Journal of Pediatric Neurology
DOI: 10.1055/s-0041-1724099
Case Report

Acute Fulminant Cerebral Edema: A Case Series at a Large Pediatric Tertiary Center

1  Division of Child Neurology, Departments of Neurology and Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Carlyn Patterson Gentile
1  Division of Child Neurology, Departments of Neurology and Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Lauren A. Beslow
1  Division of Child Neurology, Departments of Neurology and Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Mariarita Santi
2  Department of Anatomic Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Sonika Agarwal
1  Division of Child Neurology, Departments of Neurology and Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States
› Author Affiliations

Abstract

Acute fulminant cerebral edema is a poorly understood but serious neurologic condition resulting in profound neurologic disability or mortality. Here we present a case series of four children that presented to our institution with new neurologic dysfunction and neuroimaging evidence of cerebral edema. Ages ranged from 2 to 7 years with the most common presenting features being altered mental status, vomiting, and/or seizure. Two patients had normal head computed tomography, but follow-up imaging performed within 15 hours demonstrated fulminant edema. One patient was positive for influenza, and one had neuropathology consistent with acute hemorrhagic leukoencephalitis. Two had no identified cause. Treatments included broad-spectrum antibiotics and acyclovir, hyperosmolar agents, intravenous steroids, and decompressive craniectomy. Only one patient survived. Acute encephalopathy complicated by fulminant cerebral edema is a rapidly evolving and often fatal neurologic condition. Early identification with neuroimaging and intervention may improve outcomes. Repeat neuroimaging should be considered if initial imaging is normal but there is persistent or progressive unexplained encephalopathy. Further studies are required to determine optimal diagnostic and management strategies.

Supplementary Material



Publication History

Received: 22 November 2020

Accepted: 01 January 2021

Publication Date:
19 February 2021 (online)

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