[SP5-3C-7] Anti-NMDA Receptor Encephalitis: Outcome of Treatment with Cyclophosphamide IV
Anti-NMDA receptor encephalitis is increasingly diagnosed in children. Treatment has been varied as well as outcome. This study is aimed to reveal outcome of children with anti-NMDA receptor encephalitis treated at our center by retrospective chart review. All patients diagnosed with anti-NMDA receptor antibody between 1 January 2010 and 31 January 2016 were included. These patients had confirmed diagnosis with positive anti-NMDA receptor antibody in CSF and/or serum. A total of 16 children (13 girls, 3 boys) with median age of 9 years 5 months were enrolled. Patients were treated with primary immunotherapy including pulse methylprednisolone (100%) and IVIG (50%). Ovarian teratoma was noted in only one patient who died from respiratory failure and severe autonomic dysfunction after tumor removal. The other 15 patients were subsequently received a 6-month-course of cyclophosphamide IV. 14 patients responded well with gradually improvement of neurocognitive function. The non-responder was switched to azathioprine and had significant improvement. With the follow-up period of 10-73 (median 32) months, 5 patients (31%) had relapses at the period of 3-37 months after complete treatment. Extended course of cyclophosphamide IV, azathioprine or mycophenolate mofetil were used in the relapse patients. One patient passed away at home from respiratory-related problem one month after relapse. At final evaluation; 6, 7 and 1 patients had modified Rankin scale at 0,1 and 2 respectively. In conclusion, treatment of anti-NMDA receptor encephalitis with pulse steroid and/or IVIg followed by cyclophosphamide IV resulted in a favorable outcome.