Clinical and electrophysiologic characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children

Abstract

Objective To explore the clinical and electrophysiologic features of anti-N-methyl-D-aspartate receptor(NMDAR) encephalitis in children. Methods The clinical records and findings of electroencephalogram(EEG) of the anti-NMDAR encephalitis patients diagnosed in the Capital Institute of Pediatrics were reviewed and analyzed.Five patients with anti-NMDAR encephalitis were identified, including 4 boys and 1 girl, aged from 2 years and 6 months to 6 years and 8 months. Results No tumor was found in those patients.Four patients developed the symptoms of seizure at first, and suffered from consciousness disturbance and movement disorder later, while the other patient was found to be affected by language disorder at first.All patients were treated with methylprednisolone and intravenous immunoglobulin therapy, and plasma exchange and(or) CD20 monoclonal antibodies were used when the patient did not respond well to the treatment.With this immunotherapy used, the patients showed great improvement in cognitive, language and movement abilities, but 1 relapsed 9 months after discharge.All patients had abnormal electroencephalogram with diffusive slow waves, and some with focal spikes or sharp waves.After the patients recovered, EEG showed fewer slow waves, and even normal backgrounds. Conclusions Anti-NMDAR encephalitis can be found in children, even young boys may be affected by it without tumors.For those suffering from this disease, seizure and language disorder may be one of the initial symptoms, and movement disorder and consciousness alteration will occur later.In treating this disease, immunotherapy proves effective.There is a risk of disease relapse if the immune treatment doesn't sustain long enough. Key words: N-methyl-D-aspartate receptor; Encephalitis; Epilepsy; Movement disorder