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Pediatr Neurol. 2011 Dec;45(6):400-2. doi: 10.1016/j.pediatrneurol.2011.09.007.

Cytokine storm of acute necrotizing encephalopathy.

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  • 1Division of Pediatrics Neurology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.


Acute necrotizing encephalopathy is a rare, clinically distinct entity characterized by multiple, symmetric areas of edema and necrosis in the thalamus, cerebellum, brainstem, and white matter. It is postulated to arise from uncontrolled cytokine release during a febrile illness, and is most often seen in East Asia. We describe a rare North American case of acute necrotizing encephalopathy attributable to human herpes virus-6 is a 9-month-old white male. The infant moved to the United States from Hong Kong, 3 months before disease onset. A workup revealed elevations in serum interleukin-1β, interleukin-2, and interleukin-10, with normal values of interleukin-6 and tumor necrosis factor-α after the initiation of high-dose steroids. This profile is unique compared with previous cytokine profiles of this disease, possibly because of the effects of steroid therapy. A rare North American case with a history of birth in East Asia underscores the possibility of a role for environmental pathogens in this disease.

Copyright © 2011 Elsevier Inc. All rights reserved.

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