Encephalitis are an inflammatory processes of various origin, among which include autoimmune origin. The identification of antibodies against the N-methyl-D-aspartate, allowed clinical immunological characterization of an entity susceptible to immunomodulatory therapy. Originally described in young women associated with ovarian teratoma, is now a recognized entity in children even in the absence of detectable tumors. The aim of the study was conducted through review of medical records, was to describe the clinical, developmental and findings in further studies of eleven children with confirmed diagnosis of this entity through identification of specific antibodies. All debuted with psychiatric symptoms in nine associating seizures, and two extrapyramidal movements. In the evolution of language all had commitment nine severe autonomic symptoms, one with hypoventilation and requirements of ARM. Brain MRI was abnormal in three. Eight had voltage EEG asymmetry and / or amplitude, three of them had spikes. Six had CSF pleocytosis and three of seven positive oligoclonal bands. Five IgM serology for mycoplasma were positive. CPK increase occurred in conjunction with antisychotics in five. With immunomodulatory treatment, five had complete recovery three behavioral disorders / cognitive deficits and one severe. A patient's clinical picture resolved without treatment. In any associated tumor was detected. We conclude that in front of a child with acute encephalopathy and clinical support this entity after infectious cause were ruled out, immunomodulatory therapy should be started early, avoid the use of antipsychotic drugs and search for possible hidden tumors.

Las encefalitis son procesos inflamatorios de diverso origen, entre las cuales se incluyen las autoinmunes. La identificación de anticuerpos contra el receptor N-metil-D-aspartato permitió la caracterización clínico-inmunológica de una entidad susceptible a tratamiento inmunomodulador. Descripta originalmente en mujeres jóvenes, asociada a teratoma de ovario, es hoy una entidad reconocida en niños aun en ausencia de tumores detectables. El objetivo del trabajo, realizado a través de la revisión de historias clínicas, fue describir las características clínicas, evolutivas y los hallazgos en estudios complementarios de once niños con diagnóstico confirmado de esta entidad a través de la identificación de los anticuerpos específicos. Todos debutaron con síntomas psiquiátricos, en nueve de ellos asociando a convulsiones, y dos a movimientos extrapiramidales. En su evolución, todos presentaron compromiso del lenguaje, nueve, síntomas autonómicos graves, en uno con hipoventilación y requerimientos de ARM. La RM de cerebro fue anormal en tres. Ocho tuvieron EEG con asimetría del voltaje y/o amplitud, tres de ellos presentaron espigas. En el LCR seis tuvieron pleocitosis y tres de siete bandas oligoclonales positivas. Cinco, serologías IgM para micoplasma. El aumento de CPK en coincidencia con antipsicóticos ocurrió en cinco. Con el tratamiento inmunomodulador, cinco tuvieron recuperación total, tres, alteraciones conductuales/cognitivas y uno déficits severo. Un paciente resolvió su cuadro clínico sin tratamiento. En ninguno se detectó tumor asociado. Concluimos que frente a un niño con encefalopatía aguda y clínica compatible con esta entidad, una vez descartada causa infecciosa, se debe comenzar tratamiento inmunomodulador precozmente, evitar la utilización de fármacos antipsicóticos y realizar la búsqueda de posibles tumores ocultos.

As encefalopatias autoimunes constituem um grupo de condições associadas à presença, no soro, de anticorpos contra proteínas de superfície neuronais. Acredita-se que esses anticorpos sejam mediadores da ocorrência da doença, sendo reconhecidos atualmente como causas frequentes de encefalite. Apresentações clínicas características permitem, muitas vezes, predizer o grupo específico de anticorpos subjacentes. Anticorpos contra o complexo VGKF, especialmente LGI1 (leucine-rich glioma-inactivated1), CASPR2 (contactin-associated protein 2) e contactina-2, e contra o receptor NMDA(N-methyl, D-aspartate) são as associações sorológicas mais frequentemente estabelecidas. Na minoria dos casos, pode ser detectado um tumor subjacente. As maiores chances de melhora estão relacionadas à administração precoce de imunoterapia e à remoção do tumor, quando presente. A duração da imunoterapia pode se prolongada e a melhora se correlaciona, muitas vezes, com os níveis séricos de anticorpos. Neste artigo, estão resumidos os avanços recentes nos achados clínicos e laboratoriais neste campo que está em tão rápida expansão.

The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.