Warnke C, Wattjes M P, Adams O, Hartung H-P, Martin R, Weber T, Stangel M
Klinik für Neurologie, Medizinische Fakultät, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Deutschland.
Abteilung für Radiologie und Nuklearmedizin, VU University Medical Center, Amsterdam, Niederlande.
Nervenarzt. 2016 Dec;87(12):1300-1304. doi: 10.1007/s00115-016-0225-7.
Progressive multifocal leukoencephalopathy (PML) is a disease of immunosuppressed patients caused by the JC polyomavirus (JCPyV). Due to the elevated risk in patients treated with natalizumab for multiple sclerosis (MS) and also treatment with other biologicals for different indications, the relevance of PML has increased in recent years. This article summarizes the published knowledge on the biology and pathogenesis of PML with a focus on the role of cerebrospinal fluid diagnostics in the work-up for PML and the current PML case definition. Current recommendations regarding risk management are discussed, as are possible therapies and prevention.
进行性多灶性白质脑病（PML）是一种由JC多瘤病毒（JCPyV）引起的免疫抑制患者疾病。由于接受那他珠单抗治疗的多发性硬化症（MS）患者以及接受其他生物制剂治疗不同适应症患者的风险升高，近年来PML的相关性有所增加。本文总结了已发表的关于PML生物学和发病机制的知识，重点关注脑脊液诊断在PML检查中的作用以及当前的PML病例定义。讨论了关于风险管理的当前建议以及可能的治疗方法和预防措施。
