摘要
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≥330 mmH2O;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
Abstract
To analyze the clinical and imaging features of hypertrophic cranial pachymeningitis
(HCP) . Methods: One case of HCP was reported, and the relevant literature was reviewed and retrospectively
analyzed. Results: The patient experienced headache at the onset of illness along with decreased visual acuity.
Hematology test: ANA (+ ). Lumbar puncture: cerebrospinal fluid pressure ≥330 mmH2O. Cerebral MRI, plain
and enhanced: cerebral falx and tentorium of cerebellum, bilateral frontal parietal dura thickening and
enhancement. Dural biopsy: fibrous hyaline tissue scattered around lymphocytes and plasma cells, most apparent
surrounding blood vessels. Immunohistochemistry: CD138 (+ ), IgG (individual cells + ), IgG4 (individual cells
+ ). Hormone therapy significantly improved symptoms. We collected data of 189 cases of HCP reported in the
last 5 years. Headache is the most presented initial symptom. Dural hypertrophy and enhancement in different
parts of the head was observed in all patients by plain and enhanced cranial MRI. After hormone treatment,
combined hormone and immunosuppressive treatment, or surgical treatment, more than 90% of the patients'
symptoms were improved. Conclusion: The clinical symptoms of HCP can be characterized by chronic
headache, cranial nerve involvement, and ataxia. It is imperative to perform hematological examination, plain
and enhanced cranial magnetic resonance imaging, and, when necessary, dura mater biopsy to make a diagnosis.
关键词
肥厚性硬脑膜炎 /
头痛 /
颅神经受累 /
磁共振成像 /
病理
Key words
hypertrophic cranial pachymeningitis
章维
;陈浩
;崔桂云
;赵小媛
;张作慧
;张伟
;董丽果
;鲍磊
;刘永海.
肥厚性硬脑膜炎病例1例分析并文献复习[J]. 神经损伤与功能重建. 2018, 13(5): 234-236
Analysis of Hypertrophic Cranial Pachymeningitis:1 Case Report and Literature Review[J]. Neural Injury and Functional Reconstruction. 2018, 13(5): 234-236
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