Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

被引:118
作者
Wu, Xiujuan [1 ]
Wu, Wei [2 ]
Pan, Wei [3 ]
Wu, Limin [1 ,4 ]
Liu, Kangding [1 ]
Zhang, Hong-Liang [1 ]
机构
[1] Jilin Univ, Neurosci Ctr, Dept Neurol, Hosp 1, Changchun 130021, Peoples R China
[2] Jilin Univ, Dept Neurovasc Surg, Hosp 1, Changchun 130021, Peoples R China
[3] Jilin Univ, Sch Publ Hlth, Changchun 130021, Peoples R China
[4] Harvard Univ, Sch Med, Neuroprotect Res Lab, Massachusetts Gen Hosp, Charlestown, MA 02129 USA
基金
中国国家自然科学基金;
关键词
CEREBROSPINAL-FLUID; IMAGING FINDINGS; COMBINED THERAPY; INFLUENZA; CHILDHOOD; VIRUS; CHILDREN; HYPOTHERMIA; INFECTION; DIPHTHERIA;
D O I
10.1155/2015/792578
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Acute necrotizing encephalopathy (ANE) is a rare but distinctive type of acute encephalopathy with global distribution. Occurrence of ANE is usually preceded by a virus-associated febrile illness and ensued by rapid deterioration. However, the causal relationship between viral infections and ANE and the exact pathogenesis of ANE remain unclear; both environmental and host factors might be involved. Most cases of ANE are sporadic and nonrecurrent, namely, isolated or sporadic ANE; however, few cases are recurrent and with familial episodes. The recurrent and familial forms of ANE were found to be incompletely autosomal-dominant. Further the missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 (RANBP2) were identified. Although the clinical course and the prognosis of ANE are diverse, the hallmark of neuroradiologic manifestation of ANE is multifocal symmetric brain lesions which are demonstrated by computed tomography (CT) or magnetic resonance imaging (MRI). The treatment of ANE is still under investigation. We summarize the up-to-date knowledge on ANE, with emphasis on prompt diagnosis and better treatment of this rare but fatal disease.
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页数:10
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