Ikeda Ken, Takazawa Takanori, Ito Hirono, Ishikawa Yuichi, Miura Ken, Yoshii Yasuhiro, Kawabe Kiyokazu, Iwasaki Yasuo
Department of Neurology, Toho University Omori Medical Center, Tokyo, Japan.
Intern Med. 2010;49(17):1911-6. doi: 10.2169/internalmedicine.49.3492. Epub 2010 Sep 1.
A 56-year-old man with rheumatoid arthritis developed emotional lability and myoclonic seizure in the left arm, followed by fever and generalized convulsion. Brain magnetic resonance imaging (MRI) revealed leptomeningeal lesions with abnormal enhancement. MRI lesions were localized predominantly in the right cerebral subarachnoid spaces. Electroencephalogram showed epileptogenic focus at the right frontal and central points. After administration of valproate sodium improved convulsion and myoclonus, single photon emission computed tomography (SPECT) using N-isopropyl-p-(123)I-iodoamphetamine was performed. Brain SPECT displayed hypoperfusion predominantly in the right cerebral hemisphere. Cerebrospinal fluid (CSF) disclosed mild pleocytosis and marked elevations of interleukin-6 levels. Repeated CSF analyses showed cytology of class I and negative results for infectious pathogens. Methylprednisolone pulse therapy (1 g for 3 days, iv) and subsequent prednisolone administration (daily 50 mg, po) ameliorated neurological symptoms dramatically. Prednisolone was tapered to 20 mg/day for 5 months. Leptomeningeal MRI lesions were attenuated gradually followed by restoration of cerebral hypoperfusion on SPECT. He was diagnosed as rheumatoid leptomeningitis (RLM). Although clinical features of RLM exhibited variable deficits of the central nervous system (CNS), MRI failed to detect the corresponding CNS lesions. We first highlighted neuroradiological changes of cerebral hypoperfusion and leptomeningeal lesions in RLM. These neuroimages of our patient supported that leptomeningeal inflammation and the adjacent cerebrocortical ischemia could cause encephalitis-like symptoms in RLM patients.
一名56岁的类风湿性关节炎男性患者出现情绪不稳定和左臂肌阵挛性发作，随后出现发热和全身性惊厥。脑磁共振成像（MRI）显示软脑膜病变伴异常强化。MRI病变主要位于右侧脑蛛网膜下腔。脑电图显示右侧额叶和中央点有癫痫病灶。给予丙戊酸钠后惊厥和肌阵挛有所改善，随后进行了使用N-异丙基-p-（123）I-碘安非他明的单光子发射计算机断层扫描（SPECT）。脑SPECT显示主要在右侧大脑半球灌注不足。脑脊液（CSF）显示轻度细胞增多和白细胞介素-6水平显著升高。重复的脑脊液分析显示为I类细胞学且感染病原体检测结果为阴性。甲基强的松龙脉冲疗法（静脉注射1 g，共3天）及随后给予强的松龙（口服，每日50 mg）使神经症状显著改善。强的松龙逐渐减量至每日20 mg，持续5个月。软脑膜MRI病变逐渐减轻，随后SPECT显示脑灌注不足恢复。他被诊断为类风湿性软脑膜炎（RLM）。尽管RLM的临床特征表现为中枢神经系统（CNS）的各种缺陷，但MRI未能检测到相应的CNS病变。我们首次强调了RLM中脑灌注不足和软脑膜病变的神经放射学变化。我们患者的这些神经影像支持软脑膜炎症和相邻脑皮质缺血可导致RLM患者出现脑炎样症状。
