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Acute Fulminant Cerebral Edema: A Newly Recognized Phenotype in Children With Suspected Encephalitis

Preetha Krishnan1, Orit A Glenn2, Michael C Samuel3

  • 1Pediatric Critical Care Medicine, Randall Children's Hospital, Portland, Oregon, USA.

Journal of the Pediatric Infectious Diseases Society
|July 16, 2020
PubMed

Insights

Acute fulminant cerebral edema (AFCE) is a severe encephalitis subtype in children with a high mortality rate. This condition, potentially triggered by common infections, requires a defined case definition for better identification and management.

Area of Science:

  • Pediatric Neurology
  • Infectious Diseases
  • Neurocritical Care

Background:

  • Encephalitis presents a significant global health challenge, marked by substantial morbidity and mortality.
  • The California Encephalitis Project (CEP) has provided extensive data on pediatric encephalitis cases over more than a decade.
  • A distinct subset of patients exhibited acute and fulminant cerebral edema, necessitating further investigation.

Purpose of the Study:

  • To define and characterize a specific phenotype of pediatric encephalitis presenting with acute fulminant cerebral edema (AFCE).
  • To compare the demographic, clinical, and outcome data of AFCE patients with non-AFCE encephalitis cases.
  • To propose a case definition for AFCE to aid in its identification and study.

Main Methods:

  • Retrospective review of pediatric encephalitis patients with cerebral edema referred to the CEP (1998-2012).
  • Development of an AFCE case definition incorporating encephalitis criteria, diffuse cerebral edema on imaging/autopsy, and exclusion of other etiologies.
  • Comparison of prodromal features, demographics, laboratory data, neuroimaging, and outcomes between AFCE and non-AFCE groups.

Main Results:

  • Out of 1955 pediatric cases, 30 (1.5%) met the AFCE criteria.
  • AFCE cases showed a higher proportion of Asian-Pacific Islanders (44% vs. 14%) and significantly higher mortality (80% vs. 13%) compared to non-AFCE cases.
  • Etiologies were rarely identified (2 confirmed cases); respiratory pathogens were suggested in 10 cases.

Conclusions:

  • Acute fulminant cerebral edema (AFCE) represents a distinct and severe phenotype of pediatric encephalitis.
  • AFCE is associated with a high mortality rate and may be precipitated by common pediatric infections.
  • A proposed case definition for AFCE is presented to facilitate recognition and clinical management.
Abstract

Keywords:
California Encephalitis Projectacute fulminant cerebral edemaencephalitispediatrics

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