多系统萎缩的诊断与治疗进展

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100037BradburgEggtestotonShyDragerChokrovertyprimaryorthostatichypotensionPOHPOHOHGrahamOppenheimermultiplesystematrophyMSAShy-DragerSDSPOHOPCASND1MSAMSA1CNSMSA2MSAcyclin-dependentkinases55cdk52MSAα-α-synuclein3MSACNSMSACNSWenning4203MSAOHOnufsSDSSNDOPCAMSAOPCA50MSASDSOPCASND1.1POH/30/20mmHg1mmHg=0.133kPaPOHMSAMSAMSAPOH2Beck562MSA100%96%37%43%57%EMG3Horner2.13.4.EMG65.6.SA1/3MSAstridorSA7Williams12SDS84SA41120024352ChinJNeurolApril2002Vol35No.27.Nee88SDS57Testa959MSA7.560SDS197~8544%10MSAEMGEMG510EMGCTMRIMSAMRIMSAT1T211T212PETMSA13MSA1415MSA①②POH③④SA⑤EMG⑥⑦⑧⑨1.16MSAPOHSDAIOHIOHIOHCNSKontosIOHNENEMSAIOHNEMSANENEIOHNESDS2.16NE3.MSAOH4.SDSOHNEMSA10%16①②OHOH③OH9-α-α1④SA⑤⑥CEBQ10⑦1InoueMYagishitaSRyoMetal.ThedistributionanddynamicdensityofoligodendroglialcytoplasmicinclusionsGCIsinmultiplesystematrophyacorrelationbetweenthedensityofGCIsandthedegreeofinvolvementofstriatonigralandolivopontocerebellarsystems.ActaNeuropatholBerl199793585-591.2NakamuraSKawamotoYNakanoSetal.Cyclin-dependentkinase5andmitogen-activatedproteinkinaseinglialcytoplasmicinclusionsinmultiplesystematrophy.JNeuropatholExpNeurol199857690-698.3ShojiMHarigayaYSasakiAetal.AccumulationofNACP/alpha-synucleininLewybodydiseaseandmultiplesystematrophy.JNeurolNeurosurgPsychiatry200068605-608.4WenningGKTisonFBenShlomoYetal.Multiplesystematrophyareviewof203pathologicallyprovencases.MovDisord199712133-147.5BeckROBettsCDFowlerCJ.Genitourinarydysfunctioninmultiplesystematrophyclinicalfeaturesandtreatmentin62cases.JUrol19941511336-1341.6BluntSBKhalilNMPerkinGD.Facialmyokymiainmultiplesystematrophy.MovDisord199712235-238.51120024352ChinJNeurolApril2002Vol35No.27HughesRGGibbinKPLoweJ.Vocalfoldabductorparalysisasasolitaryandfatalmanifestationofmultiplesystematrophy.JLaryngolOtol1998112177-178.8NeeLEScottJPolinskyRJ.OlfactorydysfunctionintheShy-Dragersyndrome.ClinAutonRes19933281-282.9TestaDFilippiniGFarinottiMetal.Survivalinmultiplesystematrophyastudyofprognosticfactorsin59cases.JNeurol1996243401-404.10StocchiFCarboneAInghilleriMetal.UrodynamicandneurophysiologicalevaluationinParkinson'sdiseaseandmultiplesystematrophy.JNeurolNeurosurgPsychiatry199762507-511.11YagishitaTKojimaSHirayamaK.MRIstudyofdegenerativeprocessinmultiplesystematrophy.RinshoShinkeigaku199535126-131.12LuXYChenJWangLetal.CorrelativestudiesofMRfindingswithneuropathologyinShy-Dragersyndromeandstriatonigraldegeneration.ChinMedJEngl1997110628-631.13OtsukaMIchiyaYKuwabaraYetal.GlucosemetabolisminthecorticalandsubcorticalbrainstructuresinmultiplesystematrophyandParkinsonsdiseaseapositronemissiontomographicstudy.JNeurolSci199614477-83.14WenningGKBen-shlomoYHughesAetal.WhatclinicalfeaturesaremostusefultodistinguishdefinitemultiplesystematrophyfromParkinsonsdiseaseJNeurolNeurosurgPsychiatry200068434-440.15LitvanIGoetzCGJankovicJetal.WhatistheaccuracyoftheclinicaldiagnosisofmultiplesystematrophyAclinicopathologicstudy.ArchNeurol199754937-944.16CotéLJ.Neurogenicorthostatichypotension.InRowlandLPed.Merritt'stextbookofNeurology.9thed.LondonWilliams&Wilkins1995.830-833.2000-12-252652001996PYM56563257.1%2442.9%30~6556.9±8.15235.14610Ⅱ31Ⅲ18ⅡⅢ756CT2%2%4mlPYM8mg1ml1ml2mgPYMⅡⅢ1PYM4mg14114mg34885.7%4814.3%14885.7%1610.7%123.6%96.4%56PYM10.6mg1.4mg15652.2/10PYM2001-02-21200210300080030042100710200291061120024352ChinJNeurolApril2002Vol35No.2

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