Encefalitis subaguda por anticuerpos anti receptor de N-metil-D-aspartato: Serie de 13 casos pediátricos / Subacute anti-N-methyl-D-aspartate receptor encephalitis: A serie of 13 paediatric cases

Introducción: La encefalitis subaguda por anticuerpos anti-receptor N-metil-Daspartato (NMDA) es un cuadro autoinmune reconocido el año 2007 como entidad clínica y descrito primero en mujeres jóvenes con teratoma ovárico. El año 2009 se describe primera serie pediátrica no relacionada con tumores. Objetivo: Mostrar características clínicas, tratamiento y pronóstico de 13 pacientes con encefalitis por anticuerpos anti receptor NMDA en Chile. Pacientes y método: Se describen 13 niños, 9 varones, de edades comprendidas entre 1-16 años, estudiados entre 2009-2016 en 7 hospitales y diagnosticados entre 2009-2016. Los pacientes fueron estudiados con resonancia magnética cerebral, electroencefalograma y estudio de líquido cefalorraquídeo, citoquímico, bandas oligoclonales y panel viral (anticuerpos y PCR). Todos fueron estudiados para detección de anticuerpos anti receptor NMDA en suero y líquido cefalorraquídeo (LCR) y pesquisa de tumores por imágenes. Resultados: Trece de 13 niños iniciaron la enfermedad con síntomas psiquiátricos y 11/13 tuvieron crisis epilépticas. Posteriormente todos presentaron agitación psicomotora, distonías y discinesias bucolinguales; 11/13 pérdida de lenguaje y 6/13 trastornos autonómicos. Todos ellos tuvieron anticuerpos anti receptor NMDA positivo. El LCR fue normal en 12/13 niños, hubo bandas oligoclonales positivas en 6/10 pacientes, la resonancia cerebral fue normal en todos los niños, el EEG estuvo alterado en 12/13 niños y la SPECT resultó anormal en 6/6 niños. Doce de 13 niños recibieron metilprednisolona en bolos 30 mg/kg/3-5 días y 6 recibieron inmunoglobulina 2 g/kg. Doce de 13 niños se recuperaron de 2 a 4 meses después de iniciada la enfermedad. Un niño tuvo recidiva un año después y se recuperó rápidamente. Conclusiones: La encefalitis subaguda por anticuerpos anti-receptor NMDA debe sospecharse en niños con alteraciones psiquiátricas y movimientos anormales. Los estudios funcionales (EEG y SPECT) son valiosos para apoyar el diagnóstico. La detección precoz de esta encefalitis permite la recuperación más rápida de los pacientes.

Introduction: Subacute anti-NMDA receptor encephalitis was recognised in 2007 as a clinical entity, and was first described in young women with ovarian teratoma. The first paediatric series unrelated with tumours was reported in 2009. Objective: To present the clinical features, treatment, and prognosis of 13 patients with anti-NMDA receptor encephalitis in Chile. Patients and method: A description is presented of 13 children, 9 males, aged between 1 and 16 years, diagnosed between 2009 and 2016 in 7 hospitals. All patients were evaluated with cerebral magnetic resonance and electroencephalogram. Cytochemical, oligoclonal bands and virus studies (PCR and antibodies) were performed in cerebrospinal fluid. All patients were evaluated in search of anti NMDA receptor in serum and cerebrospinal fluid. Tumor imaging studies were performed in all children. Results: All children began the disease with psychiatric symptoms, and 11/13 had seizures. All of them subsequently presented with psychomotor agitation, dystonia, and bucolingual dyskinesias, with 11/13 loss of language and 6/13 autonomic disorders. All of them (13/13) had positive anti-NMDA receptor antibodies. CSF was normal in 12/13 children, positive oligoclonal bands in 6/10 patients, normal brain resonance in 13/13 children, EEG changes in 11/13 children, and abnormal SPECT in 6/6 children. A methylprednisolone bolus of 30 mg/kg was given for 3-5 days to 12/13 children, and 6 received immunoglobulin 2 g/kg. The large majority (12/13) of children recovered 1-4 months after disease onset. One child had a recurrence one year later, and recovered quickly. Conclusions: Subacute encephalitis due to NMDA anti-receptor antibodies should be suspected in children with psychiatric disorders and abnormal movements. Functional studies, such as EEG and SPECT are valuable diagnostic support. Early detection of this encephalitis leads to a faster recovery of patients.

[Subacute anti-N-methyl-D-aspartate receptor encephalitis. A serie of 13 paediatric cases]. / Encefalitis subaguda por anticuerpos anti receptor de N-metil-D-aspartato. Serie de 13 casos pediátricos.

INTRODUCTION: Subacute anti-NMDA receptor encephalitis was recognised in 2007 as a clinical entity, and was first described in young women with ovarian teratoma. The first paediatric series unrelated with tumours was reported in 2009. OBJECTIVE: To present the clinical features, treatment, and prognosis of 13 patients with anti-NMDA receptor encephalitis in Chile. PATIENTS AND METHOD: A description is presented of 13 children, 9 males, aged between 1 and 16 years, diagnosed between 2009 and 2016 in 7 hospitals. All patients were evaluated with cerebral magnetic resonance and electroencephalogram. Cytochemical, oligoclonal bands and virus studies (PCR and antibodies) were performed in cerebrospinal fluid. All patients were evaluated in search of anti NMDA receptor in serum and cerebrospinal fluid. Tumor imaging studies were performed in all children. RESULTS: All children began the disease with psychiatric symptoms, and 11/13 had seizures. All of them subsequently presented with psychomotor agitation, dystonia, and bucolingual dyskinesias, with 11/13 loss of language and 6/13 autonomic disorders. All of them (13/13) had positive anti-NMDA receptor antibodies. CSF was normal in 12/13 children, positive oligoclonal bands in 6/10 patients, normal brain resonance in 13/13 children, EEG changes in 11/13 children, and abnormal SPECT in 6/6 children. A methylprednisolone bolus of 30mg/kg was given for 3-5 days to 12/13 children, and 6 received immunoglobulin 2g/kg. The large majority (12/13) of children recovered 1-4 months after disease onset. One child had a recurrence one year later, and recovered quickly. CONCLUSIONS: Subacute encephalitis due to NMDA anti-receptor antibodies should be suspected in children with psychiatric disorders and abnormal movements. Functional studies, such as EEG and SPECT are valuable diagnostic support. Early detection of this encephalitis leads to a faster recovery of patients.