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1、·論著·2009年6月第47卷第18期19例多系統(tǒng)萎縮臨床分析及早期診斷楊萍華1黃堅(jiān)2趙忠新2(1.上海市浦東新區(qū)公利醫(yī)院神經(jīng)內(nèi)科,上海200135;2.第二軍醫(yī)大學(xué)附屬長(zhǎng)征醫(yī)院神經(jīng)內(nèi)科,上海200003)[摘要]目的探討多系統(tǒng)萎縮(MSA)的臨床表現(xiàn),評(píng)價(jià)輔助檢查對(duì)其臨床早期診斷的意義。方法回顧性分析我院及長(zhǎng)征醫(yī)院2006年1月~2008年12月診治的19例MSA患者的臨床資料及主要輔助檢查。結(jié)果多系統(tǒng)萎縮是一組緩慢起病,逐漸加重的疾病,臨床表現(xiàn)有自主神經(jīng)功能障礙、小腦共濟(jì)失調(diào)和帕金森綜合征。植物神經(jīng)癥狀出現(xiàn)較早,錐體外系癥狀是MSA最常
2、見(jiàn)的運(yùn)動(dòng)異常,這兩者是MSA容易誤診的主要原因。神經(jīng)影像顯示腦萎縮和葡萄糖代謝減低;肛門括約肌肌電圖檢查具有較高敏感度和特異度。結(jié)論MSA臨床表現(xiàn)復(fù)雜多樣,早期易誤診。依據(jù)詳細(xì)的病史和全面的神經(jīng)系統(tǒng)檢查,并輔以相關(guān)檢查,可提高M(jìn)SA早期診斷的準(zhǔn)確性。[關(guān)鍵詞]多系統(tǒng)萎縮;臨床分析;早期診斷[中圖分類號(hào)]R74[文獻(xiàn)標(biāo)識(shí)碼]A[文章編號(hào)]1673-9701(2009)18-12-04TheEarlyDiagnosisandClinicalAnalysisof19CasesofMultipleSystemAtrophyYANGPinghua1H
3、UANGJian2ZHAOZhongxin21.DepartmentofNeurology,GongliHospital,Shanghai,200135,China;2.DepartmentofNeurology,AffiliatedChangzhengHospital,TheSecondMilitaryMedicalUniversityofPLA,Shanghai,200003,China[Abstract]ObjectiveToexploretheclinicalmanifestationsofmultiplesystematrophy
4、(MSA),andassessthesignificanceoftheassistantexaminationfortheearlydiagnosis.MethodsTheclinicalandassistantexaminationdataof19casesofMSAwereretrospectivelyanalyzed.ResultsMultiplesystematrophycompriseadiversegroupofdiseaseswhichslowlyonset,andgraduallyincrease.Theclinicalma
5、nifestationswereautonomicnervedysfunction,cerebellarataxiaandParkinsonism.Theautonomicnervesymptomsweretheearlyclinicalmanifestation,andtheextrapyramidalsymptomsweretheoptimafrequentmotionabnormality.ThesewerethetwomainreasonsforMSAtobemisdiagnosised.Brainatrophybrainatrop
6、hyandhypometabolismofglucoseweredetectedintheNeuroimagingimaging.Analsphincter(EAS)eletromyography(EMG)wassensitiveandspecific.ConclusionClinicalmanifestationsofthisdiseasearecomplexanddiverseearlyinthedisease,weoftenmisdiagnosed.Basedondetailedmedicalhistoryandcomprehensi
7、veexaminationofthenervoussystem,andsupplementedbyrelevantinspection,wecanimprovetheaccuracyofearlydiagnosisofMSA.[KeyWords]Multiplesystematrophy;Clinicalanalysis;Earlydiagnosis多系統(tǒng)萎縮(Multiplesystematrophy,MSA)是一種成年起例,女性4例;發(fā)病年齡39~78歲,平均(61.3±7.3)歲;病程病,進(jìn)行性加重的神經(jīng)變性疾病,病因不明。臨床癥狀主
8、要累及0.5~6年,平均(2.5±1.7)年。均為隱匿起病,進(jìn)行性發(fā)展。全組錐體外系、自主神經(jīng)和小腦三大系統(tǒng)。此病臨床癥狀較復(fù)雜,尤均無(wú)明顯的家族史,無(wú)腦炎、腦外傷史,無(wú)藥物、毒