Acute necrotizing encephalopathy associated with hemophagocytic syndrome

Pediatric Neurology
Kensuke AkiyoshiTatsuro Izumi

Abstract

A 7-year-old female suddenly exhibited high fever and convulsions, and entered a semi-coma. She also had thrombocytopenia, elevated aminotransferase, prolonged prothrombin time and activated partial thromboplastin time, and hemophagocytes in the bone marrow. The brain magnetic resonance imaging revealed multiple low-intensity areas on the T1-weighted images, and high-intensity areas on the T2-weighted images bilaterally in the thalamus, the dorsal part of the pons, and the cerebellar white matter. The patient was diagnosed as having both acute necrotizing encephalopathy and hemophagocytic syndrome. Serum and cerebrospinal fluid interleukin-6 and tumor necrosis factor-alpha were elevated to the same high levels (serum:cerebrospinal fluid interleukin-6, 103:101 pg/mL; tumor necrosis factor-alpha 753:753 pg/mL). The clinical symptoms and the magnetic resonance imaging findings improved immediately after the administration of dexamethasone. These results suggest that the hypercytokinemia and the hyperpermeability of both the blood-brain barrier and the capillary walls of the central nervous system might be essential in the pathogenesis of acute necrotizing encephalopathy, and that early steroid therapy might be effective in these c...Continue Reading

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Citations

Dec 15, 2010·Archives of Neurology·Sita KediaSamuel R Dominguez
Jan 1, 2011·BMJ Case Reports·Regina Maria Papais AlvarengaSolange Camargo
Feb 10, 2010·Pediatrics·Elysa J MarcoJonathan B Strober
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Apr 16, 2015·Mediators of Inflammation·Xiujuan WuHong-Liang Zhang
Jun 17, 2014·Brain & Development·Hiroyuki YamamotoMasashi Mizuguchi
Mar 2, 2010·Journal of Korean Medical Science·Hye-Eun SeoSoonhak Kwon
Dec 17, 2009·The American Journal of Forensic Medicine and Pathology·Meredith A LannB K Kleinschmidt-DeMasters
Feb 19, 2021·PLoS Genetics·Qingtang ShenAlexander F Palazzo
Mar 30, 2021·Annals of Clinical and Translational Neurology·Umberto PensatoFrancesca Bisulli
Jul 1, 2021·Infectious Diseases in Clinical Practice : IDCP·Amaka AwoniyiFolasade Arinze
Feb 3, 2022·Developmental Medicine and Child Neurology·Nurin ChaturE Ann Yeh

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