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

Abstract

Pediatric 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.

Keywords

children - autoimmune encephalitis - NMDAR - antibody - Sri Lanka

Ethical Approval

The study received its ethical approval from EC/21/46 from Ethics Review Committee of Faculty of Medicine, Colombo, Sri Lanka.

Consent for Publication

Consent has been obtained from individual guardians in respective cases.

Availability of Data and Materials

The datasets used and/or analyzed during the current case series are available from the corresponding author on reasonable request.

Authors' Contributions

J.W.was the principal investigator of the project, and was also responsible for diagnosis, management, and follow-up of all the patients in this study. She is also responsible for major proportion of the writing of the article. She takes full responsibility for the data and its accuracy and the conduct of the research.

K.W.D.A. A. was a coauthor, involved in the care of the patients, data recording, and compiling the data. She also contributed toward improvement of the article.

T.C, another coauthor, was responsible for shared care of some of the patients in the study; helped coordinate the antibody assays of these patients. He has significantly contributed toward the writing and improving the article.

Publication History

Received: 18 June 2021

Accepted: 18 September 2021

Article published online:
16 November 2021

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