Department of Neurology, Rockefeller Neuroscience Institute, West Virginia University, Morgantown, West Virginia, USA.
Department of Neurology, Wayne State University, Detroit, Michigan, USA.
J Med Virol. 2022 Jun;94(6):2860-2869. doi: 10.1002/jmv.27493. Epub 2021 Dec 13.
Progressive multifocal leukoencephalopathy (PML), a demyelinating disease of the brain, caused by the John Cunningham virus (JCV) is usually seen in patients who are immunocompromised. Here, we describe a case of an immunocompetent patient diagnosed with PML and a comprehensive literature review. A 64-year-old Caucasian male presented with acute worsening of progressive neurological decline with difficulty in vision and reading. Based on history, examination, cerebrospinal fluid markers, histopathology, and magnetic resonance imaging brain at the time of presentation diagnosed the patient with PML in a setting of no immunosuppression disorder. The patient was started on Pelfilgrastim with significant systematic improvement. In our literature review, it was seen that the average age of symptom presentation was 57.5 with predominance in males. Most of the patients presented with progressive neurological deficits with symptomology ranging from mild confusion, aphasia, anxiety to sensory disturbances with numbness, hemiparesis, and hemianopsia. Out of the 21 cases, patients responded to mirtazapine and intravenous pulse methylprednisolone (IVMP). The mortality rate was close to 50% with 11 fatal cases and 10 nonfatal cases. Our case and literature review demonstrate the possibility that PML may very rarely occur in patients that are immunocompetent. Furthermore, our review showed that patients responded well to mirtazapine and IVMP. We also want to highlight that the mortality rate was lower in this review and was only compared to mortality in PML associated with immunocompromised status.
进行性多灶性白质脑病（PML）是一种由约翰·坎宁安病毒（JCV）引起的脑脱髓鞘疾病，通常发生在免疫功能低下的患者中。在这里，我们描述了一例免疫功能正常的 PML 患者，并进行了全面的文献复习。一名 64 岁的白人男性因进行性神经功能恶化伴视力和阅读困难而急性加重。根据病史、检查、脑脊液标志物、组织病理学和发病时的脑部磁共振成像，诊断患者在无免疫抑制障碍的情况下患有 PML。患者开始接受培非格司亭治疗，症状有明显系统改善。在我们的文献复习中，发现症状出现的平均年龄为 57.5 岁，男性居多。大多数患者出现进行性神经功能缺损，症状从轻度意识模糊、失语、焦虑到感觉障碍，表现为麻木、偏瘫和偏盲。在 21 例患者中，有 15 例患者对米氮平和静脉注射脉冲甲基强的松龙（IVMP）有反应。死亡率接近 50%，有 11 例死亡病例和 10 例非死亡病例。我们的病例和文献复习表明，免疫功能正常的患者也可能很少发生 PML。此外，我们的综述显示，患者对米氮平和 IVMP 反应良好。我们还想强调的是，在本综述中死亡率较低，仅与免疫抑制状态相关的 PML 死亡率进行了比较。
