Pediatric anti-N-methyl-d-aspartate receptor encephalitis: A review with pooled analysis and critical care emphasis

Kenneth E. Remy, Jason W. Custer, Joshua Cappell, Cortney B. Foster, Nan A. Garber, L. Kyle Walker, Liliana Simon, Dayanand Bagdure

Research output: Contribution to journalReview articlepeer-review

32 Scopus citations

Abstract

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.

Original languageEnglish
Article number250
JournalFrontiers in Pediatrics
Volume5
DOIs
StatePublished - Nov 24 2017

Keywords

  • Autoimmune
  • Critical care
  • Encephalitis
  • N-methyl-d-aspartate
  • N-methyl-d-aspartate receptor
  • Paraneoplastic
  • Pediatrics

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