1. Acute Fulminant Cerebral Edema: A Newly Recognized Phenotype in Children With Suspected Encephalitis
- Author
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Christopher P. Preas, Debra A. Wadford, Michael C. Samuel, Audrey Foster-Barber, Preetha Krishnan, James J. Sejvar, Carol A. Glaser, Heather Sheriff, Arup Roy-Burman, Orit A. Glenn, and Jay H. Tureen
- Subjects
Pediatrics ,medicine.medical_specialty ,Fulminant ,Brain Edema ,Neuroimaging ,Autopsy ,medicine.disease_cause ,Cerebral edema ,Prodrome ,03 medical and health sciences ,0302 clinical medicine ,Enterovirus Infections ,medicine ,Humans ,Child ,030304 developmental biology ,0303 health sciences ,business.industry ,fungi ,General Medicine ,respiratory system ,medicine.disease ,Phenotype ,Infectious Diseases ,Pediatrics, Perinatology and Child Health ,Vomiting ,Etiology ,Encephalitis ,Enterovirus ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
BackgroundEncephalitis is a severe neurological syndrome associated with significant morbidity and mortality. The California Encephalitis Project (CEP) enrolled patients for more than a decade. A subset of patients with acute and fulminant cerebral edema was noted.MethodsAll pediatric encephalitis patients with cerebral edema referred to the CEP between 1998 and 2012 were reviewed. A case definition was developed for acute fulminant cerebral edema (AFCE) that included the CEP case definition for encephalitis and progression to diffuse cerebral edema on neuroimaging and/or autopsy, and no other recognized etiology for cerebral edema (eg, organic, metabolic, toxin). Prodromic features, demographic and laboratory data, neuroimaging, and outcomes were compared with non-AFCE encephalitis cases.ResultsOf 1955 pediatric cases referred to the CEP, 30 (1.5%) patients met the AFCE case definition. The median age for AFCE and non-AFCE cases was similar: 8.2 years (1–18 years) and 8.0 years (0.5–18 years), respectively. Asian-Pacific Islanders comprised a larger proportion of AFCE cases (44%) compared with non-AFCE cases (14%, P Thirty pediatric patients referred to the California Encephalitis Project with a unique, and often fatal, form of encephalitis are reported. Demographic and clinical characteristics, possible etiologies and a proposed case definition for acute fulminant cerebral edema (AFCE) are described.ConclusionsAFCE is a recently recognized phenotype of encephalitis with a high mortality. AFCE may be triggered by common pediatric infections. Here, we propose a case definition.
- Published
- 2020
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