Abstract
Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been discovered for more than a decade, but the establishment of standardized immunotherapy protocol for pediatric patients still needs more clinical evidence.Methods: A multicenter, retrospective study was conducted on pediatric patients diagnosed with anti-NMDAR encephalitis between November 2011 and December 2018. The clinical records including clinical manifestations, immunotherapy strategies, and outcomes were collected and analyzed.Results: A total of 386 patients were included in our study and the median onset age was 8.00 (IQR 4.83–10.90) years. All patients received first-line immunotherapy and the majority (341, 88.3%) used the standard combination of methylprednisolone pulses (MEP) and intravenous immunoglobulins (IVIG), but 211 patients did not show satisfactory improvement (mRS ≥ 3). Mainly three treatment strategies were applied after first-line immunotherapy: second-line immunotherapy, repetitive first-line immunotherapy, and maintaining oral prednisolone. For patients with mRS ≥ 4 after first-line immunotherapy, the incidence of poor outcome (mRS ≥ 3) in oral prednisolone group was higher than that in other treatment groups (p = 0.039). No difference in complete recovery rate (mRS = 0) was found between patients receiving second-line and repetitive first-line immunotherapy, or patients using long-term and short-term prednisolone. Out of 149 patients who received anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab) test, 27 (18.12%) were positive. Patients with concomitantly positive MOG-Ab showed milder conditions compared to patients with typical anti-NMDAR encephalitis and were more inclined to relapses. We also identified female, MOG-Ab positive, and not receiving second-line and/or repetitive first-line immunotherapy were risk factors for relapses.Conclusions: For patients with mRS ≥ 4 after first-line immunotherapy and patients with concomitantly positive MOG-Ab, second-line immunotherapy is recommended. When second-line immunotherapy is not applicable, repetitive first-line immunotherapy can be considered as an option. Both second-line and repetitive first-line immunotherapy are beneficial to reduce relapse rate. The duration of sequential oral prednisolone can be shortened after fully evaluating patients' conditions.
Highlights
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first described in 2007 due to the discovery of specific autoantibodies to NMDA receptors in a series of patients who developed a constellation of neuropsychiatric symptoms, associated with ovarian teratoma [1, 2]
The most widely accepted first-line immunotherapy is high-dose intravenous methylprednisolone pulses (MEP) alone or combined with intravenous immunoglobulins (IVIG); rituximab for second-line immunotherapy is another area of agreement [5, 6]
Co-existing myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in patients diagnosed with anti-NMDAR encephalitis is not rare, and this subpopulation of patients may have different response to treatment according to previous studies [8,9,10]
Summary
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first described in 2007 due to the discovery of specific autoantibodies to NMDA receptors in a series of patients who developed a constellation of neuropsychiatric symptoms, associated with ovarian teratoma [1, 2]. Pediatric patients showed a different clinical symptom profile and lower tumor association rate, and they may have different response to immunotherapy and better outcome [4]. High dose of oral prednisolone followed by tapering is another major maintenance treatment after first-line immunotherapy, but the dosage and duration are open to discussion. How these clinical decisions will affect the outcome of patients is still a pending question. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been discovered for more than a decade, but the establishment of standardized immunotherapy protocol for pediatric patients still needs more clinical evidence
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