Background and Objectives: The most common antibody associated with pediatric autoimmune encephalitis is the anti-N-methyl-D-aspartate receptor (anti-NMDAR), wherein antibodies produced by the body’s own immune system attack NMDA receptors in the brain. NMDA receptors are proteins that control electrical impulses critical for judgment, perception, memory, language, and autonomic functions, leading to a wide range of neuropsychiatric complications and the prolonged recovery seen in anti-NMDAR encephalitis.The cornerstone of treatment of anti-NMDAR encephalitis lies in the prompt initiation of immunotherapy, which is first done with corticosteroids. If the response is inadequate after corticosteroids, additional first-line immunotherapy with intravenous immunoglobulin (IVIg) and therapeutic plasma exchange (TPE) is then recommended.This study aimed to identify the clinical profile of pediatric patients with anti-NMDAR encephalitis, as well as to compare outcomes in those treated with IVIg versus TPE after corticosteroids, analyzed in terms of: change in disability, risk of relapse, and length of hospital stay.Method: A systematic review of studies published between 2015 to 2021 was done, with 5 studies included in the final review. A total of 125 patients were evaluated for the clinical profile, with 63 patients analyzed for the final outcomes.Learning Points Discussion: The clinical profile of patients obtained from this review are consistent with previously reported studies on pediatric anti-NMDAR encephalitis. Patients showed a female predominance among school-age children. Behavioral changes were the most common clinical manifestation after the prodromal phase, which was seen across all age groups. EEG consistently showed encephalopathic changes, but the characteristic finding identified as the delta brush pattern in other anti-NMDAR studies that included the adult population was not a universal finding among children. Cranial MRI was mostly normal.Both IVIg and TPE-treated patients obtained lower scores on the modified Rankin scale (mRS) upon follow-up, showing less disability after treatment with either of the two treatment groups. This suggests an improvement in functional outcome after either treatment and further demonstrates the responsiveness of the disease to immunotherapy. However, patients who underwent TPE exhibited a greater change in disability score on the mRS, suggesting a better functional outcome. In terms of reducing risk of relapse, there was no significant difference between the two treatment groups.Results were inconclusive in the comparison of length of hospital stay, where duration of admission may be affected by the timeliness of diagnosis, disease severity, and socioeconomic factors. Regardless of the combination of first-line immunotherapy used, it is agreed that early diagnosis and intervention are a strong determinant of improvement. The limited number of studies on TPE in children reflects the need for further research on this treatment modality to analyze patient outcomes. (picture)
