Anti- N -methyl- d -aspartate receptor encephalitis in children of Central South China: Clinical features, treatment, influencing factors, and outcomes

摘要

Abstract Background and purpose We analyzed the clinical manifestations of children with anti- N -methyl- d -aspartate receptor (anti-NMDAR) encephalitis in Central South China and the factors influencing the effectiveness of treatment. Methods A retrospective study of children (0–14years old) with anti-NMDAR encephalitis in Central South China was carried out from March 2014 to November 2016. Demographics, clinical features, treatment, outcome, and the factors influencing the effectiveness of treatment were reviewed. Results Fifty-one patients with anti-NMDAR encephalitis were enrolled (age from 4months to 14years old; median age, 8years; 30 females). Forty-five patients (88%) presented with psychiatric symptoms, 40 (78%) with dyskinesia and movement disorders, 39 (77%) with sleep disturbances, 34 (67%) with seizures, 30 (59%) with a decreased level of consciousness (Glasgow score Conclusions This study investigated the clinical characteristics of children (aged 14 or younger) with anti-NMDAR encephalitis in Central South China. Patients with decreased consciousness, PICU stay and autonomic instability were more likely to have no or limited response to first-line immunotherapy and to require second-line or even more aggressive immunotherapy. Children with anti-NMDAR encephalitis in China have a much lower incidence of tumors, lower mortality rates, and a lower proportion of lethal autonomic instability than adults. Highlights ? The study is the largest of anti-NMDAR encephalitis in China, reviewing clinical features, examinations, therapy and outcome. ? Patients with decreased consciousness, PICU stay and autonomic instability were likely to poor response to first-line therapy. ? Young patients in China have a much lower incidence of tumor, mortality rate, and lethal autonomic instability than adults.