Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
J Neuroimmunol. 2011 Sep 15;238(1-2):81-6. doi: 10.1016/j.jneuroim.2011.07.003. Epub 2011 Aug 12.
Progressive multifocal leukoencephalopathy (PML) is typically associated with minimal inflammation; however, patients may develop an inflammatory response due to immune reconstitution (IRIS). The authors aimed to determine if characteristics and outcomes of PML are altered in those with IRIS. A retrospective records review was performed on 87 patients diagnosed with PML at Johns Hopkins, 27 of which had a syndrome consistent with IRIS. Gadolinium enhancement on MRI occurred in 44.4% of cases of PML-IRIS versus 5.1% in PML (p<0.05), and thus had low diagnostic sensitivity and specificity. In HIV+ cases, CD4 counts were lower in those who later developed IRIS (mean 34.8 vs. 71.7, p<0.05) and was predictive of the development of IRIS (p<0.05). Improved prognosis was seen with higher cerebrospinal fluid (CSF) white blood cell counts and protein levels, but not for gadolinium enhancement and there were no differences in survival for PML versus PML-IRIS.
进行性多灶性白质脑病（PML）通常与最小炎症相关；然而，患者可能由于免疫重建（IRIS）而产生炎症反应。作者旨在确定患有 IRIS 的患者的 PML 特征和结局是否发生改变。对约翰霍普金斯大学诊断为 PML 的 87 名患者进行了回顾性记录审查，其中 27 名患者的综合征与 IRIS 一致。PML-IRIS 中出现钆增强 MRI 的比例为 44.4%，而 PML 中为 5.1%（p<0.05），因此具有低诊断敏感性和特异性。在 HIV+病例中，后来发生 IRIS 的患者的 CD4 计数较低（平均 34.8 对 71.7，p<0.05），并且是 IRIS 发展的预测因素（p<0.05）。脑脊液（CSF）白细胞计数和蛋白水平较高与预后改善相关，但与钆增强无关，PML 与 PML-IRIS 的生存率无差异。
