Hayashi Seiya, Sugeno Naoto, Nishiyama Syuhei, Hasegawa Takafumi, Aoki Masashi
Department of Neurology, Tohoku University School of Medicine.
Rinsho Shinkeigaku. 2012;52(3):152-5. doi: 10.5692/clinicalneurol.52.152.
We report a 79-year-old man presenting MPO-ANCA associated hypertrophic pachymeningitis and bilateral visual impairment. Two years before, microscopic hematuria and positive MPO-ANCA were indicated, then oral steroids and cyclophosphamide were given as systemic vasculitis. On admission, lateral hemianopsia in the right visual field was documented. Some weeks after admission, he complained of a left-hand side headache, and the visual impairment of a right eye. Brain MRI detected thick dura matter with abnormal enhancement predominantly on the left side of the basal temporal lobe and a tumor-like lesion at the sphenoid sinus near the right cavernous sinus. Multiple scotomas in the left visual field were compatible with ischemic changes caused by MPO-ANCA related vasculitis. On the other hand, the hemianopsia in his right eye was related with a tumor-like lesion. The visual problems showed a favorable response to the steroid pulse therapy. ANCA-positive cases can demonstrate various symptoms including intra-/extra-cranial involvement. Thus, thorough clinical workup is needed to determine the actual site of the lesion when cranial nerve involvement is observed in MPO-ANCA positive hypertrophic pachymeningitis.
我们报告了一名79岁男性，患有髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）相关的肥厚性硬脑膜炎和双侧视力障碍。两年前，发现镜下血尿和MPO-ANCA阳性，随后给予口服类固醇和环磷酰胺治疗系统性血管炎。入院时，记录到右侧视野出现同向性偏盲。入院几周后，他主诉左侧头痛和右眼视力障碍。脑部磁共振成像（MRI）检测到硬脑膜增厚，主要在颞叶底部左侧有异常强化，右侧海绵窦附近蝶窦处有一个肿瘤样病变。左侧视野的多个暗点与MPO-ANCA相关血管炎引起的缺血性改变相符。另一方面，他右眼的偏盲与肿瘤样病变有关。视力问题对类固醇冲击疗法反应良好。ANCA阳性病例可表现出各种症状，包括颅内/颅外受累。因此，当在MPO-ANCA阳性肥厚性硬脑膜炎中观察到颅神经受累时，需要进行全面的临床检查以确定病变的实际部位。
