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Pediatric N-Methyl-d-Aspartate (NMDA) Receptor Encephalitis, With and Without Herpes Encephalitis

Lydia Marcus1, Jayne M Ness1

  • 1Division of Pediatric Neurology, Department of Pediatrics, Children's Hospital of Alabama, 9968University of Alabama at Birmingham, Birmingham, AL, USA.

Journal of Child Neurology
|April 5, 2021
PubMed

Insights

Children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and prior herpes simplex encephalitis (HSE) showed distinct clinical features and received less immunotherapy than those without HSE. Infant outcomes in the HSE+NMDAR group reflected HSE sequelae.

Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
  • Herpes simplex encephalitis (HSE) is a viral infection of the brain that can have long-term consequences.
  • Understanding the interplay between prior HSE and subsequent NMDAR encephalitis is crucial for diagnosis and management.

Purpose of the Study:

  • To compare clinical, diagnostic, management, and outcome factors in pediatric patients with NMDAR encephalitis who had a history of HSE versus those without.
  • To identify differences in presentation, treatment, and prognosis between these two groups.

Main Methods:

  • Retrospective review of pediatric patients with anti-NMDAR antibodies in cerebrospinal fluid between 2012 and 2019.
  • Patients were divided into two groups: those with a history of HSE (HSE+NMDAR) and those without (NMDAR-only).
  • Demographic, clinical, immunotherapy, and outcome data were collected and compared.

Main Results:

  • Six of 17 patients had a history of HSE; 5 were infants, presenting younger than the NMDAR-only group.
  • Seizures were more common in HSE+NMDAR patients (100%) compared to NMDAR-only patients (73%).
  • HSE+NMDAR patients received significantly less immunotherapy (median 1) than NMDAR-only patients (median 4.5).

Conclusions:

  • Clinical presentation and treatment intensity differ between pediatric NMDAR encephalitis with and without a history of HSE.
  • Outcomes in infants with HSE and subsequent NMDAR encephalitis appear to be primarily influenced by the sequelae of HSE.
  • Further research is needed to elucidate the specific mechanisms and optimize management strategies for these complex cases.
Abstract

Keywords:
NMDA receptor antibodyencephalitisherpespediatric

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