All issues > Volume 44(8); 2001

Case Report

J Korean Pediatr Soc. 2001;44(8):954-958. Published online August 15, 2001.

A Case of Acute Necrotizing Encephalopathy

Sung Kee SK Kim¹, Se Wook SW Oh¹, Young Kyoun YK Kim¹, Se Chang SC Ham¹, Yong Won YW Park¹, Sang Woo SW 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

Correspondence Se Wook SW 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.

Keywords :Acute encephalopathy