The 50th birthday of progressive multifocal leukoencephalopathy: New insights into pathogenesis.
The 50th birthday of progressive multifocal leukoencephalopathy: New insights into pathogenesis.
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DOI：
10.1002/ana.21486
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发表时间：
2008-10
影响因子：
11.2
通讯作者：
Tyler, Kenneth L.
中科院分区：
文献类型：
--
作者：
Tyler, Kenneth L.
进行性多灶性白质脑病（PML）是一种由JC多瘤病毒引起的中枢神经系统机会性脱髓鞘疾病，Astrom、Mancall和Richardson在半个世纪前首次对其进行了描述（1）。当JC病毒有效感染少突胶质细胞导致其溶解性破坏时，可能通过涉及细胞凋亡的机制，导致脱髓鞘，从而发生PML。在用几种免疫调节剂治疗期间出现的PML病例，所述免疫调节剂对于治疗免疫介导的神经和非神经疾病具有很大的治疗前景，包括那他珠单抗（Tysabri）、利妥昔单抗（Rituxan）和吗替麦考酚酯（CellCept）（参见http：//www.mycophenolatemofetil）。FDA. gov/medwatch/safety/2008/safety08。JC病毒本身是一种普遍存在的病原体，血清流行病学研究表明，大多数人在成年前就已被感染（2）。原发性感染发生在儿童期，无症状或亚临床。初次感染后，病毒在宿主体内潜伏，在绝大多数个体中持续存在，并不引人注目。是什么因素使这种通常无害和无致病性的病毒以PML的形式产生毁灭性的神经系统疾病？
Progressive multifocal leukoencephalopathy (PML) is an opportunistic demyelinating disease of the central nervous system caused by JC polyomavirus, that was initially described by Astrom, Mancall and Richardson half a century ago (1). PML develops when JC virus productively infects oligodendrocytes leading to their lytic destruction, possibly through mechanisms involving apoptosis, with resulting demyelination. Cases of PML emerging during treatment with several immunomodulatory agents of great therapeutic promise for treatment of immune-mediated neurological and non-neurological disease, including natalizumab (Tysabri), rituximab (Rituxan), and mycophenolate mofetil (CellCept)(see http://www. fda. gov/medwatch/safety/2008/safety08. htm), has re-focused emphasis on understanding the pathogenesis of these devastating and currently untreatable neurological disorder.JC virus itself is a ubiquitous agent, and seroepidemiological studies suggest that the majority of the population has been infected before reaching adult life (2). Primary infection occurs in childhood and is asymptomatic or subclinical. Following primary infection virus becomes latent in the host, persisting unobtrusively in the overwhelming majority of individuals for their lifetime. What are the factors that allow this normally innocuous and apathogenic virus to produce devastating neurological disease in the form of PML?
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影响因子：
5.4
作者：
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