Anti-N-methyl-D-aspartate receptor encephalitis with demyelinated lesions in 3 children and literature review

Objective To summarize the clinical and prognostic features of anti-N-methyl-D-aspartate receptor (NMDAR)encephalitis with demyelinated lesions and discuss the possible pathogenesis. Methods The clinical and imaging features of 3 pediatric patients diagnosed as anti -NMDAR with demyelinated lesions were analyzed.The published papers were browsed by using anti-NMDA receptor encephalitis and demyelinating as key words into CNKI, Wanfang and PubMed database from starting point to May, 2017. Results In 3 cases, anti-NMDAR occurred simultaneously with demyelinated episodes in 2 cases, successively in the other case.One case had AQP4-IgG positive.Two cases had recurrent course, and 1 case had a single course and poor prognosis.A total of 15 articles reported 41 cases, including 16 (39.02%)pediatric cases. In these pediatric cases, anti-NMDAR occurred in 7 cases (43.75%)successively and demyelinated episodes occurred in 9 cases (56.25%)simultaneously. AQP4 antibody and MOG antibody in serum and/or cerebrospinal fluid were detected in all cases, with either of two antibodies positive in 9 cases (56.25%). Conclusion Anti-NMDAR might occur simultaneously or successively with demyelinated episodes.Compared with typical patients with anti-NMDAR encephalitis, patients with demyelinated lesions are more likely to relapse and have worse outcomes.Anti-NMDAR and demyelinated lesions are both based on similar immune dysfunction or demyelinated lesions are also induced by anti-NMDAR antibodies, which is the probable pathogenesis. Key words: Anti-N-methyl-D-aspartate receptor encephalitis; Demyelinated lesions; Child