Aicardi-Goutieres syndrome

被引：69

作者：

Goutières, F

机构：

来源：

BRAIN & DEVELOPMENT | 2005年 / 27卷 / 03期

关键词：

familial encephalopathy; basal ganglia calcifications; CSF lymphocytosis; interferon-alpha;

D O I：

10.1016/j.braindev.2003.12.011

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Aicardi-Goutieres syndrome is a familial progressive early onset encephalopathy with basal ganglia calcifications, chronic CSF lymphocytosis and high level of interferon-alpha in CSF. Cutaneous necrotic lesions and the neuropathological aspect of microangiopathy and microinfarctions suggest a vascular process in relation to elevated interferon-alpha. A genetic defect in the regulation of its synthesis may be the causal factor of the disorder. (c) 2004 Published by Elsevier B.V.

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页码：201 / 206

页数：6

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