Department of Neurology and St Vincent's Center for Applied Medical Research, St Vincent's Hospital and University of New South Wales, Victoria Street, Sydney, NSW 2010, Australia.
Nat Rev Neurol. 2010 Dec;6(12):667-79. doi: 10.1038/nrneurol.2010.164.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the JC virus (JCV). PML usually occurs via reactivation of JCV when an immune system becomes compromised. A diagnosis of PML is normally made on the basis of distinguishing neurological features at presentation, characteristic brain MRI changes and the presence of JCV DNA in cerebrospinal fluid. PML has a 3 month mortality rate of 20-50%, so prompt intervention is essential. Currently, reconstitution of the immune system affords the best prognosis for this condition. When PML is first suspected, and where possible, immunosuppressant or immunomodulatory therapy should be suspended or reduced. If PML is associated with a protein therapy that has a long half-life the use of plasma exchange to accelerate the removal of the drug from the circulation may aid the restoration of immune system function. Rapid improvements in immune function, however, might lead to transient worsening of the disease. In this Review, we critically appraise the controversies surrounding JCV infection, and provide practical management guidelines for PML.
进行性多灶性白质脑病（PML）是一种由 JC 病毒（JCV）引起的脑脱髓鞘疾病。当免疫系统受损时，JCV 通常会被重新激活而导致 PML。PML 的诊断通常基于以下几点：发病时的神经学特征、特征性脑 MRI 改变以及脑脊液中 JCV DNA 的存在。PML 的 3 个月死亡率为 20-50%，因此及时干预至关重要。目前，重建免疫系统为这种情况提供了最好的预后。当首次怀疑 PML 时，在可能的情况下，应暂停或减少免疫抑制剂或免疫调节剂治疗。如果 PML 与半衰期较长的蛋白治疗相关，则使用血浆置换加速药物从循环中清除可能有助于恢复免疫系统功能。然而，免疫功能的快速改善可能导致疾病的短暂恶化。在这篇综述中，我们批判性地评估了与 JCV 感染相关的争议，并为 PML 提供了实用的管理指南。
