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

- 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.

Background and purposeWe 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.MethodsA retrospective study of children (0-14 years 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.ResultsFifty-one patients with anti-NMDAR encephalitis were enrolled (age from 4 months to 14 years old; median age, 8 years; 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 < 15), 28 (55%) with speech disturbances, and twelve (24%) with autonomic instability. None presented with hypoventilation, and only one patient (female, 14 years old) had an ovarian teratoma. All patients received first-line immunotherapy, 25 patients both received firstline and second-line immunotherapy. Forty-four of the 51 patients achieved good outcomes (score on the modified Rankin Scale [mRS] of 0-2), while the other seven had poor outcomes (mRS score of 3-5).ConclusionsThis 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.