Clinical characteristics and treatment of severe autoimmune encephalitis in children / 中华急诊医学杂志

ABSTRACT

Objective:To summarize the clinical features, imaging changes, treatment, and prognosis of children with severe autoimmune encephalitis (AE).Methods:A retrospective study was conducted on patients with severe AE admitted to PICU of Shanghai Children’s Hospital from June 2017 to May 2020. Clinical features, treatment protocols and follow-up data were collected.Results:A total of 27 children were included, among which 18 cases (66.7%) were girls. The on-set age was (7.9±3.2) years. Eighteen cases were diagnosed with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Fever (77.8%), headache (40.7%) and vomiting (44.4%) were most of prodromal symptoms in children with severe AE. Patients’ neurological symptoms showed seizures (88.9%), mental behavior abnormalities (81.5%), speech disorders (70.4%) and dyskinesia (70.4%). Moreover, epileptic discharge and slow wave activity were critical feature of electroencephalogram (EEG) abnormalities, and the abnormal signal changes on T2-weighted and FLAIR sequence of head MRI were in the posterior horn of the lateral ventricle. In addition, the main comorbidities included refractory status epilepticus (RSE), cardiovascular dysfunction, central hypoventilation syndrome and acute intracranial hypertension syndrome. For patients with central respiratory failure, the median duration of mechanical ventilation was 19.8 (14.8, 29.1) days. According to treatment protocol, the first-line immune treatment included the combination therapies of methylprednisolone, intravenous immunoglobulin (IVIG) and therapeutic plasma exchange (TPE). Eighteen cases were given with methylprednisolone [10-30 mg/(kg. d), 3-5 d] + IVIG (2 g/kg, within 2 d) + TPE, 1 case was treated with methylprednisolone [10-30 mg/(kg·d), 3-5 d] + TPE and 8 cases were given with[10-30 mg/(kg·d), 3-5 d] + IVIG (2 g/kg, within 2 d). Sequential therapy was given with methylprednisolone (1-2 mg/kg), gradually reduced from 3 to 6 months. Finally, 16 children (59.3%) had neurological damages at the first discharge, among which 8 cases (29.6%) were with dyskinesia, 5 cases (18.5%) were with speech disturbance, and 5 cases (18.5%) were with abnormal mental behaviors.Conclusions:The most of first clinical symptom is epileptic seizures in pediatric severe AE, and most of these patients are diagnosed with Anti-NMDA receptor encephalitis. RSE, cardiovascular dysfunction, central respiratory and acute intracranial hypertension syndrome constitute to main organ dysfunctions.