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Infectious profiles in pediatric anti-N-methyl-d-aspartate receptor encephalitis
Alexander J Sandweiss1, Timothy A Erickson2, Yike Jiang3
1Department of Pediatrics, Division of Pediatric Neurology and Developmental Neuroscience, Baylor College of Medicine and Texas Children's Hospital, United States of America; Department of Pediatrics, Section of Pediatric Tropical Medicine, Center for Human Immunobiology, Baylor College of Medicine and Texas Children's Hospital, United States of America.
Abstract:
Anti-N-methyl-d-aspartate receptor autoimmune encephalitis (NMDAR AE) is an antibody-mediated neurological disorder that may be caused by post-herpes simplex virus-1 meningoencephalitis (HSV ME) and ovarian teratomas, although most pediatric cases are idiopathic. We sought to evaluate if other infections precede NMDAR AE by conducting a single-center, retrospective, case-control study of 86 pediatric cases presenting to Texas Children's Hospital between 2006 and 2022. HSV ME (HSV-1 and HSV-2) was a significantly more common preceding infection in the experimental group compared to control patients with idiopathic intracranial hypertension, while there was no difference in remote HSV infection between the two groups. Recent Epstein-Barr virus infection was evident in 8/42 (19%) tested experimental patients in comparison to 1/25 (4%) tested control patients which provided evidence for a genuine measure of effect but was not statistically significant due to small sample size (p = 0.07). The other 25 infectious etiologies were not different among the two groups and not all variables were clinically indicated or obtained in every subject, highlighting the need for future standardized, multi-institutional studies on underlying infectious precursors of autoimmune encephalitis.
Insights
Herpes simplex virus meningoencephalitis (HSV ME) may precede anti-N-methyl-d-aspartate receptor autoimmune encephalitis (NMDAR AE) in children. Further research is needed to confirm other infectious triggers for NMDAR AE.
Area of Science:
- Neurology
- Immunology
- Infectious Diseases
Background:
- Anti-N-methyl-d-aspartate receptor autoimmune encephalitis (NMDAR AE) is a serious neurological condition.
- While ovarian teratomas and post-herpes simplex virus meningoencephalitis (HSV ME) are known triggers, most pediatric cases are idiopathic.
- Identifying preceding infections is crucial for understanding NMDAR AE pathogenesis.
Purpose of the Study:
- To investigate potential infectious antecedents of NMDAR AE in pediatric patients.
- To compare infection rates in children with NMDAR AE versus controls with idiopathic intracranial hypertension.
Main Methods:
- A single-center, retrospective, case-control study was conducted.
- 86 pediatric cases of NMDAR AE were analyzed between 2006 and 2022.
- Infections were compared between NMDAR AE patients and controls with idiopathic intracranial hypertension.
Main Results:
- Herpes simplex virus meningoencephalitis (HSV ME) was significantly more common preceding NMDAR AE than in controls.
- Recent Epstein-Barr virus infection showed a trend towards higher prevalence in NMDAR AE patients but was not statistically significant.
- No significant differences were found for other infectious etiologies.
Conclusions:
- HSV ME is a potential preceding infection in pediatric NMDAR AE.
- Further large-scale, multi-institutional studies are needed to standardize data collection and confirm infectious precursors.
- Understanding infectious triggers may lead to improved diagnosis and treatment strategies for NMDAR AE.
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