Krupp L B, Lipton R B, Swerdlow M L, Leeds N E, Llena J
Ann Neurol. 1985 Apr;17(4):344-9. doi: 10.1002/ana.410170407.
Between April 1982 and March 1984 7 pathologically confirmed cases of progressive multifocal leukoencephalopathy (PML) were diagnosed at our institution. Only 1 case had been seen in the preceding twenty years. Four patients had acquired immunodeficiency syndrome (AIDS). The others had chronic lymphocytic leukemia, Hodgkin's lymphoma, and systemic lupus erythematosus. All patients presented with progressive neurological deficits. In most, the initial computed tomographic (CT) scan was disproportionately less abnormal than the clinical findings. In 5 patients the first CT scan revealed hypodensities of the cerebral white matter which lacked mass effect and did not enhance with contrast agent. The lesions were observed to enlarge progressively on CT scans but often lagged behind the rate of clinical evolution. Although 3 patients were treated with cytosine arabinoside, none improved. PML had similar clinical, radiographic, and pathological features in the AIDS and non-AIDs patients. Of 79 AIDS patients cared for at our institution between December 1979 and December 1983, 3.8% had PML. PML should be suspected in AIDS patients in the presence of the characteristic CT features, especially when CT-clinical dissociation occurs.
1982年4月至1984年3月期间，我院确诊了7例经病理证实的进行性多灶性白质脑病（PML）病例。在过去二十年中仅见过1例。4例患者患有获得性免疫缺陷综合征（AIDS）。其他患者患有慢性淋巴细胞白血病、霍奇金淋巴瘤和系统性红斑狼疮。所有患者均出现进行性神经功能缺损。大多数情况下，最初的计算机断层扫描（CT）显示的异常程度与临床表现相比不成比例地较轻。5例患者的首次CT扫描显示脑白质低密度，无占位效应，且不被造影剂强化。在CT扫描上观察到病变逐渐增大，但通常落后于临床进展速度。尽管3例患者接受了阿糖胞苷治疗，但均无改善。AIDS患者和非AIDS患者的PML具有相似的临床、影像学和病理特征。在1979年12月至1983年12月期间我院诊治的79例AIDS患者中，3.8%患有PML。当AIDS患者出现特征性CT表现，尤其是发生CT与临床分离时，应怀疑患有PML。
