Kepes J J, Chou S M, Price L W
Neurology. 1975 Nov;25(11):1006-12. doi: 10.1212/wnl.25.11.1007.
A 46-year-old man with nontropical sprue had anemia and hypoproteinemia for several years, until his condition was diagnosed and treated with dietary measures. Within a year after the diagnosis, progressive multifocal leukoencephalopathy developed, and the patient had a slightly fluctuating chronic downhill course until he died 10 years later. It is postulated that this patient's immune deficiency was related to his malabsorption syndrome and hypoglobulinemia, and the course became unusually protracted (longest reported course in the American literature) because of restoration of plasma protein levels. Autopsy showed the classic findings of progressive multifocal leukoencephalopathy, with much tissue loss of subcortical white matter and active perivascular inflammatory foci with numerous eosinophilic granulocytes. On electron microscopy, oligodendrocyte nuclei and cytoplasm were crowded with virions, but many myelin sheaths invested by severely infected oligodendrocytic processes were remarkably well preserved. This fact would argue against a direct cause-and-effect relationship between infection of oligodendrocytes and myelin breakdown in progressive multifocal leukoencephalopathy. The likelihood of an autoimmune mechanism at work in this disease is suggested, and the role of eosinophils and other cells in such process is considered.
一名46岁的非热带性口炎性腹泻男性患者，数年来一直患有贫血和低蛋白血症，直至其病情被诊断出来并采取饮食措施进行治疗。确诊后一年内，患者患上了进行性多灶性白质脑病，其病情呈慢性、逐渐恶化且略有波动，直至10年后死亡。据推测，该患者的免疫缺陷与他的吸收不良综合征和低球蛋白血症有关，由于血浆蛋白水平的恢复，病程变得异常漫长（在美国文献中报道的最长病程）。尸检显示了进行性多灶性白质脑病的典型表现，皮质下白质有大量组织丢失，血管周围有活跃的炎症病灶，伴有大量嗜酸性粒细胞。电子显微镜检查显示，少突胶质细胞核和细胞质中充满了病毒粒子，但许多被严重感染的少突胶质细胞突起包裹的髓鞘却保存得非常完好。这一事实表明，在进行性多灶性白质脑病中，少突胶质细胞感染与髓鞘破坏之间不存在直接的因果关系。提示了自身免疫机制在该疾病中起作用的可能性，并考虑了嗜酸性粒细胞和其他细胞在这一过程中的作用。
