Clinical diagnosis and treatment of pediatric anti-N-methyl-D-aspartate receptor encephalitis: A single center retrospective study

Exp Ther Med. 2018 Aug;16(2):1442-1448. doi: 10.3892/etm.2018.6329. Epub 2018 Jun 20.


The aim of the present retrospective study was to investigate the diagnosis, treatment and prognosis of pediatric anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. A total of 23 pediatric patients with anti-NMDAR encephalitis were included in the present study. The clinical data, laboratory test results, imaging examination, treatment outcomes, and follow-up records were reviewed and analyzed. A total of 8 patients exhibited prodromal symptoms, including fever, cough, and vomiting. Clinical symptoms included epilepsy, convulsions, ataxia, coma, dyskinesia, personal behavior change and hallucinations. A total of 20 cases had the initial neurologic symptoms of dyskinesia or seizure, whereas 3 cases exhibited psychiatric symptoms of personal behavior change and hallucinations. Furthermore, pediatric patients >6 years old had more psychiatric symptoms than those ≤6 years. A total of 20 cases exhibited abnormal electroencephalography records, with 1 case of extreme δ brush. A total of 10 cases exhibited abnormal brain magnetic resonance imaging detection. Furthermore, the CSF protein contents for pediatric patients ≤6 years old was significantly higher than those >6 years. For treatment, 18 pediatric patients received the first-line treatment of methylprednisone and intravenous injection of immunoglobulin, and 6 cases were subjected to the second-line treatment of rituximab. A total of 2 patients underwent plasma exchange and/or cyclophosphamide treatment. In follow-up, 12 cases reported no convulsion, whereas 11 cases had moderate or severe neurological and psychiatric sequelae. The recovery rate for pediatric patients ≤6 years old was significantly higher than those >6 years. Anti-NMDAR encephalitis is commonly seen in pediatric patients, mainly with initial neurological symptoms. These patients could respond to immunotherapy, and younger pediatric patients typically have a better prognosis.

Keywords: autoimmune encephalitis; clinical treatment; extreme δ brush; pediatric patients.