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Received:September 28, 2019 Published Online:May 20, 2020
Received:September 28, 2019 Published Online:May 20, 2020
中文摘要: 目的 探讨肥厚性硬脑膜炎(HCP)的临床表现、化验及影像学检查特点。方法 对2014年1月至2018年12月16例明确诊断为HCP患者的临床资料、实验室化验、影像学检查、颅内压及脑脊液(CSF)常规、生化等结果进行回顾性分析,并总结其特点。结果 (1)HCP患者临床表现多样:首发症状表现为头痛14例,视力下降4例,听力减退2例,面瘫1例,复视1例,眼睑下垂1例;在颅神经受损中,视神经8例,听神经6例,外展神经4例,动眼神经5例,滑车神经3例,面神经2例,舌下神经1例和嗅神经1例。(2)实验室检查:外周血检测血沉增快4例,白细胞升高4例,IgG4(+)1例,抗中性粒细胞胞浆抗体(ANCA)(+)合并抗髓过氧化物酶(MPO)抗体(+)患者1例;CSF压力升高6例,CSF蛋白升高13例,免疫球蛋白升高11例。(3)增强颅脑MRI检查:16例HCP患者均有不同程度硬脑膜强化表现,以弥散性强化表现最为常见(68.8%),按强化部位分析多见位于大脑镰、小脑幕、后颅窝区、海绵窦区,局灶性病灶也可见于枕部及额部。(4)治疗和预后:对特发性HCP,均采用糖皮质激素冲击治疗并逐渐减量维持的滴定式治疗;对继发性HCP,采取针对病因的抗感染治疗。治疗后患者神经系统功能恢复明显,复查影像学较前也明显好转。1例出现复发,予加用甲氨蝶呤治疗后病情平稳,6个月内未再复发。结论 HCP是一种临床少见病,临床表现多样,误诊率高,当考虑HCP诊断时,需完善增强MRI检查以明确硬脑膜增厚和强化程度。HCP患者早期治疗可以明显恢复神经功能。
Abstract:ObjectiveTo explore the clinical manifestations,laboratory results and imaging features in patients with hypertrophic cranial pachymeningitis（HCP）.MethodsTo analyze the clinical data,including laboratory test,imaging examination,intracranial pressure (ICP) and cerebrospinal fluid (CSF) and other biochemical changes of 16 patients diagnosed as HCP from January 2014 to December 2018,retrospectively and summarize the clinical manifestations and characteristics.Result(1) In the first symptoms of HCP patients,headache occurred in 14 cases,visual loss in 4 cases,hearing loss in 2 cases,facial paralysis in 1 case,diplopia in 1 case and drooping eyelid in 1 case;in the cranial nerve damages,there were optic nerve in 8 cases,auditory nerve in 6 cases,abductor nerve in 4 cases,oculomotor nerve in 5 cases,trochlear nerve in 3 cases,facial nerve in 2 cases,hypoglossal nerve in 1 case and olfactory nerve in 1 case.(2) ESR and leukocyte were elevated,respectively in 4 cases.There were 1 case of IgG4(+),1 case of anti-neutrophil cytoplasmic antibody (ANCA) (+) combined with anti-myeloperoxidase (MPO) antibody,6 cases of increased CSF pressure,13 cases of increased CSF protein and 11 cases of increased immunoglobulin.(3) Enhanced MRI showed that all patients had different degrees of dura mater enhancement,with the most common diffuse enhancement (68.8%).According to the locations of enhanced MRI,the lesions were mainly located in the falx cerebri,tentorium cerebelli,posterior cranial fossa and cavernous sinus,and focal lesions were also found in occipital and frontal areas.(4) After glucocorticoid shock therapy and gradual reduction and maintenance of titration therapy for idiopathic HCP and anti-infection treatment for secondary HCP,the neurological function recovered significantly,and the imaging was also improved.One case recurred and was stable with no recurrence within 6 months after treatment with methotrexate.ConclusionsHCP is a rare clinical disease with various clinical manifestations and high misdiagnosis rate.In diagnosing HCP,enhanced MRI examination is necessary to determine the degree of dural thickening and enhancement.Early treatment can significantly help to restore neurological function in HCP patients.
keywords: Hypertrophic cranial pachymeningitis,primary,secondary Headache Magnetic resonance imaging Cranial nerve damage Cerebrospinal fluid Glucocorticoid Anti-infection
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基金项目:海南省三亚市医疗科技创新项目(2014YW27);海南省三亚市专项科研试制项目(2015KS16)
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