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. 2024 Jul 2:15:1408606.
doi: 10.3389/fneur.2024.1408606. eCollection 2024.

A systematic review of the epidemiology of pediatric autoimmune encephalitis: disease burden and clinical decision-making

Jonathan D Santoro  1 , Panayotes Demakakos  2 , Shiying He  3 , Swati Kumar  4 , Molly Murton  4 , Frank Tennigkeit  5 , Cheryl Hemingway  6   7
Affiliations

A systematic review of the epidemiology of pediatric autoimmune encephalitis: disease burden and clinical decision-making

Jonathan D Santoro et al. Front Neurol. .

Abstract

Background: Autoimmune encephalitis (AIE) comprises a group of rare, immune system-mediated conditions. Clinical manifestations among children are not well-characterized, and there are challenges in testing and diagnosis. This can result in treatment delays, which has been found to correlate with poorer long-term outcomes. This challenge is exacerbated by the scarcity of epidemiological reporting of AIE. The objective of this systematic literature review (SLR) was to identify studies reporting epidemiological data on AIE in children.

Methods: MEDLINE, Embase, the Cochrane Library, and the University of York Centre for Reviews and Dissemination (CRD) were searched in May 2023 for studies reporting on the epidemiology of AIE in children. These were supplemented with additional searches of conference proceedings, gray literature, and the reference lists of identified SLRs. Quality of studies was assessed using a modified version of the Joanna Briggs Institute (JBI) Checklist for Prevalence Studies.

Results: Forty-three publications reporting on 41 unique studies were included. Nine studies reported incidence estimates of different subtypes of AIE, with only one reporting the incidence of overall AIE in children ≤ 18 years, estimated at 1.54 per million children per year in the Netherlands. Three studies reported the incidence of pediatric N-methyl-D-aspartate receptor (NMDAR)-AIE [in United Kingdom (UK), Hong Kong, and Denmark]. The other studies reported incidence data for selected populations.

Conclusion: This SLR highlights a paucity of epidemiology data for AIE in children, which is likely reflective of difficulties in testing and diagnosis. There is a clear need for further research and awareness of these challenges in clinical practice to avoid treatment delays and improve patient outcomes. A deeper understanding of the epidemiology of AIE will help determine the worldwide burden of disease and inform research, health policies and clinical decision-making.

Keywords: AIE; autoimmune encephalitis; epidemiology; pediatric; systematic review.

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Conflict of interest statement

SH, SK, and MM were employees of Costello Medical at the time this study was conducted, which received fees from UCB Pharma for conducting this work. Costello Medical receives fees for its consulting services from a range of companies across the healthcare sector. JS has received consulting fees from Cycle Pharmaceuticals and UCB. He receives grant funding through the National Institutes of Health (NHLBI and NICHD); PD was employed by UCB; SH, SK, and SM were employed by Costello Medical; FT was employed and shareholder of UCB; and CH reports grant support from the MRC and MS Society. He has served as a consultant to Novartis, Roche, UCB, and Sanofi. This literature review and all costs associated with publication, were funded by UCB Pharma. Medical writing support was provided to Costello Medical funded by UCB Pharma, in accordance with Good Publication Practice 2022 guidelines. Authors were not paid for participating in the preparation of this article. The funder was involved in the study design, data collection and analysis, decision to publish and preparation of the manuscript. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Figures

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Figure 1
PRISMA flow diagram.
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