Pediatric Autoimmune Encephalitis in Sri Lanka: A Single-Center Experience over 7 Years

Abstract

AbstractPediatric autoimmune encephalitis (AE) remains a diagnostic and therapeutic challenge in resource-poor settings. Minimizing delay in diagnosis and appropriate escalation of treatment will help reduce both the short- and long-term neurodisabilities. A retrospective observational study was performed on children consecutively diagnosed with possible AE and then prospectively followed up in a single tertiary care children's hospital in Sri Lanka. Serum and cerebrospinal fluid were tested for neuroglial surface-binding autoantibodies using cell-based assays in majority of these children. Twenty-five children (mean age 7.6 years, standard deviation = 4) were recruited. In these children, presenting symptom was psychiatric in 11 children (44%), seizures in 10 (40%), language regression in 2 (8%), and combination of psychosis and convulsions in 2 (8%). Psychiatric presentations were more common in older (>6 years) compared with young children (p = 0.001), while neurological presentations were more common in children aged ≤6 years (p = 0.001). N-methyl-D-aspartate receptor (NMDAR) antibodies were detected in 9 (45%) and unspecified voltage-gated potassium channel antibodies in 1 (5%) of the 20 tested. All received intravenous steroids and immunoglobulins; 19 (76%) plasma exchange; 7 (29%) rituximab. Complete/substantial improvement at 3 months occurred in 64%. Pediatric Cerebral Performance Category score at last review was 1 (normal function for age) in 43%. Higher proportion of younger children required less intense therapy and had better recovery (56%). Death (8%), incomplete recovery (71%), and relapses (8%) were more in older children. Clinical presentation and disease outcomes were different in children aged <6 years compared with older age group. NMDAR antibody encephalitis was the commonest AE syndrome identified in this cohort.