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Introduccion. La encefalitis autoinmune contra receptores N-metil-D-aspartato (NMDA) se diagnostica cada vez con mayor frecuencia en la edad pediatrica. Debe sospecharse en ninos con sintomatologia psiquiatrica, encefalopatia, movimientos anormales o crisis epilepticas. Los casos paraneoplasicos son menos frecuentes que en adultos. Caso clinico. Nino de 2,5 anos con cuadro encefalopatico subagudo que comenzo con crisis epilepticas seguidas de alteraciones del comportamiento, regresion neurologica, discinesias e insomnio. El estudio del liquido cefalorraquideo fue normal, en la resonancia magnetica craneal existia una lesion periventricular focal y captacion leptomeningea difusa y los electroencefalogramas seriados revelaron una actividad delta de gran amplitud intercalada con actividad epileptiforme intercritica generalizada. Recibio tratamiento empirico con altas dosis de corticoides e inmunoglobulinas intravenosas sin respuesta. Tras demostrarse la positividad de anticuerpos contra el receptor NMDA se inicio la plasmaferesis, con la que experimento una mejoria rapida y espectacular. Tras mas de 18 meses de seguimiento, sus secuelas se limitan a leves alteraciones conductuales y del lenguaje. No ha presentado recaidas ni ha precisado ningun tratamiento de mantenimiento. Conclusiones. La encefalitis anti-NMDA es un trastorno tratable y, ocasionalmente, el primer indicio de una neoplasia subyacente, por lo que su reconocimiento y tratamiento precoz es fundamental. El tratamiento de las formas no paraneoplasicas se basa en la inmunoterapia: glucocorticoides, inmunoglobulinas intravenosas, plasmaferesis e inmunosupresores. La plasmaferesis puede inducir una mejoria rapida y espectacular.

INTRODUCTION Epilepsy is a chronic disorder. The prognosis of one particular subtype, that of children with generalized spike and wave discharges (P-O) on the electroencephalogram (EEG), is unknown. OBJECTIVE To determine the prognosis of children with epilepsy with P-O on EEG and the factors which affect this. PATIENTS AND METHODS All EEGs done over ten years (39,322) were reviewed, and the epileptic patients under 14 years old identified. Absence and recurrence of crises were evaluated and the factors influencing them analyzed. Minimum significance was p < 0.05. The chi square test, Student's t test and Kaplan-Meier method were used. Logistic regression models were devised. RESULTS Of the children studied, 78% became crisis-free. After monovariant and multivariant analysis the only conditions which fitted the regression models were: abnormal pregnancy; West syndrome or learning difficulties; having certain types of crises, myoclonic, clonic-tonic, partial simple or complex crises; a normal EEG with hypsarrhythmia, slow or fast P-O and requiring multiple medication. When medication ceased, 31.4% had recurrence of the episodes. On monovariant and multivariant analysis, protective factors were absence of attacks and risk factors were a family history of epilepsy or an EEG with rapid P-O. CONCLUSIONS The overall prognosis for epileptic children with P-O is good. There are factors which improve or worsen the pronosis, but these cannot be modified.

Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults.We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment.Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.