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A Case of Acute Necrotizing Encephalopathy

Journal of the Korean Pediatric Society 2001;44(8):954-958.
Published online August 15, 2001.
A Case of Acute Necrotizing Encephalopathy
Sung Kee Kim1, Se Wook Oh1, Young Kyoun Kim1, Se Chang Ham1, Yong Won Park1, Sang Woo Kim2
1Department of Pediatrics, College of Medicine, Inje University, Seoul Paik Hospital, Seoul, Korea
2Department of Pediatrics, College of Medicine, Inje University, Sang-Gye Paik Hospital, Seoul, Korea
급성 괴사성 뇌증 1례
김성기1, 오세욱1, 김영균1, 함세창1, 박용원1, 김상우2
1인제대학교 의과대학 소아과학교실 서울백병원
2인제대학교 의과대학 소아과학교실 상계백병원
Correspondence: 
Se Wook Oh, Email: sohmd@orgio.net
Abstract
This disease predominantly affects infants and young children living in Japan and Taiwan, and manifests itself as acute encephalopathy following viral infections. The hallmark of this encephalopathy is multifocal, symmetric brain lesions affecting bilateral thalamus, brainstem tegmentum, cerebral periventricular white matter and cerebellar medulla, which can be visualized by computed tomography and magnetic resonance imaging. Both the gray and white matter are involved, with neuropathological evidence of local breakdown of the blood-brain barrier. The prognosis was poor in the 1980s, but has improved recently. A characteristic combination of focal neurologic signs is often recognized as the sequelae. We experienced a case of a 7-month-old male infant who had been transferred to our hospital for comatous mentality and clonic seizure of both arms and legs. We report this case with a review of the related literatures.
Key Words: Acute encephalopathy


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