Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children: an analysis of 90 cases

Abstract:

Objective：To investigate the clinical characteristics，treatment outcome，and prognosis of anti-N-methyl-D-aspartate re－ceptor（NMDAR） encephalitis in children，and to improve the understanding of the disease. Methods：The clinical data of 90 children with anti-NMDAR encephalitis diagnosed in Children’s Hospital of Chongqing Medical University from August 2012 to January 2018 were retrospectively analyzed. Results：Of the 90 children aged from 8 months to 13 years，38（42.2%） were males，and 52（57.8%） were females. The first symptoms of the disease were seizure（47.8%） and psychiatric disorders（33.3%），and the most common clinical symptoms were dyskinesia in 81 cases（90%），psychiatric disorders in 70 cases（77.8%），seizure in 58 cases（64.4%），sleep disorders in 51 cases（56.7%），and cognitive dysfunction in 35 cases（38.9%）. Anti-NMDAR antibodies in cerebrospinal fluid were positive in all patients，more than 90% of the patients had abnormal electroencephalogram，about 50% had abnormal brain magnetic resonance imaging，and tumor screening was negative in all patients. Seventy-four patients（82.2%） were treated with intravenous immunoglobulin（IVIG） and methylprednisolone，8 patients（8.9%） were treated with IVIG only，2 patients（2.2%） were treated with methylpred－nisolone only，2 patients（2.2%） received plasma exchange treatment，and 3 patients with recurrence and no remission received ritux－imab，mycophenolate mofetil，and azathioprine. After 2 to 52 months of follow-up，94.3% of the patients had a good prognosis，3.2% died，and 23.4% relapsed. Conclusion：Anti-NMDAR encephalitis has various clinical symptoms，and some clinical symptoms may be associated with the severity of the disease. The positive finding of anti-NMDAR antibody in cerebrospinal fluid can confirm the diag－nosis of the disease. The incidence of tumor in children with anti-NMDAR encephalitis is low. Early diagnosis and timely immunotherapy can improve prognosis.