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Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Review with Pooled Analysis and Critical Care Emphasis
Kenneth E Remy1,2,3, Jason W Custer1, Joshua Cappell4
1Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, MD, United States.
Insights
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a serious condition in children. Early recognition and multidisciplinary care are crucial for improving outcomes in pediatric NMDAR encephalitis.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is increasingly diagnosed in pediatric populations.
- Limited evidence exists for guiding critical care management in affected children.
Purpose of the Study:
- To review the literature and perform a pooled analysis of published case reports and series on pediatric anti-NMDAR encephalitis.
- To understand the clinical presentation, treatment strategies, and outcomes in children with anti-NMDAR encephalitis.
Main Methods:
- A comprehensive literature search was conducted across PubMed, Scopus, EMBASE, and Web of Science.
- Studies published between 2005 and May 2016 were included, using keywords related to NMDAR encephalitis in children.
Main Results:
- Analysis of 373 cases from 48 studies revealed common first-line treatments including corticosteroids (89.8%), intravenous immunoglobulin (79.3%), and plasma exchange (31%).
- Outcomes showed 50.1% full recovery with minor deficits, 46.7% partial recovery with major deficits, and 12 deaths. Mechanical ventilation was required in 14 patients.
Conclusions:
- Anti-NMDAR encephalitis presents with diverse clinical features and variable treatment responses.
- Early diagnosis and a multidisciplinary approach are essential for timely access to therapies.
- Further research is necessary to evaluate the long-term efficacy of current treatment strategies.
Purpose:
Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is being recognized with increasing frequency among children. Given the paucity of evidence to guide the critical care management of these complex patients, we provide a comprehensive review of the literature with pooled analysis of published case reports and case series.
Methods:
We performed a comprehensive literature search using PubMed, Scopus, EMBASE, and Web of Science for relevant published studies. The literature search was conducted using the terms NMDA, anti-NMDA, Anti-N-methyl-d-aspartate, pediatric encephalitis, and anti-NMDAR and included articles published between 2005 and May 1, 2016.
Results:
Forty-eight references met inclusion criteria accounting for 373 cases. For first-line treatments, 335 (89.8%) received high-dose corticosteroids, 296 received intravenous immunoglobulin (79.3%), and 116 (31%) received therapeutic plasma exchange. In these, 187 children (50.1%) had a full recovery with only minor deficits, 174 patients (46.7%) had partial recovery with major deficits, and 12 children died. In addition, 14 patients were reported to require mechanical ventilation.
Conclusion:
Anti-NMDA encephalitis is a formidable disease with great variation in clinical presentation and response to treatment. With early recognition of this second most common cause of pediatric encephalitis, a multidisciplinary approach by physicians may provide earlier access to first- and second-line therapies. Future studies are needed to examine the efficacy of these current therapeutic strategies on long-term morbidity.
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