시상 출혈을 동반한 급성 괴사성 뇌증 1례

• 영문명: A Case of Acute Necrotizing Encephalopathy with a Thalamic Hemorrhage.
• 발행기관: 대한소아신경학회
• 저자명: 박상수(Sang Soo Park) 노규태(Kyu Tae Noh) 최순섭(Sun Seob Choi) 황규근(Kyu Geun Hwang)
• 간행물 정보: 『Annals of Child Neurology(구 대한소아신경학회지)』대한소아신경학회지 제12권 제2호, 223~228쪽, 전체 6쪽
• 주제분류: 의약학 > 의학일반
• 파일형태: PDF
• 발행일자: 2004.11.30

국문 초록

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.

영문 초록

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.

키워드

Acute encephalopathy Thalamic hemorrhage