以视功能损害为主要表现的髓过氧化物酶中性粒细胞胞质抗体相关性肥厚性硬脑膜炎临床分析  

Clinical analysis of hypertrophic cranial pachymeningitis associated with anti-myeloperoxidase and anti-neutrophil cytoplasmic antibody with optic nerve dysfunction

作　　者：刘欣欣[1] 朱静[1] 王佳伟[1] 景筠[1] Liu Xinxin;Zhu Jing;Wang Jiawei;Jing Yun(Department of Neurology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院神经内科,100730

出　　处：《中华神经科杂志》2020年第8期603-610,共8页Chinese Journal of Neurology

摘　　要：目的探讨以视功能损害为主要表现的抗髓过氧化物酶-抗中性粒细胞胞质抗体(MPO-ANCA)相关性肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)患者的临床特点。方法搜集2008—2019年首都医科大学附属北京同仁医院收治的MPO-ANCA相关性HCP患者的临床资料,对临床特点、诊治方法及预后进行分析。结果共确诊55例HCP患者,其中有5例为MPO-ANCA相关性HCP,其中2例男性,3例女性,发病年龄61~80岁。5例患者的临床表现依次为多脑神经受累5例,头痛4例,鼻窦炎4例,中耳乳突炎3例,轻度肾脏受累2例,脑梗死1例,均无明显下呼吸道损害。病情活动期5例外周血MPO-ANCA浓度、红细胞沉降率均升高,3例类风湿因子升高,蛋白酶3-ANCA均为阴性。3例脑脊液蛋白含量升高,1例白细胞升高。头MRI增强扫描异常的频率依次为硬脑膜5例,视神经鞘膜5例,鼻窦黏膜4例,内听道脑膜3例,眼眶壁骨膜2例和脑实质1例。所有患者均接受糖皮质激素治疗,2例同时接受环磷酰胺治疗,患者头痛及脑神经损害改善,但视功能损害改善不明显。结论MPO-ANCA相关性HCP是HCP的特殊类型,在累及的硬脑膜部位中,视神经鞘膜是该病常见的受累部位,鼻窦黏膜及内听道脑膜也常受累。对临床中发现硬脑膜病变,尤其是伴有视神经鞘膜病变、鼻窦炎、中耳乳突炎者,需鉴别该病。Objective To investigate the clinical characteristics,diagnosis,treatment and prognosis of patients with hypertrophic cranial pachymeningitis(HCP)associated with myeloperoxidase-antineutrophil cytoplasmic antibody(MPO-ANCA)with optic nerve dysfunction.Methods Clinical data of patients with MPO-ANCA associated HCP admitted to Beijing Tongren Hospital,Capital Medical University from 2008 to 2019 were collected and analyzed.Results A total of 55 HCP patients were diagnosed,including five patients with MPO-ANCA associated HCP,two males and three females,with the onset ages ranging from 61 to 80 years.The main clinical manifestations were successively five cases of multiple cranial neuropathies,four cases of headache,four cases of chronic sinusitis,three cases of otitis media or mastoiditis,one case of cerebral infarction,while two cases of mild kidney involvement,all without lower airways involvement.Peripheral blood MPO-ANCA concentration and erythrocyte sedimentation rate were elevated in all patients,and protease 3-ANCA was negative.Cerebrospinal fluid protein content was elevated in three patients and leukocyte was increased in one patient.The abnormal frequency of gadolinium-enhanced MRI of brain scan was dura mater(five cases),optic nerve sheath(five cases),sinus mucosa(four cases),inner auditory tract meninges(three cases),orbital wall periosteum(two cases),and brain parenchyma(one case).All patients received glucocorticoid therapy,meanwhile two patients received cyclophosphamide treatment.Multiple cranial neuropathies and headache were improved,but the improvement of visual function damage was not obvious.Conclusions MPO-ANCA associated HCP is a special type of HCP.Among the dural region involved,the optic nerve sheath is a common site of this disease,and the sinus mucosa and inner auditory canal meninges are also frequently involved.MPO-ANCA associated HCP should be distinguished,if patients with dural lesions,especially those accompanied by optic nerve sheath lesions,sinusitis,and middle ear mastoiditis.

关 键 词：脑膜炎 自身抗体 粒细胞 胞质 血管炎 颅神经损伤 

分 类 号：R742.9[医药卫生—神经病学与精神病学]