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[进行性风疹全脑炎]

[Progressive rubella panencephalitis].

作者信息

Kuroda Y, Matsui M

机构信息

Department of Internal Medicine, Saga Medical School.

出版信息

Nihon Rinsho. 1997 Apr;55(4):922-5.

PMID:9103895
Abstract

Progressive rubella panencephalitis (PRP) is a slow virus infection of the central nervous system. PRP was first reported in 1974, and fewer than 20 cases have been reported since then. All patients were male who were between the ages of 8 and 21 years at onset, and most had signs of congenital rubella syndrome. Although PRP may exhibit clinical features resembling SSPE, the age at onset is much older and the clinical course is more benign. The main neurological features of PRP are dementia, cerebellar ataxia, and seizures. Increases in antirubella antibody titer and IgG are found in the CSF and diffuse atrophy of the brain with ventricular dilatation may be found on MRI. The pathomechanism of PRP remains unclarified.

摘要

进行性风疹全脑炎(PRP)是一种中枢神经系统的慢病毒感染。PRP于1974年首次报道,自那时起报告的病例少于20例。所有患者均为男性,发病年龄在8至21岁之间,大多数有先天性风疹综合征的体征。尽管PRP可能表现出类似于亚急性硬化性全脑炎(SSPE)的临床特征,但其发病年龄要大得多,临床病程也更为良性。PRP的主要神经学特征是痴呆、小脑共济失调和癫痫发作。脑脊液中抗风疹抗体滴度和IgG升高,MRI可能显示脑弥漫性萎缩伴脑室扩张。PRP的发病机制仍未阐明。

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