Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the central nervous system that is caused by John Cunningham virus (JCV). It occurs almost exclusively in immunosuppressed individuals, for example, patients with AIDS and hematological and lymphoreticular malignancies. In this article, we present a review of the literature and 2 case reports with PML. The first report examines a 15-year-old male (who presented with dedicator of cytokinesis 8 deficiency) who was diagnosed as having PML based on characteristic magnetic resonance imaging (MRI) lesions and a positive PCR for JCV in cerebrospinal fluid. He was transferred for bone marrow transplantation after stabilization with therapy of maraviroc and cidofovir. The second report examines a 6-year-old male who presented with encephalitis and was also diagnosed with AIDS. He was diagnosed with PML and started treatment with cidofovir. His clinical status and MRI findings deteriorated rapidly. In immunosuppressive patients who developed encephalopathy, JCV ought to be considered.

中文翻译：

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原发性和继发性免疫缺陷儿童进行性多灶性白质脑病

进行性多灶性白质脑病 (PML) 是一种致命的中枢神经系统脱髓鞘疾病，由约翰坎宁安病毒 (JCV) 引起。它几乎只发生在免疫抑制个体中，例如，患有 AIDS 和血液系统和淋巴网状恶性肿瘤的患者。在本文中，我们回顾了文献和 2 例 PML 病例报告。第一份报告检查了一名 15 岁男性（他出现胞质分裂 8 缺乏症），他根据特征性磁共振成像 (MRI) 病变和脑脊液中 JCV 的阳性 PCR 被诊断为患有 PML。经马拉韦罗和西多福韦治疗稳定后转入骨髓移植。第二份报告检查了一名 6 岁男性，他患有脑炎并被诊断出患有艾滋病。他被诊断出患有 PML，并开始使用西多福韦治疗。他的临床状况和 MRI 结果迅速恶化。在发生脑病的免疫抑制患者中，应考虑 JCV。