Abstract:

Objective To describe the clinical manifestations and prognosis of children with anti-N-methyl-D-aspartate receptor encephalitis.Methods Clinical manifestations, lab examination, management and prognosis of children with anti-N-methyl-D-aspartate receptor encephalitis hospitalized in Neurology Department of Beijing Children′s Hospital from January 2012 to December 2014 were retrospectively analyzed and the effect at the last follow-up was assessed by Modified Rankin Scale (MRS).Results A total of 102 cases diagnosed as anti-N-methyl-D-aspartate receptor encephalitis with the average onset age of (7.5±4.0) years ranged from 6 months to 15.8 years were enrolled into the analysis including 38 males and 64 females. The average onset age of females was elder than that of males (P=0.009). Psychiatric disturbances was found in 40 cases and neurology disorder was found in 62 cases as the initial symptoms. The symptoms in acute stage included mental and behavior disorder (99/102，97.1%), dyskinesia (98/102，96.1%), speech disorder (91/102，89.2%), sleep disorder (79/102，77.5%) and central hypoventilation (7/102，6.9%), and there was no significant difference among different age groups. All cases received the first line therapy, 18(17.3%) cases received the second line therapy, 14 cases(15.9%) had sequelae (MRS≥3), 3 cases died and 4 cases relapsed. Conclusion Anti-N-methyl-D-aspartate receptor encephalitis happened in different age groups, clinical manifestations were similar to adult, most of cases had favourable prognosis, but young children and those secondary to virus encephalitis had poor prognosis.