Gentile S, Sacerdote I, Roccatello D, Giordana M T
Divisione di Neurologia, Ospedale Giovanni Bosco, Torino, Italy.
Ital J Neurol Sci. 1996 Oct;17(5):363-6. doi: 10.1007/BF01999899.
A 45-year-old patient suffering from chronic renal insufficiency developed dermatopolymyositis. Since the patient did not respond to treatment with high doses of prednisone and immunoglobulin, concomitant cyclosporine A was added. Four months later, worsening signs of bilateral pyramidal disorder and an altered state of consciousness appeared. Serial computed tomography (CT) and magnetic resonance imaging (MRI) revealed multiple alterations of the cerebral white matter. Cyclosporine was then discontinued. One month later, exitus occurred. Microscopic examination of the brain showed diffuse tumescence; histological examination revealed perivascular and diffuse lymphomonocyte infiltrations, areas of demyelination, astrocytes with bizarre nuclei, and oligodendrocytes with enlarged nuclei due to hyperchromatic inclusion. Morphological examination confirmed the presence of intranuclear icosahedral viral bodies. Progressive multifocal leukoencephalopathy was diagnosed. The literature contains only one report of an analogous case observed during a course of cyclosporine treatment for Wegener's granulomatosis.
一名45岁患有慢性肾功能不全的患者患上了皮肌炎。由于该患者对大剂量泼尼松和免疫球蛋白治疗无反应，遂加用环孢素A。四个月后，出现双侧锥体束征加重及意识状态改变。系列计算机断层扫描（CT）和磁共振成像（MRI）显示脑白质有多处改变。随后停用环孢素。一个月后，患者死亡。脑部显微镜检查显示弥漫性肿胀；组织学检查发现血管周围及弥漫性淋巴细胞浸润、脱髓鞘区域、核形态怪异的星形胶质细胞以及因核内染色质包涵体而核肿大的少突胶质细胞。形态学检查证实存在核内二十面体病毒体。诊断为进行性多灶性白质脑病。文献中仅有一篇关于在环孢素治疗韦格纳肉芽肿病过程中观察到类似病例的报告。
