肥厚性硬脑膜炎17例首诊误诊及临床分析  

Analysis of misdiagnosis in 17 patients with hypertrophic cranial pachymeningitis

作　　者：林小锋[1] 周衡 宋田[2] 张星虎[2] LIN Xiaofeng;ZHOU Heng;SONG Tian;ZHANG Xinghu(不详;Department of Neuroinfection&Neuroimmunology,Neurology Center,Beijing Tiantan Hospital,Capital Medical University,Beijing100050,China)

机构地区：[1]广州中医药大学顺德医院,528300 [2]首都医科大学附属北京天坛医院神经病学中心神经感染与免疫科,100050

出　　处：《中国神经免疫学和神经病学杂志》2020年第4期276-280,共5页Chinese Journal of Neuroimmunology and Neurology

基　　金：首都医科大学校培育基金(PYZ2018076);佛山市卫生和计生局医学科研课题(20190336)。

摘　　要：目的探讨肥厚性硬脑膜炎(HCP)的临床表现、辅助检查、误诊情况、治疗及预后等特点,以提高对本病的认识,减少误诊。方法回顾性分析2016年4月至2018年4月首都医科大学附属北京天坛医院神经感染与免疫科收治临床确诊的17例HCP患者的病历资料。结果17例HCP患者中,慢性起病14例,亚急性起病3例,病程18 d至8年,中位病程4个月,主要临床表现为反复头痛(16例)和脑神经麻痹(14例)。头颅磁共振成像(MRI)均可见到硬脑膜局限性或弥漫性肥厚,其中小脑幕(8例)、大脑镰(4例)及额(5例)、颞(5例)、顶枕(4例)硬脑膜局限性或弥漫性肥厚,MRI增强扫描均可见强化。首诊诊断误诊为感音神经性耳聋及颅内感染各3例,误诊为痛性眼肌麻痹及颅内占位各2例,误诊为紧张性头痛、躯体化障碍、中耳炎、急性播散性脑脊髓膜炎、颈椎病、视神经炎和颅内静脉窦血栓形成各1例。17例患者中11例患者经糖皮质激素治疗后症状均明显好转,6例患者在停药时有加重,再次行糖皮质激素冲击并联合免疫抑制剂治疗后症状缓解,随访6~24个月未再复发。结论HCP症状无特异性,易出现误诊、漏诊。头部MRI增强扫描是明确诊断、避免误诊的关键检查。Objective To explore the clinical manifestations,auxiliary examination,misdiagnosis,treatment and prognosis of hypertrophic cranial pachymeningitis(HCP),so as to improve the understanding of the disease and reduce misdiagnosis.Methods The clinical data of 17 inpatients with HCP in Beijing Tiantan Hospital from April 2016 to April 2018 were analyzed retrospectively.Results 14 patients had a chronic onset,and 3 patients had a subacute onset.Median disease duration of all the cases was 4 months.The main clinical manifestations were chronic headaches(16/17)and cranial nerves paralysis(14/17).The MRI demonstrated local or diffused thickened dura located intentorium(8/17),falxcerebri(4/17),frontallobe(5/17),temporallobe(5/17)and parieto/occipital lobe(4/17),which showed marked enhancement after contrast administration.3 cases were misdiagnosed as sensorineuarl deafness,3 cases were misdiagnosed as intracranial infection,2 cases were misdiagnosed as painful ophthalmoplegia,2 cases were misdiagnosed as intracranial space occupying lesions,1 case each was misdiagnosed as tension-type headache,somatization disorder,otitis media,acute disseminated cerebrospinal meningitis,cervical spondylosis,optic neuritis and intracranial venous sinus thrombosis.Most patients showed significant improvement after corticosteroids therapy,but symptoms recurred or progressed in six patients.After repeating steroids therapy and combined with immunosuppressant,clinical symptoms were improved.Conclusions The clinical manifestation of HCP Varies,it is easily misdiagnosed.Enhanced MRI should be performed to clarify diagnosis and avoid misdiagnosis.

关 键 词：肥厚性硬脑膜炎 误诊 临床特点 治疗 

分 类 号：R742.89[医药卫生—神经病学与精神病学]