肥厚性硬脑膜炎病例1例分析并文献复习
Neural Injury and Functional Reconstruction(2018)
Abstract
目的:探讨肥厚性硬脑膜炎(HCP)的临床特征、影像学表现.方法:报道1例HCP患者的临床资料并结合文献进行回顾性分析.结果:该患者以头痛起病,伴视力下降;血液学检验:ANA(+);腰椎穿刺示:脑脊液压力≥330mmH2O;头颅MRI平扫+增强示:大脑镰、小脑幕、双侧额顶叶硬脑膜增厚伴强化;硬脑膜活检病理示:玻璃样变纤维血管组织中散在淋巴细胞、浆细胞,以血管周围较明显;免疫组化:CD138(散在+), IgG(个别细胞+),IgG4(个别细胞+);予以激素治疗,症状明显好转.搜索近5年报道的HCP患者189例,最常见的首发症状为头痛,所有患者头颅磁共振平扫+增强均见不同部位的硬脑膜肥厚伴强化,予以激素、激素联合免疫抑制剂或手术治疗后,90%以上患者症状改善.讨论:HCP临床症状可表现为慢性头痛、颅神经受累、共济失调.需进一步完善血液学检验、头颅磁共振平扫+增强检查,必要时硬脑膜活检以确诊.
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Key words
hypertrophic cranial pachymeningitis,headache,cranial nerve involvement,magnetic resonance imaging,pathology
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