Aguilar-Amat Maria Jose, Abenza-Abildúa Maria Jose, Vivancos Francisco, Rodríguez de Rivera Francisco Javier, Morales-Bastos Carmen, Gandía-Gonzalez Maria Luisa, Pérez-Zamarrón Alvaro, Arpa Javier
Department of Neurology, La Paz University Hospital, Madrid, Spain.
Neurologist. 2011 May;17(3):136-40. doi: 10.1097/NRL.0b013e31821735ad.
Rheumatoid meningitis is an uncommon manifestation of longstanding rheumatoid arthritis and few cases have been described. The clinical presentation is extremely variable as reported in medical literature.
We report a 71-year-old woman with 15 years of seropositive rheumatoid arthritis who developed neurological complications: cognitive deterioration; hypomimia; limitation on vertical gaze; and axial stiffness, resembling progressive supranuclear palsy and seizures. Brain magnetic resonance imaging showed a diffuse dural plaque on both frontal and temporal lobes exhibiting homogeneous gadolinium enhancement. There was diffuse leptomeningeal enhancement and hyperintense white matter lesions. The final diagnosis made by image-guided biopsy showed rheumatoid pachymeningitis. After the definitive diagnosis, high doses of corticosteroids and immunosuppressive treatment were started.
We emphasize the diagnostic importance of the biopsy in cases of chronic pachymeningitis and stress that diverse entities can cause progressive supranuclear palsy-like phenotypes.
类风湿性脑膜炎是长期类风湿性关节炎的一种罕见表现，仅有少数病例被描述。正如医学文献所报道的，其临床表现极具多样性。
我们报告一例71岁女性，患有15年血清阳性类风湿性关节炎，出现了神经并发症：认知功能减退；表情减少；垂直凝视受限；以及轴向僵硬，类似于进行性核上性麻痹和癫痫发作。脑部磁共振成像显示额叶和颞叶均有弥漫性硬脑膜斑块，呈均匀钆增强。存在弥漫性软脑膜强化和高信号白质病变。影像引导活检做出的最终诊断为类风湿性硬脑膜炎。确诊后，开始使用高剂量皮质类固醇和免疫抑制治疗。
我们强调活检在慢性硬脑膜炎病例诊断中的重要性，并强调多种疾病实体可导致类似进行性核上性麻痹的表型。
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