Clinical spectrum and prognostic factors of acute necrotizing encephalopathy in children.

Abstract	This study was conducted to investigate the etiology, the clinical characteristics and prognosis of acute necrotizing encephalopathy (ANE) in Korean children. Six children (1 yr to 7 yr) patients with ANE were enrolled. They were diagnosed by clinical and radiological characteristics and their clinical data were retrospectively analyzed. In a search of clinically plausible causes, brain MRI in all patients, mitochondrial DNA studies for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and myoclonus epilepsy and ragged red fibers (MERRF) in four patients, and genomic typing on HLA DRB/HLA DQB genes in three patients were performed. All had precedent illnesses and the main initial symptoms included mental change (83%), seizures (50%), and focal deficits (50%). MRI revealed increased T2 signal density in the bilateral thalami and/or the brainstem in all patients. Mitochodrial DNA studies for MELAS and MERRF were negative in those children and HLA-DRB11401, HLA-DRB30202, and HLA-DQB1*0502 seemed to be significant. A high dose steroid was given to all patients, which seemed to be partly effective except for 2 patients. In conclusion, ANE is relatively rare, but can result in serious neurological complication in children. Early detection and appropriate treatment may lead to a better neurological outcome.
Authors	Hye-Eun Seo, Su-Kyeong Hwang, Byung Ho Choe, Min-Hyun Cho, Sung-Pa Park, Soonhak Kwon
Journal	Journal of Korean medical science (J Korean Med Sci) Vol. 25 Issue 3 Pg. 449-53 (Mar 2010) ISSN: 1598-6357 [Electronic] Korea (South)
PMID	20191046 (Publication Type: Journal Article)
Chemical References	HLA-DQ Antigens HLA-DQ beta-Chains HLA-DQB1 antigen HLA-DR Antigens HLA-DRB1 Chains HLA-DRB3 Chains
Topics	Child Child, Preschool Female HLA-DQ Antigens (metabolism) HLA-DQ beta-Chains HLA-DR Antigens (metabolism) HLA-DRB1 Chains HLA-DRB3 Chains Humans Infant Korea Leukoencephalitis, Acute Hemorrhagic (diagnosis, etiology, pathology, physiopathology) MELAS Syndrome (pathology, physiopathology) MERRF Syndrome (pathology, physiopathology) Magnetic Resonance Imaging Male Prognosis Retrospective Studies

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.

Realize the full power of the drug-disease research graph!