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[获得性免疫缺陷综合征相关的进行性多灶性白质脑病]

[Progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome].

作者信息

Clavo Sánchez A, García-Gil D, Sasián Martínez S, Geraldía Lama M, Serrano González A, Bascuñana Quirell A

机构信息

Unidad de Enfermedades Infecciosas, Hospital Universitario Puerta del Mar, Cádiz.

出版信息

Rev Clin Esp. 1994 Jan;194(1):13-6.

PMID:8153407
Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection in HIV-infected persons. We present the characteristics of seven cases of PML, which represent 3.1% of our patients with AIDS. In six cases, was the "marker" disease for AIDS. The most common clinical manifestation was a slowly evolving focal neurological syndrome (average time of pre-hospitalization symptoms was 64 days). The number of T4 lymphocytes at the moment of diagnosis varied between 14 and 121 cel/mm3 (median: 51). Computerized tomography (CT) and cranial nuclear magnetic resonance (NMR) showed images of single (4 cases) or multiple white substance involvement without contrast uptake or mass-effect. The definitive diagnosis was made by cerebral biopsy. Four died within 20 to 90 days of diagnosis. Three of the five who received antiretrovirals survived with apparent stabilization of the process at 2, 13, and 23 months of diagnosis. PML can be an initial diagnostic disease for AIDS. Its symptoms and the CT/NMR should arouse suspicion. However, given that clinicoradiological data are often insufficient, it is advisable to conduct cerebral biopsies. Antiretrovirals may have a favorable effect on diseases course.

摘要

进行性多灶性白质脑病(PML)是人类免疫缺陷病毒(HIV)感染者中一种罕见的机会性感染。我们报告了7例PML患者的特征,占我们艾滋病患者的3.1%。在6例中,PML是艾滋病的“标志性”疾病。最常见的临床表现是一种缓慢进展的局灶性神经综合征(院前症状平均持续时间为64天)。诊断时T4淋巴细胞数量在14至121个细胞/立方毫米之间(中位数:51)。计算机断层扫描(CT)和头颅核磁共振成像(NMR)显示单个(4例)或多个白质受累的图像,无对比剂摄取或占位效应。最终诊断通过脑活检确定。4例在诊断后20至90天内死亡。5例接受抗逆转录病毒治疗的患者中,3例存活,病情在诊断后2个月、13个月和23个月时明显稳定。PML可能是艾滋病的初始诊断疾病。其症状以及CT/NMR表现应引起怀疑。然而,鉴于临床放射学数据往往不足,建议进行脑活检。抗逆转录病毒药物可能对病程有有利影响。

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