Clinical diagnosis and treatment of pediatric anti-N-methyl-D-aspartate receptor encephalitis: A single center retrospective study

Abstract

The aim of the present retrospective study was to investigate the diagnosis, treatment and prognosis of pediatric anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. A total of 23 pediatric patients with anti-NMDAR encephalitis were included in the present study. The clinical data, laboratory test results, imaging examination, treatment outcomes, and follow-up records were reviewed and analyzed. A total of 8 patients exhibited prodromal symptoms, including fever, cough, and vomiting. Clinical symptoms included epilepsy, convulsions, ataxia, coma, dyskinesia, personal behavior change and hallucinations. A total of 20 cases had the initial neurologic symptoms of dyskinesia or seizure, whereas 3 cases exhibited psychiatric symptoms of personal behavior change and hallucinations. Furthermore, pediatric patients >6 years old had more psychiatric symptoms than those ≤6 years. A total of 20 cases exhibited abnormal electroencephalography records, with 1 case of extreme δ brush. A total of 10 cases exhibited abnormal brain magnetic resonance imaging detection. Furthermore, the CSF protein contents for pediatric patients ≤6 years old was significantly higher than those >6 years. For treatment, 18 pediatric patients received the first-line treatment of methylprednisone and intravenous injection of immunoglobulin, and 6 cases were subjected to the second-line treatment of rituximab. A total of 2 patients underwent plasma exchange and/or cyclophosphamide treatment. In follow-up, 12 cases reported no convulsion, whereas 11 cases had moderate or severe neurological and psychiatric sequelae. The recovery rate for pediatric patients ≤6 years old was significantly higher than those >6 years. Anti-NMDAR encephalitis is commonly seen in pediatric patients, mainly with initial neurological symptoms. These patients could respond to immunotherapy, and younger pediatric patients typically have a better prognosis.

Keywords: autoimmune encephalitis; clinical treatment; extreme δ brush; pediatric patients.

Figure 1.

Electroencephalography pattern of a 10-year and 3-month old female child with anti-N-methyl-D-aspartate receptor encephalitis. Extreme δ brush consisted of almost completely continuous δ activities, with superimposed fast activities, typically in the β wave range of patients who were not under sedation or anesthesia. This pattern resembled the δ brush appearing in premature infants, but the extreme δ brush was mainly associated with frontal lobe symmetry and synchronization.

Figure 2.

Magnetic resonance imaging detection of a representative pediatric patient (male, 13-year and 6-month old, admitted for convulsions for 1 week) with anti-N-methyl-D-aspartate receptor encephalitis. Multiple long T1 and long T2 signal shadows were noted in the bilateral frontal and temporal lobes, right thalamus and corpus callosum, which was obvious in the right front temporal lobe (as indicated by white arrows). Fluid-attenuated inversion recovery sequence mainly demonstrated high signals, within which low signals could be observed. Widened and deepened adjacent fissures were observed, as well as slightly enlarged bilateral lateral ventricle and third ventricle, with no obvious shift of the midline structure.