Objective: To investigate the clinical features, treatment strategies and long term outcomes of children with anti-
N-methyl-D-aspartate receptor (NMDAR)
encephalitis. Methods: The data of clinical features, auxiliary examinations, treatments and prognosis in children with
anti-NMDAR encephalitis in Xiangya Hospital of Central South University from March 2014 to October 2017 were collected and retrospectively analyzed. A total of 71 patients were enrolled, including 33 males and 38 females. The youngest age of onset was 4 months old, and the age of onset was (9±4) years. The first-line
immunotherapy treatment for
anti-NMDAR encephalitis was short course
corticosteroid (high-dose impulse
therapy and oral maintenance
therapy for 1 month in acute period) and (or)
immunoglobulin. The clinical evaluation was performed 2 weeks after first-line
immunotherapy treatment. The second-line
immunotherapy treatment, including
rituximab and (or)
cyclophosphamide, would be started if the symptoms did not improve significantly and the modified Rankin scale (mRS) score ≥3. All patients were followed up and evaluated for prognosis. T-test, Mann-Whitney U, Chi square test and Fisher's exact probability method were used for comparison between good outcome group and poor outcome group, first-line
immunotherapy group and first-line
immunotherapy combined with second-line
immunotherapy group. Results: The more common clinical manifestations were psychiatric symptoms (n=61, 86%),
dyskinesia (n=55, 77%) and convulsions (n=51, 72%). Two cases (3%) had
tumors. Electroencephalogram (EEG), cerebro-spinal fluid (CSF) and brain magnetic resonance imaging (MRI) studies were abnormal in 83% (59/71), 39% (27/69) and 38% (27/71) patients, respectively. For the treatment regimens, all the 71 patients underwent first-line
immunotherapy, resulting in improvement within 14 days in 40 cases (56%), and 1 case (1%) died. The rest 30 cases (42%) received second-line
immunotherapy. The patients were followed up for 5.0-41.8 months, with a median of 19.3 months. At the last follow-up, 49 cases (69%) recovered completely, 15 cases (21%) had mild disability, 6 cases (8%) had severe disability, 1 case (1%) died and 3 cases (4%) had relapse. There were significant differences between the groups with good prognosis and poor prognosis on admission to pediatric intensive care unit (PICU) and
consciousness disorder (10/64 vs. 5/7, 39/64 vs. 7/7, P=0.047, 0.004). There were significant differences between first-line
immunotherapy group and the first-line combined second-line
immunotherapy group on admission to PICU,
consciousness disorder,
sleep disorder and first mRS score (12% (5/41) vs. 33% (10/30), 44% (18/41) vs. 93% (28/30), 56% (23/41) vs. 90% (27/30), 3 (1-5) vs. 4 (3-5), respectively; χ(2)=4.645, 18.555, 9.560, Z=5.184, P=0.031, <0.01, 0.002, <0.01, respectively). Conclusions:
Anti-NMDAR encephalitis can occur in all ages of children. The most common clinical manifestations are psychotic symptoms,
dyskinesia and convulsions. Paraneoplastic cases are less common in children.
Immunotherapy is effective. The second-line
immunotherapy should be given after the failure of first-line
therapy (mRS score≥3).