PDF(Pubmed)
Abstract：
Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating infectious disease of the central nervous system, primarily affecting immunosuppressed individuals, such as those with acquired immunodeficiency syndrome (AIDS) or undergoing immunosuppressive therapy. The causative agent is the dormant John Cunningham (JC) polyomavirus, which reactivates in immunocompromised patients. PML is diagnosed through clinical observations, imaging, and polymerase chain reaction (PCR) analysis, detecting JC virus deoxyribonucleic acid (DNA) in the cerebrospinal fluid (CSF). Here, we report a case of a 42-year-old male, recently diagnosed with human immunodeficiency virus (HIV), who presented with slurred speech, difficulty articulating, tingling in both feet, difficulty walking, and significant weight loss. Examination revealed absent reflexes, coordination impairment, and diminished vibration sense. Blood tests showed anemia, elevated D-dimer, and HIV-1 positivity with a low CD4 count. CSF analysis indicated a lymphocytic profile with elevated protein and marginally increased adenosine deaminase (ADA). Autoantibody testing was positive for antinuclear antibodies (ANA), but CSF culture and India ink staining were negative. Magnetic resonance imaging (MRI) of the brain revealed hyperintense lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the left peritrigonal and parietal white matter, suggesting demyelination. The diagnosis of PML was confirmed by a positive JC virus PCR result from the CSF. The patient was started on combination antiretroviral therapy (cART) and supportive measures to improve immune status. This case underscores the importance of considering PML in patients with new-onset neurological symptoms and immunosuppression.
摘要：
进行性多灶性白质脑病（PML）是一种罕见的，中枢神经系统脱髓鞘传染病，主要影响免疫抑制个体，例如获得性免疫缺陷综合征（AIDS）或接受免疫抑制治疗的患者。致病因子是休眠的约翰·坎宁安（JC）多瘤病毒，在免疫功能低下的患者中重新激活。PML是通过临床观察诊断的，成像,和聚合酶链反应（PCR）分析，检测脑脊液（CSF）中的JC病毒脱氧核糖核酸（DNA）。这里,我们报道了一个42岁的男性病例,最近被诊断出患有人类免疫缺陷病毒（HIV），他发表了含糊不清的演讲，难以表达，双脚刺痛，行走困难,和显著的体重减轻。检查显示反应缺失，协调障碍，和减弱的振动感。血液检查显示贫血,D-二聚体升高,和低CD4计数的HIV-1阳性。CSF分析表明淋巴细胞谱具有升高的蛋白质和略微增加的腺苷脱氨酶(ADA)。自身抗体检测为抗核抗体(ANA)阳性,但脑脊液培养和印度墨水染色均为阴性。大脑的磁共振成像（MRI）显示，在左甲状腺和顶叶白质的T2加权和液体衰减倒置恢复（FLAIR）图像上，提示脱髓鞘.通过来自CSF的阳性JC病毒PCR结果证实了PML的诊断。患者开始接受抗逆转录病毒联合治疗（cART）和支持性措施以改善免疫状态。该病例强调了在具有新发神经系统症状和免疫抑制的患者中考虑PML的重要性。
