시상 출혈을 동반한 급성 괴사성 뇌증 1례

A Case of Acute Necrotizing Encephalopathy with a Thalamic Hemorrhage.

저자들은 생후 6개월 환아에서 급성 호흡기 감염 후 급격하게 발생한 의식 장애와 경련 등의 신경학적 증상과 뇌자기공명영상으로 양측 시상, 뇌간, 뇌실 주위의 백직을 대칭적, 다발적으로 침범한 전형적인 급성 괴사상 뇌증을 진단하였고, steroid를 투여하여 입상 증상 및 뇌 병변의 호전을 보인 1례를 경험하였기에 보고하는 바이다.

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.