The short-term (6-month) outcomes were assessed using the Liverpool score by the same pediatric neurologist. and cognitive disorder (31.4%). Two were transferred to another hospital, 45 (91.8%) received intravenous immunoglobulins, 41 (83.7%) received methylprednisolone, and 8 (16.3%) received plasma exchange. Eight (16.3%) received rituximab for second-line treatment, six after intravenous immunoglobulin Ziyuglycoside I and methylprednisolone treatment, and two after plasma exchange therapy failed. Seven were lost to follow-up. The short-term outcome was good in Ziyuglycoside I 23 patients. Cognitive disorder [odds ratio (OR): 23.97, 95% confidence interval (CI): 1.12C513.30, = 0.042) and abnormal brain MRI (OR: 14.29, 95% CI: 1.36C150.10, = 0.027] were independently associated with a poor short-term outcome after adjustment for age, GCS, and rituximab use. Conclusions: MRI abnormalities and cognitive disorders are independently associated with poor short-term outcomes in children with anti-NMDA receptor encephalitis. The use of rituximab is not associated with the 6-month outcomes. Keywords: anti-N-methyl-D-aspartate receptor encephalitis, pediatrics, neurology, prognosis, treatment, rituximab Introduction N-methyl-D-aspartate (NMDA)-receptor encephalitis is an acute form of encephalitis caused by an autoimmune reaction to the GluN1 subunit of the NMDA neuronal receptor (1, 2). It usually affects women of reproductive age but can occur in men and patients of all ages (2, 3). Although the prevalence and incidence of anti-NMDA receptor encephalitis remain unclear (4), pediatric patients accounted for 37% of the patients in a previous study (5). Anti-NMDA receptor encephalitis is identified more frequently than any specific viral etiology in children with unknown encephalitis (6). In addition, anti-NMDA receptor encephalitis is a leading cause of autoimmune encephalitis (7, 8). Compared with adults, anti-NMDA receptor encephalitis in children is characterized mainly by seizures, dyskinesia, and localized neurological symptoms (5, 9). Recovery is typically slow and erratic and may take >3 years (2). Relapse occurs in 20C25% of the patients (2, 10). The disease is fatal in 4% of the cases (3). The first-line treatment for anti-NMDA receptor encephalitis is immunotherapy, including methylprednisolone, intravenous immunoglobulins, and plasma exchange. The second-line treatment is immunosuppression, typically with rituximab or cyclophosphamide. Nevertheless, standardized treatments are not well-defined (5, 8, 9). In addition, who should receive second-line treatment and when second-line treatment should be initiated remain unclear, with wide variations among hospitals. Hence, the use rate of second-line immunosuppression is 14C81% (5, 9, 11C14). According to a recent survey of pediatric neurologists, the time of initiating second-line therapy varies widely, from started at diagnosis (0 months) or at 6 months in the absence of improvement to the primary therapy (8). The reason for the variability in second-line treatment use is the unclear effect reported in previous observational studies (15C17). In a study by Zekeridou et al. (9), although more patients received second-line treatment, the relapse rate (8%) was similar to that reported in other studies in which fewer patients received second-line treatment (12%) (14). On the other hand, Titulaer et al. (5) reported that second-line treatment was a factor associated with good outcomes. The first case of anti-NMDA receptor TMOD3 encephalitis in China was reported in 2010 2010, followed by many adult and some pediatric cases (18, 19). Still, very few dedicated studies have investigated anti-NMDA receptor encephalitis in pediatric patients or a South China population. Therefore, this study aimed to describe the clinical characteristics of children with anti-NMDA receptor encephalitis in South China and explore the prognostic factors associated with short-term outcomes. Methods Study Design This retrospective observational cohort study was approved by the institutional review board of Hunan Children’s Hospital, Changsha, China. The need for individual consent was waived by the committee. Subjects The children diagnosed with anti-NMDA receptor encephalitis between January 1, 2014, and December 1, 2017, at the neurology department of Hunan Children’s Hospital of Ziyuglycoside I China were included. The diagnostic criteria of pediatric anti-NMDA receptor encephalitis were (1) age 0C16 years, (2) with at least one sign or symptom among the following: abnormal.