本文選題：視神經(jīng)脊髓炎譜系疾病 + 隨訪��； 參考：《廣西醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的本研究對(duì)39例視神經(jīng)脊髓炎譜系疾病(Neuromyelitis optica spectrum disorder,NMOSD)患者進(jìn)行病例資料的回顧性分析并對(duì)其中19例患者進(jìn)行門診隨訪,旨在加強(qiáng)對(duì)該疾病的認(rèn)識(shí)以及對(duì)新診斷標(biāo)準(zhǔn)在臨床實(shí)踐中的運(yùn)用,并探討影響復(fù)發(fā)的因素。方法本研究病例來源于在2014年4月至2017年4月至我院住院或門診就診的患者,應(yīng)用2015年NMOSD診斷標(biāo)準(zhǔn)對(duì)患者進(jìn)行重新診斷,最終確診NMOSD39例,分析統(tǒng)計(jì)患者的一般資料、臨床表現(xiàn),收集實(shí)驗(yàn)室檢查資料、影像學(xué)資料,隨訪記錄患者治療用藥及復(fù)發(fā)情況。結(jié)果1.本研究中,男2例,女37例,男:女=1:18.5,首次發(fā)病年齡在16-64歲,平均發(fā)病年齡(39.2±13.9)歲;2.合并疾病:4例合并自身免疫系性疾病(10.3%),2例合并腫瘤(5.1%);3.首發(fā)季節(jié):春季占38%,夏季占23%,秋季占18%,冬季占8%,不詳占13%;4.首發(fā)誘因:有具體誘因占12.9%,包括勞累、感冒、腹瀉,無明顯誘因或不詳占87.1%;5.復(fù)發(fā)誘因:復(fù)發(fā)有具體誘因的占43.2%,以勞累最為多見,也包括感冒、情緒抑郁、激素停藥過快、硫唑嘌呤自行減量;無明顯誘因或不詳占56.8%;6.首診科室:神經(jīng)科占59%,非神經(jīng)科占41%,以眼科最為多見,也包括消化科、骨科、皮膚科;7.首發(fā)核心臨床癥狀:以脊髓炎(41%)和視神經(jīng)炎(33.3%)起病為主;極后區(qū)綜合征及急性腦干綜合征也較為多見(15.4%);8.血清學(xué)檢查:AQP4-IgG陽性26例(72.2%),陰性10例(27.7%),余下3例未檢;自身免疫性抗體異常10例(25.6%),以ANA陽性最為多見,也包括抗SSA抗體、抗SSB抗體、抗雙鏈DNA抗體陽性;9.腦脊液特點(diǎn):有27例行腰穿檢查,且資料相對(duì)完整,腦脊液壓力有4例升高(220~280mm H_2O);細(xì)胞數(shù)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寡克隆帶檢查,僅1例為弱陽性,6例同時(shí)行CSF-IgG及血清IgG檢查,僅1例IgG指數(shù)升高(0.77);10.脊髓MRI特點(diǎn):36例行脊髓MRI檢查,32例脊髓受累(88.9%),多見于頸段及頸胸段(81.3%);31例受累3個(gè)節(jié)段(96.9%);1例受累3個(gè)節(jié)段(3.1%);脊髓病灶T2WI均為長(zhǎng)T2信號(hào),T1WI可為等或長(zhǎng)T1信號(hào),橫斷面可見病灶多位于灰質(zhì),也可累及白質(zhì),中央型損害占84.3%,偏心樣損害占15.6%;16例行MRI增強(qiáng)檢查,僅1例見局部病灶斑片狀不均勻強(qiáng)化,余下15例均未見強(qiáng)化(93.8%);;11.顱腦MRI特點(diǎn):38例行顱腦MRI檢查,累及顱腦20例(52.6%),多見于延髓、橋腦和側(cè)腦室旁;12.隨訪:門診隨訪19例病人中,目前遺留癥狀包括單眼或雙眼視力下降、肢體麻木、肢體無力、言語含糊及大便困難;規(guī)律服用硫唑嘌呤且服用時(shí)間大于1年者有5例,對(duì)其用藥前后的年復(fù)發(fā)率進(jìn)行比較,發(fā)現(xiàn)用藥后年復(fù)發(fā)率均較用藥前降低。結(jié)論1.NMOSD以中青年女性多見,首次發(fā)病平均年齡為(39.2±13.9)歲;2.脊髓受累多大于3個(gè)節(jié)段,以頸段及頸胸段受累(81.3%)最常見;T2WI均為長(zhǎng)T2信號(hào),T1WI可為等或長(zhǎng)T1信號(hào),以累及灰質(zhì)為主,也可累及白質(zhì),增強(qiáng)多未見強(qiáng)化;50%以上的病人顱腦可受累,病灶多見于延髓、橋腦及側(cè)腦室旁;3.硫唑嘌呤治療可能降低患者的年復(fù)發(fā)率;4.感冒、勞累、情緒抑郁、過早停用激素、硫唑嘌呤突然減量或停用均有可能誘發(fā)復(fù)發(fā)。
[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.
【學(xué)位授予單位】：廣西醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R744.52

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