Enting R H, Portegies P, Algra P R, Valk J, Lange J M
Academisch Medisch Centrum, AIDS-afd. en afd. Neurologie, Amsterdam.
Ned Tijdschr Geneeskd. 1992 Mar 14;136(11):521-6.
This study was carried out to determine clinical features, abnormalities on CT scan and MRI, and course in patients with HIV-I-related progressive multifocal leukoencephalopathy (PML). There were 14 patients with a presumptive diagnosis of PML among 500 HIV-I infected patients with neurological complaints, examined between September 1982 and May 1991 in the University Medical Centre in Amsterdam by a neurologist. In these 14 patients clinical features, imaging abnormalities and course of the disease were analysed retrospectively. All patients presented with progressive focal neurological abnormalities. Cerebrospinal fluid analysis revealed aspecific abnormalities in 5/13 patients. CT scanning of the brain showed hypodense areas in the white matter, without mass effect and with contrast enhancing in only one patient. MR Imaging of the brain showed high signal intensity areas in white matter and in gray matter (10/13), without mass effect, and with contrast enhancement in two. Specimens for neuropathological examination were obtained from 7 patients; in all these cases the diagnosis of PML was confirmed. In patients with AIDS a presumptive diagnosis of PML can be based on clinical features, brain imaging abnormalities and course. However neuropathological confirmation remains the gold standard. Usually the course in these patients was steadily progressive. Most patients died within one year.
本研究旨在确定人类免疫缺陷病毒I型（HIV-I）相关的进行性多灶性白质脑病（PML）患者的临床特征、CT扫描和MRI异常以及病程。在1982年9月至1991年5月期间，阿姆斯特丹大学医学中心的一位神经科医生对500例有神经症状的HIV-I感染患者进行了检查，其中有14例患者初步诊断为PML。对这14例患者的临床特征、影像学异常和疾病病程进行了回顾性分析。所有患者均表现为进行性局灶性神经功能异常。脑脊液分析显示13例患者中有5例有非特异性异常。脑部CT扫描显示白质低密度区，无占位效应，仅1例有强化表现。脑部MRI显示白质和灰质有高信号强度区（13例中有10例），无占位效应，2例有强化表现。7例患者获得了神经病理学检查标本；所有这些病例均确诊为PML。对于艾滋病患者，PML的初步诊断可基于临床特征、脑部影像学异常和病程。然而，神经病理学确诊仍然是金标准。通常这些患者的病程呈稳步进展。大多数患者在一年内死亡。
