Progressive Multifocal Leukoencephalopathy with Inflammatory Reactions
Progressive Multifocal Leukoencephalopathy with Inflammatory Reactions
复制标题
伴有炎症反应的进行性多灶性白质脑病
DOI：
10.11477/mf.1416200422
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发表时间：
2016
期刊：
影响因子：
--
通讯作者：
三條伸夫
中科院分区：
文献类型：
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作者：
宍戸-原 由紀子;内原俊記;三條伸夫
进行性多灶性白质脑病 (PML) 是一种脱髓鞘疾病，由与宿主免疫力受损相关的 JC 病毒重新激活引起。然而，即使没有明显的免疫抑制原因，PML 也可能发生。在这种情况下，聚合酶链式反应 (PCR) 可能无法检测到脑脊液 (CSF) 中的 JC 病毒 DNA，此时可能需要进行脑活检。病理学可能会在 JC 病毒感染的细胞周围表现出明显的炎症反应，但很少会看到典型的核内病毒包涵体。 PML 患者的非典型炎症可能表明宿主对病毒的免疫反应。这通常发生在那些免疫抑制相对较轻且预后良好的患者中。在此，我们描述了表现出炎症反应的 PML 病例；最近的一个主题是伴有免疫重建炎症综合征 (IRIS) 的 PML，也将进行讨论。
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder caused by reactivation of the JC virus associated with impaired host immunity. However, PML may occur even without an evident cause of immunosuppression. In such cases, JC virus DNA in cerebrospinal fluid (CSF) may not be detectable with polymerase chain reactions (PCR), and a brain biopsy may be performed. Pathology may exhibit marked inflammatory reactions around the JC virus-infected cells, although typical intranuclear viral inclusions would rarely be seen. Atypical inflammation in patients with PML likely an indication of the host's immune-response against the virus. this is usually observed in those with relatively-mild immunosuppression with favorable prognosis. Here, we describe cases of PML that exhibited inflammatory reactions; a recent topic, PML with immune reconstruction inflammatory syndrome (IRIS), will also be discussed.