지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2019.1
- 수록면
- 1 - 9 (9page)
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Background: Acute necrotizing encephalopathy (ANE) is a rare, but potentially life threatening neurological condition in children. This study aimed to investigate its clinical spectrum, diagnostic and therapeutic dilemma, and prognosis. Methods: Twelve children with ANE were included in the study. The diagnosis was made by clinical and radiological characteristics from January 1999 to December 2017 and their clinical data were retrospectively analyzed. Results: A total of 12 children aged 6 to 93 months at onset (5 male: 7 female) were evaluated. The etiology was found in 4 of them (influenza A, H1N1; coxsackie A 16; herpes simplex virus; and RANBP2 gene/mycoplasma). The most common initial presentations were seizures (67%) and altered mental status (58%). The majority of the subjects showed elevation of aspartate aminotransferase/alanine aminotransferase with normal ammonia and increased cerebrospinal fluid protein without pleocytosis. Magnetic resonance imaging revealed increased T2 signal density in bilateral thalami in all patients, but the majority of the subjects (67%) also had lesions in other areas including tegmentum and white matter. Despite the aggressive immunomodulatory treatments, the long-term outcome was variable. One child and two sisters with genetic predisposition passed away. Conclusion: ANE is a distinctive type of acute encephalopathy with diverse clinical spectrum. Even though the diagnostic criteria are available, they might not be watertight. In addition, treatment options are still limited. Further studies for better outcome are needed.
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참고문헌 (32)
참고문헌 신청
Mizuguchi M / 1995 / Acute necrotising encephalopathy of childhood : a new syndrome presenting with multifocal, symmetric brain lesions / J Neurol Neurosurg Psychiatry 58(5):555~561
Mizuguchi M / 1997 / Acute necrotizing encephalopathy of childhood : a novel form of acute encephalopathy prevalent in Japan and Taiwan / Brain Dev 19(2):81~92
Wang HS / 1996 / Reports of acute encephalopathy with bilateral thalamotegmental involvement / AJNR Am J Neuroradiol 17(2):398~399
Aydin H / 2010 / Acute necrotizing encephalopathy secondary to diphtheria, tetanus toxoid and whole-cell pertussis vaccination : diffusion-weighted imaging and proton MR spectroscopy findings / Pediatr Radiol 40(7):1281~1284
Komur M / 2011 / Acute necrotizing encephalopathy associated with novel influenza A(H1N1)virus in Turkey / J Pak Med Assoc 61(12):1237~1239
Mizuguchi M / 1997 / Acute necrotizing encephalopathy of childhood : a novel form of acute encephalopathy prevalent in Japan and Taiwan / Brain Dev 19(2):81~92
Wang HS / 1996 / Reports of acute encephalopathy with bilateral thalamotegmental involvement / AJNR Am J Neuroradiol 17(2):398~399
Aydin H / 2010 / Acute necrotizing encephalopathy secondary to diphtheria, tetanus toxoid and whole-cell pertussis vaccination : diffusion-weighted imaging and proton MR spectroscopy findings / Pediatr Radiol 40(7):1281~1284
Komur M / 2011 / Acute necrotizing encephalopathy associated with novel influenza A(H1N1)virus in Turkey / J Pak Med Assoc 61(12):1237~1239
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