title: Cytokine storm of acute necrotizing encephalopathy.
abstract: 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.
authors: Division of Pediatrics Neurology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
authors: Sujay M
authors: SM
authors: Kansagra
authors: William B
authors: WB
authors: Gallentine
mesh_terms: D000410:Alanine Transaminase / Q000378:metabolism; D001219:Aspartate Aminotransferases / Q000378:metabolism; D016207:Cytokines / Q000097:blood*; D020804:Encephalitis, Varicella Zoster / Q000097:blood* / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed
chemical_list: D016207:Cytokines; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase