MPO-ANCA阳性相关性肥厚性硬脑膜炎伴肺动脉广泛血栓形成1例并文献复习  

MPO-ANCA Positive Association Hypertrophic Cranial Pachymeningitis with Extensive Pulmonary Arterial Thrombosis:A Case Report and Literature Review

作　　者：熊泽辉 陈晓露 XIONG Zehui;CHEN Xiaolu(School of Medicine,Jianghan University,Wuhan 430056,Hubei,China;Department of Neurology,Union Hospital Affiliated to Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,Hubei,China)

机构地区：[1]江汉大学医学院,湖北武汉430056 [2]华中科技大学同济医学院附属协和医院神经内科,湖北武汉430022

出　　处：《江汉大学学报（自然科学版）》2021年第5期59-64,共6页Journal of Jianghan University：Natural Science Edition

摘　　要：目的探讨MPO-ANCA阳性相关性肥厚性硬脑膜炎的临床特征及治疗方案。方法报道1例MPO-ANCA阳性的肥厚性硬脑膜炎伴肺动脉血栓的患者,并结合文献进行分析。结果患者就诊时表现为视力下降,伴有头痛,入院后查血D-二聚体7.09 mg/LFEU(<0.5),红细胞沉降率33 mm/h(<15),抗MPO抗体137.3 CU(<20.0),抗蛋白酶3抗体22.7 CU(<20.0),肺纵膈三维CT平扫+增强示双侧肺动脉广泛血栓形成,肺动脉CTA示肺动脉多发栓塞,颅脑磁共振示双侧额颞部脑膜增厚强化,海绵窦、眶尖、咽旁间隙(右侧为著)及右侧岩上/下窦条片状强化影,所及双侧视神经局部显示较纤细(右侧为著),颅底脑膜增厚局部稍明显/疑稍显厚,诊断为MPO-ANCA阳性的肥厚性硬脑膜炎,经免疫、抗凝治疗后症状好转。结论MPO-ANCA阳性相关性肥厚性硬脑膜炎临床表现具有异质性,大多数患者出现慢性头痛,伴或不伴颅脑神经麻痹、小脑共济失调、癫痫发作、脊髓病、视力障碍等神经学表现。在临床中,发现头痛、多颅神经受累,并且伴鼻窦炎、中耳炎患者,需考虑MPO-ANCA阳性的肥厚性硬脑膜炎的可能。Objective To investigate the clinical characteristics and treatment of MPO ANCA positive associated hypertrophic cranial pachymeningitis.Methods A case of MPO ANCA positive hypertrophic cranial pachymeningitis with extensive pulmonary arterial thrombosis was reported and analyzed with the literature.Results The patient presented with impaired vision and headache.After admission,blood D-dimer 7.09 mg/L FEU(<0.5),ESR 33 mm/h(<15),anti-MPO antibody 137.3 CU(<20.0),anti-protease 3 antibody 22.7 CU(<20.0).The three-dimensional CT scan of the mediastinum plus enhancement shows extensive thrombosis of both pulmonary arteries.Pulmonary artery CTA showed multiple pulmonary embolisms.Cerebral MRI showed in bilateral frontal-temporal dura thickening reinforcement,cavernous sinus,orbit,pharynx side clearance(right),and on the right side of the rock/article sinus flake reinforced under the shadow,and double side nerve local display was fine(right),the dura thickening of the skull base slightly obvious local/doubt slightly thick,diagnosis of MPO ANCA positive hypertrophic pachymeningitis,symptoms improved after immunization,anticoagulant therapy.Conclusion The clinical manifestations of MPO-ANCA positive hypertrophic cranial pachymeningitis are heterogeneous.Most patients have chronic headaches,with or without craniocerebral nerve palsy,cerebellar ataxia,epileptic seizure,myelopathy,visual impairment,and other neurological manifestations.In clinical practice,the possibility of MPO-ANCA positive hypertrophic cranial pachymeningitis should be considered in patients with headaches,multiple cranial nerve involvement,and sinusitis and otitis media.

关 键 词：肥厚性硬脑膜炎 MPO-ANCA阳性血管炎 肺动脉血栓 病例报道 

分 类 号：R742[医药卫生—神经病学与精神病学]