Objectives: We analyzed the clinical features and outcomes of children with anti-N-methyl- D –aspartate receptor (anti-NMDAR) encephalitis in Northwest China.

Methods: We retrospectively recruited 33 pediatric patients with anti-NMDAR encephalitis in Northwest China from December 2013 to April 2020. Demographics, clinical features, treatment, and outcome were reviewed.

Results: 33 patients with anti-NMDAR encephalitis were enrolled (age from 9 months to 13 years old; median age: 6.8 years ; 20 females) in this study. The initial symptoms included seizure in 14 patients (42.4%), psychiatric symptom in 13 (39.4%), speech dysfunction in 4 (12.1%), and paralysis in 2 (6.1%). During course of the disease, 31 patients (93.9%) presented with psychiatric symptoms, 29 (87.9%) with speech dysfunction, 25 (75.8%) with movement disorders, 24 (72.7%) with sleep disorders, followed by seizures, consciousness disturbance, autonomic nervous dysfunction, paralysis, and hypoventilation. 12 patients (36.4%) had abnormal cerebrospinal fluid (CSF) findings, 10 patients (30.3%) exhibited abnormal brain magnetic resonance imaging (MRI) results, and 29 patinets (87.9%) showed abnormal Electroencephalography (EEG) findings. None had tumor. All patients received first-line immunotherapy, 8 patients both received first-line and second-line immunotherapy. 30 of the 33 patients achieved good outcomes (score on the modified Rankin Scale [mRS] of 0–2), while the other 3 had poor outcomes (mRS score of 3–6).

Discussion Read tips: Abnormal cranial MRI findings, intensive care unit (ICU) stay and autonomic instability were predictors of poor response to first-line immunotherapy. Patients with higher CSF anti-NMDAR body titer were more likely to develop sleep disorder, consciousness disturbance and more severe disease states.