Suppr超能文献

西弗吉尼亚大学(WVU)关于进行性多灶性白质脑病(PML)病例、神经影像学相关性和管理的单中心经验。

Single-center experience on progressive multifocal leukoencephalopathy (PML) cases, neuroimaging relevance, and management at West Virginia University (WVU).

机构信息

Department of Neurology, West Virginia University, Morgantown, West Virginia, USA.

Department of Neurology, Wayne State University, Detroit, Michigan, USA.

出版信息

J Med Virol. 2022 Aug;94(8):4015-4022. doi: 10.1002/jmv.27804. Epub 2022 May 2.

Abstract

Progressive multifocal leukoencephalopathy (PML) is an increasingly common and rapidly fatal demyelinating infection of central nervous system caused by the highly prevalent John Cunningham (JC) virus in immunocompromised individuals belonging to all age groups and genders. Human immunodeficiency virus (HIV) is the most common predisposing factor among other immunodeficient conditions leading to reactivation and multiple neurological symptoms. It has varied findings on magnetic resonance imaging (MRI) and diagnosis is confirmed by positive JC virus in cerebrospinal fluid (CSF). We report 12 confirmed cases of PML from a single academic center. We comprehensively described clinical presentations, risk factors, CSF and neuroimaging findings, treatment and outcome for these cases of PML, a rare disease. The cases were almost equivalently distributed among young and old age groups and both genders. Positive JC virus on CSF was present in the majority of cases along with mild to severe reduction in lymphocyte counts. Significant MRI changes were present in all cases ranging from T2 hypertense signals to white matter lesions in various regions. Treatment with the reversion of immune-modulators, optimization of antiviral therapy (ART), plasmapheresis (PLEX), IVIG, Mirtazapine, oral steroids, and others was started as soon as the diagnosis was made in the majority of the cases. However, PML is a rapidly fatal illness and hence, survival was only seen in 4 cases in our study. The objective of this article is to highlight the importance of early diagnosis of PML with CSF findings and neuroimaging, early reversion of immunosuppressive medications, and careful monitoring and treatment of HIV cases with goals to reduce mortality, long-term morbidity, and deficits.

摘要

进行性多灶性白质脑病(PML)是一种日益常见且迅速致命的中枢神经系统脱髓鞘感染,由广泛存在的人类JC 病毒(JC 病毒)引起,易发生于所有年龄组和性别的免疫功能低下者。在其他免疫缺陷状态下,人类免疫缺陷病毒(HIV)是最常见的诱发因素,导致 JC 病毒重新激活和多种神经症状。其磁共振成像(MRI)表现多样,通过脑脊液(CSF)中 JC 病毒阳性来确诊。我们报告了来自单一学术中心的 12 例确诊 PML 病例。我们全面描述了这些罕见疾病患者的临床表现、危险因素、CSF 和神经影像学表现、治疗和预后。这些病例在年轻和老年组以及男女之间几乎平均分布。大多数病例的 CSF 中存在 JC 病毒阳性,伴有淋巴细胞计数轻度至重度减少。所有病例均存在明显的 MRI 改变,范围从 T2 高信号到各部位的白质病变。一旦大多数病例确诊,即开始使用免疫调节剂逆转治疗、优化抗病毒治疗(ART)、血浆置换(PLEX)、静脉注射免疫球蛋白(IVIG)、米氮平、口服类固醇和其他药物进行治疗。然而,PML 是一种迅速致命的疾病,因此,我们的研究中仅 4 例患者存活。本文的目的是强调通过 CSF 发现和神经影像学早期诊断 PML、早期逆转免疫抑制药物以及仔细监测和治疗 HIV 病例以降低死亡率、长期发病率和缺陷的重要性。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验