Clinical outcome of acute necrotizing encephalopathy in related to involving the brain stem of single institution in Korea / 소아과
Korean Journal of Pediatrics
; : 264-270, 2014.
Article
in En
| WPRIM
| ID: wpr-101629
Responsible library:
WPRO
ABSTRACT
PURPOSE:
Acute necrotizing encephalopathy (ANE) is a fulminant disease of the brain characterized by bilateral thalamic lesions, and is prevalent among children in East Asia. The prognosis of ANE is usually poor with a high mortality rate and neurological sequelae. This study aimed to delineate the clinical characteristics and prognostic factors of ANE.METHODS:
We retrospectively analyzed clinical data of 399 pediatric patients with encephalitis who were admitted to Samsung Medical Center from December 1998 to March 2011. We enrolled ten patients (11 cases) with ANE and analyzed their demographic, clinical, and neuroimaging data. The location and extent of the brain regions were checked based on fluid-attenuated inversion recovery, T1-, and T2-weighted imaging findings; the presence of contrast enhancement, restricted diffusion, and hemorrhage.RESULTS:
Ten patients were identified, including one patient with two episodes. The median age of onset was 1.5 years (0.4-8.4 years). The mortality rate was 40%, and only 30% of patients survived without neurological sequelae. The definite involvement of the brainstem on brain magnetic resonance imaging was significantly correlated with mortality (P=0.04).CONCLUSION:
Broad and extensive brainstem involvement suggested the fulminant course of ANE. Early diagnosis of ANE before brainstem involvement, through careful identification of symptoms of brain dysfunction, may be the best way to achieve better neurological outcomes.Key words
Full text:
1
Index:
WPRIM
Main subject:
Pediatrics
/
Prognosis
/
Brain
/
Brain Stem
/
Magnetic Resonance Imaging
/
Retrospective Studies
/
Mortality
/
Age of Onset
/
Early Diagnosis
/
Diffusion
Type of study:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Screening_studies
Limits:
Child
/
Humans
Country/Region as subject:
Asia
Language:
En
Journal:
Korean Journal of Pediatrics
Year:
2014
Type:
Article