ReviewClinical characteristics, treatments, and outcomes of patients with anti-N-methyl-d-aspartate receptor encephalitis: A systematic review of reported cases

- Pediatric patients had a higher ratio of seizures to psychiatric symptoms as the initial manifestation than adults.
- Pediatric patients had a lower occurrence of tumor and CSF pleocytosis than adults.
- Pediatric patients had a better outcome than adult patients.
- There was no significant differences among the outcomes of three first-line immunotherapies.
- The specific sequence of steroid and IVIG had no significant influence on the outcome.

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a recently recognized autoimmune disorder which is responsive to immunotherapy. However, the outcomes of different immunotherapies have not been defined and there have been few studies that carried out a comparison among them. To provide an overview of the clinical characteristics, treatments, and outcomes of anti-NMDAR encephalitis, we systematically reviewed the literature in the PubMed, Medline, Embase, Cochrane Library, BioMedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), and Wan-fang databases. Eighty-three studies with a total of 432 patients were included. The median age was 22 years. Two hundred ninety-three (68%) patients were female, 87 (21%) of 412 patients had a tumor, including 68 (78%) patients with ovarian teratoma. Pediatric patients had a higher ratio of seizures to psychiatric symptoms as the initial manifestation (p = 0.0012), a lower proportion with a tumor (p < 0.0001) and CSF pleocytosis (p = 0.0163), and a better outcome (p = 0.0064) than adults. Patients who died had a higher proportion of CSF pleocytosis than the patients who survived (p = 0.0021). There were no significant differences among three first-line immunotherapy used alone (p = 0.9172) or among combinations of every two of them (p = 0.3059). With regard to the use of corticosteroid and IVIG, there were no significant differences between the outcomes of early combined treatment and sequential treatment (p = 0.7277), or between using corticosteroid first and IVIG first (p = 0.5422). Our findings suggest that the clinical characteristics and outcomes for pediatric patients were different from adult patients, and no significant differences were found among different immunotherapies.