A Case of Acute Necrotizing Encephalopathy with a Thalamic Hemorrhage / 대한소아신경학회지

ABSTRACT

Acute necrotizing encephalopathy predominantly affects young children and infants living in Japan and Taiwan, and is characterised by acute encephalopathy with seizures and decreased level of consciousness. The Hallmark of the disease is diffuse and symmetrical CNS lesions of both thalami, brainstem tegmentum, cerebral periventricular white matter and cerebellar medula. The clinical, radiological and pathological features of this disease, a disease entity established recently, is proposed by Masashi Mizuguchi et al in 1995. The aetiology is unknown but infectious or parainfectious process seems likely. The diagnosis can be made without difficulty on the basis of the combination of a typical clinical figures and characteristic radiologic findings. There is no specific therapy or prevention. The prognosis was poor in the 1980s but has improved recently. We experienced a case of 6-month-old female infant with acute necrotizing encephalopathy and a thalamic hemorrhage. We report this case with a review of the related literatures.