以眼科表现为首发症状的特发性肥厚性硬脑膜炎八例

Eight cases of idiopathic hypertrophic meningitis with ophthalmic manifestations as the first symptom

目的:观察分析以眼科表现为首发症状的特发性肥厚性硬脑膜炎(IHP)患者的临床表现、实验室和影像学检查、治疗及预后特征。方法:回顾性病例分析。2016年1月至2019年4月在解放军总医院第一医学中心神经眼科住院治疗的8例IHP患者纳入研究。其中,男性6例,女性2例;年龄11~65岁,平均年龄(48.00±19.08)岁。病程30 d^7.5年,平均病程(17.00±30.08)个月。记录患者发病年龄、症状、体征。所有患者均行眼部检查、颅脑或眼眶MRI或CT检查,血常规、生化、肿瘤标记物、免疫学指标、乙肝、梅毒、人类免疫缺陷病毒、甲状腺功能等实验室检查,同时行腰椎穿刺测颅压并检测脑脊液(CSF)相关指标。总结患者临床表现、颅脑或眼眶MRI影像学和实验室检查特征。同时观察患者治疗和预后情况。结果:8例患者中,视力下降6例,视力下降伴复视1例,复视1例。双眼受累7例,单眼受累1例。其他症状包括头痛、听力下降等。眼底检查可见视盘水肿1只眼,视盘苍白6只眼。实验室检查结果显示,血管紧张素转化酶异常4例,抗甲状腺过氧化物酶抗体异常3例,免疫抗体阳性3例。CSF测量结果显示,所有患者均可见蛋白升高。颅脑和(或)眼眶MRI及CT检查发现,视神经受累6例,动眼神经受累1例,累及海绵窦区2例。所有患者均行糖皮质激素治疗,4例患者视力明显提高,2例患者复视完全缓解;7例患者联合疾病修饰治疗(DMT)预防炎症复发。所有患者经平均(26.63±16.55)个月的随访观察未见复发。结论:IHP可因双眼先后或同时视力下降就诊于眼科。IHP患者易于合并头痛和其他颅神经麻痹症状。明确IHP诊断需要依赖影像学检查。糖皮质激素早期治疗有效,但因IHP易进展及复发,应尽早联合DMT。

Objective To investigate the clinical,laboratory and imaging evaluation,treatment and prognosis of patients with idiopathic hypertrophic pachymeningitis(IHP)with ophthalmic manifestations as the first symptom.Methods A retrospective case analysis.Eight patients displaying symptoms of IHP were recruited from the Neuro-ophthalmology Department in the First Medical Center of Chinese PLA General Hospital from January 2016 to April 2019 were inculed in this study.There were 6 males and 2 females,aged from 11 to 65 years,with an average age of 48.00±19.08 years.The course of disease ranged from 30 days to 7.5 years,with an average course of 17.00±30.08 months.The age,symptoms and signs of all patients were recorded.All patients underwent ophthalmic examination,orbit or brain MRI or CT examinations,blood routine examination,biochemistry,tumor markers,immunity,hepatitis B,syphilis,HIV,thyroid function and other laboratory tests,and lumbar puncture was performed to measure the cerebrospinal fluid(CSF)pressure and indicators.The clinical manifestations,orbital or brain MRI imaging and laboratory examination characteristics were summarized.Treatment and prognosis were also observed.Results In total of 8 patients,visual loss was presented in 6 patients,visual loss and diplopia were presented in 1 patient,and diplopia was presented in 1patient.Binocular involvement in 7 patients and monocular involvement in 1 patient.Other symptoms including headache and hear loss and so on.Optic disc edema in 1 eye and optic disc pallor in 6 eyes were reviewed by fundus examination.The laboratory examination showed that the angiotensin converting enzyme abnormal in 4 patients,the anti-thyroid peroxidase antibody abnormal in 3 patients and immunoantibodies positive in 3 patients.CSF measurements showed that the protein level elevated in all patients.Orbit and/or brain MRI and CT examination showed that optic nerve involvement in 6 patients,oculomotor nerve involvement in 1 patient,and cavernous sinus region involvement in 2 patients.Glucocorticoid was effective in all patients,and the visual acuity significantly improved in 4 patients,the diplopia was completely resolved in 2 patients,and the disease modifying therapy(DMT)was combined to prevent recurrence in 7 patients.No recurrence was observed in an average follow-up time of 26.63±16.55 months.Conclusions IHP patients may be first visit an ophthalmologist due to vision loss in bilateral eyes simultaneous or sequentially.IHP patients are often associated with headache and other cranial nerve paralysis symptoms.Definitive diagnosis of IHP depends on imaging examination.Glucocorticoid treatment is effective in early phase,but it is tendency to progress and relapse,suggesting combined with DMT as early as possible.