Berger J R, Pall L, Lanska D, Whiteman M
Department of Neurology, University of Kentucky College of Medicine, Lexington, USA.
J Neurovirol. 1998 Feb;4(1):59-68. doi: 10.3109/13550289809113482.
Progressive multifocal leukoencephalopathy (PML), a formerly rare disease, is estimated to occur in up to 5% of all patients with AIDS. The high prevalence of PML in AIDS patients currently enables a comprehensive evaluation of this disorder. We evaluated the clinical and radiographic features of PML in a large cohort of AIDS patients identified by retrospective chart review from 1981 to 1994. Two hundred and five patients were diagnosed with PML of which 154 met the inclusion criteria. Seventy-two (47%) were pathologically confirmed and the remaining 82 (53%) met clinical and radiographic criteria. There was a 12-fold increase in the frequency of PML between 1981-1984 and 1991-1994. PML affected 136 men and 18 women with AIDS. Eighty-four percent of cases were 20-50 years old (range 5 to 68 years). The most common AIDS risk factors were homosexuality (57%) among men and heterosexual transmission (28%) and intravenous drug abuse (28%) among women. In 27% of patients, PML heralded AIDS. Common manifestations included weakness, gait abnormalities, speech disturbance, cognitive disorders, headache, and visual impairment. The CD4 lymphocyte counts exceeded 200 cells in 11% at the time of presentation. Involvement of posterior fossa structures was evident in 48% of cranial magnetic resonance imaging (MRI) studies, but in only 11% of computed tomographies (CT) of the brain. Contrast enhancement, typically faint and peripheral, was seen in 10% of CT scans and 15% of MRIs. The median survival was 6 months and survival exceeded 1 year in 9%. PML is no longer a rare disease. It often heralds AIDS and may occur in the absence of significant decline in CD4 lymphocytes. Survival is generally poor, although prolonged survival beyond 1 year is not unusual.
进行性多灶性白质脑病（PML），一种曾属罕见的疾病，据估计在所有艾滋病患者中发病率高达5%。目前艾滋病患者中PML的高发病率使得对该疾病能够进行全面评估。我们通过回顾性病历审查，对1981年至1994年间确诊的一大群艾滋病患者中PML的临床和影像学特征进行了评估。205例患者被诊断为PML，其中154例符合纳入标准。72例（47%）经病理证实，其余82例（53%）符合临床和影像学标准。1981 - 1984年至1991 - 1994年间，PML的发病率增加了12倍。PML累及136名男性和18名女性艾滋病患者。84%的病例年龄在20 - 50岁之间（范围为5至68岁）。男性中最常见的艾滋病危险因素是同性恋（57%），女性中是异性传播（28%）和静脉注射吸毒（28%）。27%的患者中，PML是艾滋病的先兆。常见表现包括虚弱、步态异常、言语障碍、认知障碍、头痛和视力损害。就诊时11%的患者CD4淋巴细胞计数超过200个细胞。48%的头颅磁共振成像（MRI）研究显示后颅窝结构受累，但脑部计算机断层扫描（CT）中仅11%有此表现。10%的CT扫描和15%的MRI可见对比增强，通常较淡且位于周边。中位生存期为6个月，9%的患者生存期超过1年。PML不再是一种罕见疾病。它常是艾滋病的先兆，可能在CD4淋巴细胞无显著下降时发生。总体生存率较差，不过生存期超过1年也并不罕见。
