Abstract: To explore the clinical characteristics of anti-N-methyl-D-aspartate antibody receptor (antiNMDAR) encephalitis with aphasia as the main symptom. Methods The clinical data of anti-NMDAR encephalitis with aphasia as the main manifestation in 2 children were retrospectively analyzed, and the literature was reviewed. Results In both children, one boy aged 3 years and one girl aged 5 years, the first symptom was aphasia, accompanied by abnormal mental behavior and epilepsy. Blood and cerebrospinal fluid anti-NMDA antibodies were positive in both children. No obvious abnormality was found in both by their brain magnetic resonance imaging. The electroencephalogram of the boy showed spinous and slow waves in bilateral frontal pole, frontal, anterior temporal and midline areas while no obvious abnormality was found in the electroencephalogram of the girl. Both children were treated with immunoglobulin and methylprednisolone, and a nice therapeutic effect was achieved. Conclusion The manifestations of anti-NMDAR encephalitis are various. In children with unexplained aphasia clinicians should be alert to the possibility of anti-NMDAR encephalitis.

Key words: encephalitis; N-methyl-D-aspartate receptor; antibody; aphasia