Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS
Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS
复制标题
DOI：
10.1136/jnnp-2017-316886
复制
发表时间：
2018-05-01
影响因子：
11
通讯作者：
Killestein, Joep
中科院分区：
文献类型：
--
作者：
Wattjes, Mike P.;Wijburg, Martijn T.;Killestein, Joep
背景和目的纳talizumab相关的进行性多灶性白质脑病（NTZ-PML）患者在诊断时可能表现出提示炎症的影像学征象（“炎症性PML”），使人联想到PML-免疫重建炎症综合征（PML- iris）。我们研究了炎性NTZ-PML病变和PML-IRIS的影像学特征，以确定其区分和重叠特征。方法对炎症性NTZ-PML患者的脑MRI扫描进行炎症的存在、定位和影像特征模式评分。随访影像学特征，直至出现PML-IRIS。结果44例NTZ-PML患者中有10例在诊断时出现炎症征象。诊断时的炎症模式与PML-IRIS相似，对比增强是最常见的炎症征象（诊断时90%，PML-IRIS 100%）。然而，炎症的严重程度不同，与PML-IRIS阶段（40%）相比，诊断时没有肿胀和低频率的病灶周围水肿（10%）。结论PML诊断时与PML- iris阶段的炎症模式有重叠，但炎症严重程度不同。这支持了组织病理学证据，即在同一疾病的两个阶段所见的炎症具有相似的潜在病理生理学，代表了对JC病毒的免疫反应的不同程度。
Background and objective Natalizumab-associated progressive multifocal leukoencephalopathy (NTZ-PML) patients may show imaging signs suggestive of inflammation at diagnosis ('inflammatory PML'), reminiscent of PML-immune reconstitution inflammatory syndrome (PML-IRIS). We investigated the imaging characteristics of inflammatory NTZ-PML lesions and PML-IRIS to determine differentiating and overlapping features.Methods We scored the presence, localisation and pattern of imaging characteristics of inflammation on brain MRI scans of inflammatory NTZ-PML patients. The imaging characteristics were followed up until the occurrence of PML-IRIS.Results Ten out of the 44 NTZ-PML patients included showed signs suggestive of inflammation at the time of diagnosis. The inflammation pattern at diagnosis was similar to the pattern seen at PML-IRIS, with contrast enhancement representing the most frequent sign of inflammation (90% at diagnosis, 100% at PML-IRIS). However, the severity of inflammation differed, with absence of swelling and low frequency of perilesional oedema (10%) at diagnosis, as compared with the PML-IRIS stage (40%).Conclusion Patterns of inflammation at the time of PML diagnosis and at the PML-IRIS stage overlap but differ in their severity of inflammation. This supports histopathological evidence that the inflammation seen at both stages of the same disease shares a similar underlying pathophysiology, representing the immune response to the JC virus to a variable extend.