Schlitt M, Morawetz R B, Bonnin J, Chandra-Sekar B, Curtiss J J, Diethelm A G, Whelchel J D, Whitley R J
Neurosurgery. 1986 Apr;18(4):407-14. doi: 10.1227/00006123-198604000-00003.
Since 1980, three immunocompromised patients have been proved to have progressive multifocal leukoencephalopathy (PML) by brain biopsy at the University of Alabama at Birmingham. Two patients presented with focal neurological findings, and the third presented with dementia. Computed tomography (CT) revealed white matter low density lesions in areas appropriate to the neurological abnormalities. Brain biopsy of areas that were abnormal on CT produced diagnostic tissue in all three patients. No patient suffered ill effects from the biopsy. Neuropathological findings on light microscopy were compatible with PML in each case, although there was diversity within the group. Involvement of gray and white matter was present in all biopsy specimens; oligodendrocytes, astrocytes, and neurons were affected. Electron microscopic demonstration of particles compatible with polyoma virus confirmed the diagnosis in each case. Immunosuppressive medication was discontinued in two of the patients; these two have survived more than 2 years after diagnosis. One of these two has gradually improved and is independent in simple activities of daily life. Brain stem and cerebellar involvement and seizure disorders have been present in all reported cases. PML can be accurately and rapidly diagnosed by brain biopsy, enabling therapeutic manipulations that may prolong survival.
自1980年以来，阿拉巴马大学伯明翰分校通过脑活检证实有3例免疫功能低下患者患有进行性多灶性白质脑病（PML）。2例患者出现局灶性神经系统症状，第3例表现为痴呆。计算机断层扫描（CT）显示，在与神经功能异常相应的区域有白质低密度病变。对CT上显示异常的区域进行脑活检，3例患者均获得了诊断性组织。没有患者因活检出现不良后果。尽管该组病例存在差异，但每例患者光镜下的神经病理学表现均符合PML。所有活检标本均有灰质和白质受累；少突胶质细胞、星形胶质细胞和神经元均受到影响。电镜显示与多瘤病毒相符的颗粒，证实了每例病例的诊断。2例患者停用了免疫抑制药物；这2例患者在诊断后存活了2年多。其中1例已逐渐好转，能独立进行简单的日常生活活动。所有报告的病例均有脑干和小脑受累以及癫痫发作。通过脑活检可以准确、快速地诊断PML，从而进行可能延长生存期的治疗操作。
