视神经脊髓炎谱系疾病临床特征分析及随访

发布时间：2018-05-26 14:09

本文选题：视神经脊髓炎谱系疾病 + 随访　；参考：《广西医科大学》2017年硕士论文

【摘要】：目的本研究对39例视神经脊髓炎谱系疾病(Neuromyelitis optica spectrum disorder,NMOSD)患者进行病例资料的回顾性分析并对其中19例患者进行门诊随访,旨在加强对该疾病的认识以及对新诊断标准在临床实践中的运用,并探讨影响复发的因素。方法本研究病例来源于在2014年4月至2017年4月至我院住院或门诊就诊的患者,应用2015年NMOSD诊断标准对患者进行重新诊断,最终确诊NMOSD39例,分析统计患者的一般资料、临床表现,收集实验室检查资料、影像学资料,随访记录患者治疗用药及复发情况。结果1.本研究中,男2例,女37例,男:女=1:18.5,首次发病年龄在16-64岁,平均发病年龄(39.2±13.9)岁;2.合并疾病:4例合并自身免疫系性疾病(10.3%),2例合并肿瘤(5.1%);3.首发季节:春季占38%,夏季占23%,秋季占18%,冬季占8%,不详占13%;4.首发诱因:有具体诱因占12.9%,包括劳累、感冒、腹泻,无明显诱因或不详占87.1%;5.复发诱因:复发有具体诱因的占43.2%,以劳累最为多见,也包括感冒、情绪抑郁、激素停药过快、硫唑嘌呤自行减量;无明显诱因或不详占56.8%;6.首诊科室:神经科占59%,非神经科占41%,以眼科最为多见,也包括消化科、骨科、皮肤科;7.首发核心临床症状:以脊髓炎(41%)和视神经炎(33.3%)起病为主;极后区综合征及急性脑干综合征也较为多见(15.4%);8.血清学检查:AQP4-IgG阳性26例(72.2%),阴性10例(27.7%),余下3例未检;自身免疫性抗体异常10例(25.6%),以ANA阳性最为多见,也包括抗SSA抗体、抗SSB抗体、抗双链DNA抗体阳性;9.脑脊液特点:有27例行腰穿检查,且资料相对完整,脑脊液压力有4例升高(220~280mm H_2O);细胞数有6例升高(35~150*10~6/L);蛋白有6例升高(486.5~984.8mg/L);糖有5例升高(4.54~5.4mmol/L);氯有3例升高(131~134.7mmol/L),1例降低(109mmol/L),余正常;9例行CSF寡克隆带检查,仅1例为弱阳性,6例同时行CSF-IgG及血清IgG检查,仅1例IgG指数升高(0.77);10.脊髓MRI特点:36例行脊髓MRI检查,32例脊髓受累(88.9%),多见于颈段及颈胸段(81.3%);31例受累3个节段(96.9%);1例受累3个节段(3.1%);脊髓病灶T2WI均为长T2信号,T1WI可为等或长T1信号,横断面可见病灶多位于灰质,也可累及白质,中央型损害占84.3%,偏心样损害占15.6%;16例行MRI增强检查,仅1例见局部病灶斑片状不均匀强化,余下15例均未见强化(93.8%);;11.颅脑MRI特点:38例行颅脑MRI检查,累及颅脑20例(52.6%),多见于延髓、桥脑和侧脑室旁;12.随访:门诊随访19例病人中,目前遗留症状包括单眼或双眼视力下降、肢体麻木、肢体无力、言语含糊及大便困难;规律服用硫唑嘌呤且服用时间大于1年者有5例,对其用药前后的年复发率进行比较,发现用药后年复发率均较用药前降低。结论1.NMOSD以中青年女性多见,首次发病平均年龄为(39.2±13.9)岁;2.脊髓受累多大于3个节段,以颈段及颈胸段受累(81.3%)最常见;T2WI均为长T2信号,T1WI可为等或长T1信号,以累及灰质为主,也可累及白质,增强多未见强化;50%以上的病人颅脑可受累,病灶多见于延髓、桥脑及侧脑室旁;3.硫唑嘌呤治疗可能降低患者的年复发率;4.感冒、劳累、情绪抑郁、过早停用激素、硫唑嘌呤突然减量或停用均有可能诱发复发。
[Abstract]:Objective to review the retrospective analysis of 39 cases of Neuromyelitis optica spectrum disorder (NMOSD) and to follow up 19 of them in the outpatient clinic. The purpose of this study is to strengthen the understanding of the disease and the application of the new diagnostic criteria in the practice of the clinic, and to discuss the factors that affect the recurrence. Methods the cases were derived from patients who were hospitalized or outpatient in our hospital from April 2014 to April 2017. The patients were rediagnosed by the NMOSD diagnostic standard in 2015, and the patients were finally diagnosed as NMOSD39 cases. The general data and clinical manifestations of the patients were analyzed, and the laboratory examination data were collected, the imaging data were collected, and the patients were followed up to record the patients' treatment. Results in the 1. study, 2 men and 37 women, male: female =1:18.5, the first onset age of 16-64 years, the average age of onset (39.2 + 13.9) years, 2. combined diseases: 4 cases with autoimmune disease (10.3%), 2 cases with tumor (5.1%); 3. season: Spring occupies 38%, summer occupies 23%, winter occupied, not detailed account; The first inducement: 12.9% specific inducements, including fatigue, colds, diarrhea, no apparent cause or unknown account of 87.1%; 5. recurrent causes: 43.2% of the recurrence of specific inducement, the most common cause of fatigue, including cold, emotional depression, excessive withdrawal of hormone, azathioprine self reduction; no obvious inducement or unknown 56.8%; 6. first clinic department: neurology department: Neurology Department 59%, the non neurology department accounted for 41%, the most common Ophthalmology, including the digestive department, Department of orthopedics, Department of Dermatology; 7. first core clinical symptoms: myelitis (41%) and optic neuritis (33.3%) primary disease; the extreme posterior zone syndrome and the acute brainstem syndrome are more common (15.4%); 8. serological examination: AQP4-IgG positive 26 cases (72.2%), negative 10 cases (27.7%), remaining 3 10 cases (25.6%) of autoimmune antibodies (10 cases) were the most common, including anti SSA, anti SSB, double stranded DNA, 9. cerebrospinal fluid, 27 cases of lumbar puncture, relatively complete data, 4 cases of cerebrospinal fluid pressure (220 ~280mm H_2O), 6 cell counts (35~150*10~6/L), and 6 cases of protein. High (486.5~984.8mg/L); 5 cases of sugar increased (4.54~5.4mmol/L); chlorine has 3 cases of elevated (131~134.7mmol/L), 1 cases (109mmol/L), Yu Zhengchang; 9 cases of CSF oligoclonal band examination, only 1 cases of weak positive, 6 cases with CSF-IgG and serum IgG examination, only 1 cases of IgG index increased (0.77); 10. spinal MRI characteristics: 36 routine spinal MRI examination, 32 spinal cord involvement (8) (8 (8)) 8.9%) more common in cervical and cervico thoracic segment (81.3%), 31 cases involving 3 segments (96.9%) and 1 cases involving 3 segments (3.1%); T2WI of the spinal cord was long T2 signal, T1WI was equal or long T1 signal, the lesions were mostly located in gray matter, white matter, central type of 84.3%, and eccentricity lesion in 15.6%; 16 routine MRI enhancement examination, only 1 cases, see only 1 cases. Local lesion patchy inhomogeneous enhancement, no enhancement (93.8%) in the remaining 15 cases; 11. craniocerebral MRI characteristics: 38 cases of craniocerebral MRI examination, involving 20 cases of craniocerebral (52.6%), more seen in the medulla, pontine and lateral ventricle; 12. follow up: among the 19 patients followed up, the symptoms included the decrease of visual acuity, numbness of the extremities, and the weakness of the limbs. There were 5 cases of azathioprine taking the regular use of azathioprine for more than 1 years, and compared the annual recurrence rate before and after the use of azathioprine. It was found that the annual recurrence rate was lower than that before the medication. Conclusion 1.NMOSD is more common in young and middle-aged women, the average age of the first onset is (39.2 + 13.9) years; 2. spinal cord involvement is more than 3 segments, with neck more than 3 segments. Segment and cervical thoracic segment involvement (81.3%) was the most common; T2WI was long T2 signal, T1WI could be equal or long T1 signal, mainly with gray matter, may involve white matter, and increased multiple no enhancement; more than 50% of the patients can be involved in the craniocerebral, and the focus is mostly in the medulla, bridge brain and lateral ventricle; 3. azathioprine may reduce the rate of recurrence of the patients; 4. colds, tired, Emotional depression, premature withdrawal of hormones, sudden reduction or withdrawal of azathioprine may induce relapse.
【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R744.52

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