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

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<15), 28 (55%) with speech disturbances, and twelve (24%) with autonomic instability. None presented with hypoventilation, and only one patient (female, 14years 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).

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.

Keywords: Encephalitis; Immunotherapy; N-methyl-d-aspartate receptor antibody.