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对比增强型进行性多灶性白质脑病:影像学与病理学相关性:病例报告

Contrast-enhancing progressive multifocal leukoencephalopathy: radiological and pathological correlations: case report.

作者信息

Woo H H, Rezai A R, Knopp E A, Weiner H L, Miller D C, Kelly P J

机构信息

Department of Neurosurgery, New York University Medical Center, New York, USA.

出版信息

Neurosurgery. 1996 Nov;39(5):1031-4; discussion 1034-5. doi: 10.1097/00006123-199611000-00029.

DOI:10.1097/00006123-199611000-00029
PMID:8905761
Abstract

OBJECTIVE AND IMPORTANCE

Progressive multifocal leukoencephalopathy (PML), a demyelinating disease caused by the JC papovavirus, is an opportunistic infection afflicting patients with impaired cellular immunity. Although initially described in patients with hematological malignancies, PML has become associated with several other immunocompromised states, particularly human immunodeficiency virus (HIV) infection. There are numerous central nervous system manifestations in patients with acquired immunodeficiency syndrome. A major characteristic that distinguishes PML from other more common lesions, such as toxoplasmosis or non-Hodgkin's lymphoma, is the lack of contrast enhancement. We describe a case of PML that exhibits contrast enhancement, and we conclude that the diagnosis of PML must be considered in patients with HIV who have contrast-enhancing lesions.

CLINICAL PRESENTATION

A 40-year-old woman presented with progressive hemiparesis, blurred vision, and ataxia. Magnetic resonance imaging revealed a contrast-enhancing lesion involving the left middle cerebellar peduncle, causing mild compression of the fourth ventricle.

INTERVENTION

The patient underwent a stereotactic serial biopsy with the presumptive diagnosis of moderate- to high-grade glioma. Histological examination of the biopsy specimen revealed early PML. Subsequently, a test for HIV was obtained and the results were positive.

CONCLUSION

We have reported another atypical radiographic characteristic of PML associated with HIV. We conclude that PML lesions can enhance after the administration of gadolinium. Therefore, the diagnosis of PML must be entertained in patients whose test results were positive for HIV with contrast-enhancing lesions and that a stereotactic serial biopsy may be necessary to provide a definitive diagnosis.

摘要

目的及重要性

进行性多灶性白质脑病(PML)是一种由JC多瘤病毒引起的脱髓鞘疾病,是一种侵袭细胞免疫受损患者的机会性感染。尽管最初在血液系统恶性肿瘤患者中被描述,但PML已与其他几种免疫功能低下状态相关,尤其是人类免疫缺陷病毒(HIV)感染。获得性免疫缺陷综合征患者有许多中枢神经系统表现。PML与其他更常见病变(如弓形虫病或非霍奇金淋巴瘤)相区别的一个主要特征是缺乏对比增强。我们描述了一例表现出对比增强的PML病例,并得出结论,对于有对比增强病变的HIV患者必须考虑PML的诊断。

临床表现

一名40岁女性出现进行性偏瘫、视力模糊和共济失调。磁共振成像显示一个对比增强病变累及左小脑中间脚,导致第四脑室轻度受压。

干预措施

患者接受了立体定向系列活检,初步诊断为中高级别胶质瘤。活检标本的组织学检查显示为早期PML。随后进行了HIV检测,结果呈阳性。

结论

我们报告了另一例与HIV相关的PML的非典型影像学特征。我们得出结论,PML病变在给予钆后可出现强化。因此,对于HIV检测结果呈阳性且有对比增强病变的患者必须考虑PML的诊断,并且可能需要进行立体定向系列活检以提供明确诊断。

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The effect of highly active antiretroviral therapy-induced immune reconstitution on development and outcome of progressive multifocal leukoencephalopathy: study of 43 cases with review of the literature.
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