Clinical Spectrum and Prognostic Factors of Acute Necrotizing Encephalopathy in Children.
10.3346/jkms.2010.25.3.449
- Author:
Hye Eun SEO
1
;
Su Kyeong HWANG
;
Byung Ho CHOE
;
Min Hyun CHO
;
Sung Pa PARK
;
Soonhak KWON
Author Information
1. Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea. shkwon@knu.ac.kr
- Publication Type:Original Article
- Keywords:
Acute Necrotizing Encephalopathy;
Mitochodrial;
MELAS Syndrome;
MERRF Syndrome;
HLA-DR Antigens;
HLA-DQ Antigens
- MeSH:
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
- From:Journal of Korean Medical Science
2010;25(3):449-453
- CountryRepublic of Korea
- Language:English
-
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-DRB1*1401, HLA-DRB3*0202, 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.
