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与人类免疫缺陷病毒感染相关的进行性多灶性白质脑病。文献综述及16例报告

Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. A review of the literature with a report of sixteen cases.

作者信息

Berger J R, Kaszovitz B, Post M J, Dickinson G

出版信息

Ann Intern Med. 1987 Jul;107(1):78-87. doi: 10.7326/0003-4819-107-1-78.

Abstract

Progressive multifocal leukoencephalopathy, a common complication of infections with human immunodeficiency virus (HIV), occurs in as many as 3.8% of patients with the acquired immunodeficiency syndrome (AIDS). We report 16 cases and review 12 previously reported cases of progressive multifocal leukoencephalopathy associated with HIV infection. This illness was the presenting manifestation of HIV infection in 8 cases. Limb weakness, gait abnormalities, visual loss, aand altered mental status were the commonest initial complaints. Computed tomography of the brain frequently showed hypodense, nonenhancing white matter lesions. Magnetic resonance imaging was more sensitive than computed tomography in detecting lesions. Cerebrospinal fluid analysis and electroencephalography were nondiagnostic. Impaired cell-mediated immunity was typically noted, even in the absence of other immunodeficiency-associated illnesses. Death occurred within 10 days to 18 months of the onset of symptoms in 22 patients. However, 4 patients remain alive at 3 to 23 months; of these 4, 2 have had significant improvement without treatment. Various therapies were unsuccessful.

摘要

进行性多灶性白质脑病是人类免疫缺陷病毒(HIV)感染的常见并发症,在获得性免疫缺陷综合征(AIDS)患者中发生率高达3.8%。我们报告16例,并回顾之前报道的12例与HIV感染相关的进行性多灶性白质脑病病例。8例患者以此病作为HIV感染的首发表现。肢体无力、步态异常、视力丧失和精神状态改变是最常见的初始症状。脑部计算机断层扫描常显示低密度、无强化的白质病变。磁共振成像在检测病变方面比计算机断层扫描更敏感。脑脊液分析和脑电图检查无诊断意义。即使在没有其他免疫缺陷相关疾病的情况下,也通常会发现细胞介导免疫受损。22例患者在症状出现后10天至18个月内死亡。然而,4例患者在3至23个月时仍存活;其中4例中有2例未经治疗病情有显著改善。各种治疗均未成功。

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