Morita Yuko, Shinohara Nobuaki, Kazahari Masashi, Takahashi Wakoh, Shibuya Makoto, Shinohara Yukito
Department of Neurology, Tokai University School of Medicine.
Rinsho Shinkeigaku. 2003 Aug;43(8):477-81.
A 74-year-old woman, noticed sudden onset of quadriparesis when getting up in the morning on July 17th, 1992. At admission, she presented mild weakness of the limbs bilaterally, and deep tendon hyperreflexia, which were improved after about 4 hours. Serum and urinary laboratory examinations revealed no abnormal findings. Cerebrospinal fluid showed increased cells (75/mm3), and protein (58 mg/dl). Computed tomography revealed multiple low density lesions in the frontal, parietal, and occipital cortex. Magnetic resonance imaging showed low signal intensities on T1-weighted images and high signal intensities on T2-weighted images, suggesting tumorous lesions. On the gadolinium-enhanced images, the enhanced lesions were surrounded with low signal intensity. She developed hallucination and disorientation since around the 8th hospital day, and disturbance of consciousness at the 15th day, followed by coma, and she died from respiratory failure on the 17th hospital day. At autopsy, there were multiple small infarctions in the cerebral cortex and dark areas in the meninges, suggesting localized inflammation. On microscopical examination, remarkable proliferation of cells was seen at the small arteries of the leptomeninges and cerebral cortex. Parts of the vessels were replaced by multinucleated giant cells. No similar abnormalities were found in arteries in the other organs, except the central nervous system. The pathological diagnosis of primary angiitis of the central nervous system was made based on the above findings. The enhanced MRI findings were in excellent agreement with the pathological leptomeningeal vasculitis. The surrounding low intensity areas on the enhanced MRI were considered to reveal edematous change and infarction in the parenchymal lesions. We conclude that enhanced MRI is useful to make a diagnosis of CNS vasculitis.
一名74岁女性，于1992年7月17日早晨起床时突然出现四肢瘫痪。入院时，她双侧肢体轻度无力，腱反射亢进，约4小时后症状有所改善。血清和尿液实验室检查未发现异常。脑脊液显示细胞数增加（75/mm³），蛋白质含量升高（58mg/dl）。计算机断层扫描显示额叶、顶叶和枕叶皮质有多个低密度病变。磁共振成像显示T1加权图像上为低信号强度，T2加权图像上为高信号强度，提示肿瘤性病变。在钆增强图像上，增强病变被低信号强度环绕。自住院第8天左右起，她出现幻觉和定向障碍，第15天意识障碍，随后昏迷，于住院第17天死于呼吸衰竭。尸检时，大脑皮质有多个小梗死灶，脑膜有暗区，提示局部炎症。显微镜检查发现，软脑膜和大脑皮质的小动脉处细胞显著增殖。部分血管被多核巨细胞取代。除中枢神经系统外，其他器官的动脉未发现类似异常。根据上述发现，做出了中枢神经系统原发性血管炎的病理诊断。增强MRI表现与病理软脑膜血管炎高度吻合。增强MRI上周围的低强度区域被认为显示了实质病变中的水肿变化和梗死。我们得出结论，增强MRI有助于诊断中枢神经系统血管炎。
