Ojeda Chris, Assina Rachid, Barry Maureen, Baisre Ada, Gandhi Chirag
Biomedical Engineering, Rutgers New Jersey Medical School; Newark, NJ, USA -
Neurosurgery Department, Rutgers New Jersey Medical School; Newark, NJ, USA.
Neuroradiol J. 2014 Jun;27(3):322-6. doi: 10.15274/NRJ-2014-10040. Epub 2014 Jun 17.
Progressive multifocal leukoencephalopathy (PML) is an opportunistic demyelinating disease of the CNS caused by the JC papovavirus (JCV). Demyelination due to oligodendrocyte death leads to multifocal, asymmetric lesions. MRI is a valuable tool for detecting and differentiating PML from other neuropathies. Radiographically, PML classically presents as bilateral, subcortical white matter lesions with a lack of brain atrophy. As the disease progresses, lesions become larger and coalesce to become confluent. Minor edema and mass effect are infrequently described and the presence of significant mass effect suggests an alternative diagnosis. In our case, a patient demonstrated atypical marked infratentorial mass effect. Bilaterally, cerebellar lesions with associated mass effect were observed, as well as effacement of cerebellar folia and partial effacement of the fourth ventricle. The diagnosis of PML was confirmed with a biopsy of the right cerebellar lesion showing classic PML histology, with JCV DNA detection by polymerase chain reaction in the biopsy material.
进行性多灶性白质脑病（PML）是一种由JC多瘤病毒（JCV）引起的中枢神经系统机会性脱髓鞘疾病。少突胶质细胞死亡导致的脱髓鞘会引起多灶性、不对称性病变。磁共振成像（MRI）是检测PML并将其与其他神经病变区分开来的重要工具。在影像学上，PML典型表现为双侧皮质下白质病变且无脑萎缩。随着疾病进展，病变会变大并融合。很少描述有轻微水肿和占位效应，而显著占位效应的出现提示需考虑其他诊断。在我们的病例中，一名患者表现出非典型的明显幕下占位效应。双侧观察到小脑病变伴有相关占位效应，以及小脑小叶消失和第四脑室部分受压。通过对右侧小脑病变进行活检，显示典型的PML组织学特征，并在活检材料中通过聚合酶链反应检测到JCV DNA，从而确诊为PML。
