Lipton R B, Krupp L, Horoupian D, Hershkovitz S, Arezzo J C, Kurtzberg D
Department of Neurology, Albert Einstein College of Medicine, Bronx, N.Y.
Eur Neurol. 1988;28(5):258-61. doi: 10.1159/000116280.
A 39-year-old intravenous drug user presented with dysarthria and a syndrome of the left cerebellar hemisphere. While in hospital, he developed progressive brainstem findings. Repeated CT scans revealed a lucency in the white matter of the left cerebellar hemisphere. Brainstem auditory and short-latency somatosensory evoked potentials provided evidence of brainstem dysfunction without corresponding lesions on CT. Biopsy of the cerebellum established the diagnosis of progressive multifocal leukoencephalopathy (PML) and the acquired immunodeficiency syndrome (AIDS). Postmortem examination revealed brainstem lesions appropriate to the evoked potential findings and a radiographically inapparent lesion in the right internal capsule. Based on this case and a review of the literature we conclude that: (1) PML occurs with significant prevalence in AIDS patients and may involve the posterior fossa; (2) the diagnosis of posterior fossa PML is suggested by certain clinical and radiographic criteria and may be confirmed by brain biopsy; (3) evoked potentials may be abnormal in PML and can reveal functional abnormalities of white matter without apparent CT abnormalities.
一名39岁的静脉吸毒者出现构音障碍和左侧小脑半球综合征。住院期间，他出现了进行性脑干病变。多次CT扫描显示左侧小脑半球白质有透亮区。脑干听觉和短潜伏期体感诱发电位提供了脑干功能障碍的证据，但CT上无相应病变。小脑活检确诊为进行性多灶性白质脑病（PML）和获得性免疫缺陷综合征（AIDS）。尸检发现脑干病变与诱发电位结果相符，右侧内囊有一处影像学上不明显的病变。基于此病例并回顾文献，我们得出以下结论：（1）PML在艾滋病患者中发病率较高，可能累及后颅窝；（2）某些临床和影像学标准提示后颅窝PML的诊断，可通过脑活检确诊；（3）PML患者的诱发电位可能异常，可揭示无明显CT异常的白质功能异常。
