Department of Neurology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany.
Department of Neurology, University of Florida, Gainesville, FL, USA.
Eur J Neurol. 2019 Apr;26(4):566-e41. doi: 10.1111/ene.13906. Epub 2019 Feb 8.
John Cunningham virus (JCV) infection of the central nervous system causes progressive multifocal leukoencephalopathy (PML) in patients with systemic immunosuppression. With the increased application of modern immunotherapy and biologics in various immune-mediated disorders, the PML risk spectrum has changed. Thus, new tools and strategies for risk assessment and stratification in drug-associated PML such as the JCV antibody indices have been introduced. Imaging studies have highlighted atypical presentations of cerebral JCV disease such as granule cell neuronopathy. Imaging markers have been developed to differentiate PML from new multiple sclerosis lesions and to facilitate the early identification of pre-clinical manifestations of PML and its immune reconstitution inflammatory syndrome. PML can be diagnosed either by brain biopsy or by clinical, radiographic and virological criteria. Experimental treatment options including immunization and modulation of interleukin-mediated immune response are emerging. PML should be considered in any patient with compromised systemic or central nervous system immune surveillance presenting with progressive neurological symptoms.
约翰·坎宁安病毒（JCV）感染中枢神经系统会导致系统性免疫抑制患者发生进行性多灶性脑白质病（PML）。随着现代免疫疗法和生物制剂在各种免疫介导性疾病中的广泛应用，PML 的风险谱发生了变化。因此，出现了用于药物相关 PML 风险评估和分层的新工具和策略，如 JCV 抗体指标。影像学研究强调了脑 JCV 疾病的非典型表现，如颗粒细胞神经元病。已经开发出成像标志物来区分 PML 与新的多发性硬化病变，并有助于早期识别 PML 及其免疫重建炎症综合征的临床前表现。PML 可以通过脑活检或临床、影像学和病毒学标准来诊断。正在出现包括免疫接种和白细胞介素介导的免疫反应调节在内的实验性治疗选择。任何出现进行性神经症状、全身或中枢神经系统免疫监视受损的患者都应考虑 PML。
