognitive impairment and seizures, mainly in young people. They are a consequence of inflammation or dysfunction of parts of the brain caused by antibodies against specific brain antigens, usually located in the limbic system, resulting in clinical presentation as a limbic encephalitis. Hay algunos criterios muy sensibles, pero poco específicos como los de “encefalitis autoinmune posible” (Tabla 1B), categoría en la que pueden coincidir fácilmente cuadros de origen primariamente psiquiátrico, lo cual limita su validez, pero da énfasis al inicio precoz del tratamiento inmunosupresor una vez descartadas las causas infecciosas y metabólicas[4]. Finalmente hicimos un resumen solo de los cuadros clínicos que se manifiestan principalmente con “alteración del estado mental y/o alteración cognitiva y/o cambios en la conducta”, excluyendo a trastornos que se presentan con síntomas de compromiso motor y sensitivo primariamente[4,5]
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