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:100037,();()BradburgEggtestoton,ShyDrager,Chokroverty(primaryorthostatichypotension,POH)POH(OH),,,GrahamOppenheimer(multiplesystematrophy,MSA),,Shy2Drager(SDS)POH(OPCA)(SND),,[1],MSA,MSA,()[1],(CNS),MSA[2],MSAcyclin2dependentkinases5(5,cdk5),,2MSA2(2synuclein)[3],MSACNSMSA,CNS,Wenning[4]203MSA,,,OHOnufs,,SDSSNDOPCA,MSA,OPCA50,,,MSA(SDS)(OPCA)(SND),,,1.:(1)(POH):,/30/20mmHg(1mmHg=01133kPa),POHMSA,MSA,MSAPOH(2):,,,Beck[5]62MSA,100%,96%,37%,43%,57%,,,(EMG)(3):,,,Horner2.:1,3.:,,,,4.:,,,,EMG,[6]5.:,,,61(SA):,,1/3MSA(stridor)SA,[7]Williams12SDS8,4,SA,,,41120024352ChinJNeurol,April2002,Vol35,No.27.:Nee[8]8SDS5,,,,7Testa[9]59MSA7.5,60SDS,19,78,544%,10MSAEMG(EMG)[5,10]EMG,CTMRI,MSAMRIMSA,,T1T2[11]T2,[12]PETMSA,[13]MSA[14,15]MSA:,,;POH,;;();()SA,;,EMG;(),;,;;1.[16]:MSAPOHSDA(IOH),IOH,IOHCNSKontosIOH,(NE),NEMSAIOHNE,MSANE,NEIOHNE,,SDS2.[16]:,,,NE,3.:MSA,,,OH4.:,SDS,OHNE,MSA10%,,,[16],:,OH,;OHOH,,,,,922,(1),SA,,,,:,;,,CEB,Q10:;1InoueM,YagishitaS,RyoM,etal.Thedistributionanddynamicdensityofoligodendroglialcytoplasmicinclusions(GCIs)inmultiplesystematrophy:acorrelationbetweenthedensityofGCIsandthedegreeofinvolvementofstriatonigralandolivopontocerebellarsystems.ActaNeuropatholBerl,1997,93:5852591.2NakamuraS,KawamotoY,NakanoS,etal.Cyclin2dependentkinase5andmitogen2activatedproteinkinaseinglialcytoplasmicinclusionsinmultiplesystematrophy.JNeuropatholExpNeurol,1998,57:6902698.3ShojiM,HarigayaY,SasakiA,etal.AccumulationofNACP/alpha2synucleininLewybodydiseaseandmultiplesystematrophy.JNeurolNeurosurgPsychiatry,2000,68:6052608.4WenningGK,TisonF,BenShlomoY,etal.Multiplesystematrophy:areviewof203pathologicallyprovencases.MovDisord,1997,12:1332147.5BeckRO,BettsCD,FowlerCJ.Genitourinarydysfunctioninmultiplesystematrophy:clinicalfeaturesandtreatmentin62cases.JUrol,1994,151:133621341.6BluntSB,KhalilNM,PerkinGD.Facialmyokymiainmultiplesystematrophy.MovDisord,1997,12:2352238.51120024352ChinJNeurol,April2002,Vol35,No.27HughesRG,GibbinKP,LoweJ.Vocalfoldabductorparalysisasasolitaryandfatalmanifestationofmultiplesystematrophy.JLaryngolOtol,1998,112:1772178.8NeeLE,ScottJ,PolinskyRJ.OlfactorydysfunctionintheShy2Dragersyndrome.ClinAutonRes,1993,3:2812282.9TestaD,FilippiniG,FarinottiM,etal.Survivalinmultiplesystematrophy:astudyofprognosticfactorsin59cases.JNeurol,1996,243:4012404.10StocchiF,CarboneA,InghilleriM,etal.UrodynamicandneurophysiologicalevaluationinParkinsonsdiseaseandmultiplesystematrophy.JNeurolNeurosurgPsychiatry,1997,62:5072511.11YagishitaT,KojimaS,HirayamaK.MRIstudyofdegenerativeprocessinmultiplesystematrophy.RinshoShinkeigaku,1995,35:1262131.12LuXY,ChenJ,WangL,etal.CorrelativestudiesofMRfindingswithneuropathologyinShy2Dragersyndromeandstriatonigraldegeneration.ChinMedJ(Engl),1997,110:6282631113OtsukaM,IchiyaY,KuwabaraY,etal.GlucosemetabolisminthecorticalandsubcorticalbrainstructuresinmultiplesystematrophyandParkinsonsdisease:apositronemissiontomographicstudy.JNeurolSci,1996,144:77283.14WenningGK,Ben2shlomoY,HughesA,etal.WhatclinicalfeaturesaremostusefultodistinguishdefinitemultiplesystematrophyfromParkinsonsdisease?JNeurolNeurosurgPsychiatry,2000,68:4342440.15LitvanI,GoetzCG,JankovicJ,etal.Whatistheaccuracyoftheclinicaldiagnosisofmultiplesystematrophy?Aclinicopathologicstudy.ArchNeurol,1997,54:9372944.16CotLJ.Neurogenicorthostatichypotension.In:RowlandLP,ed.MerrittstextbookofNeurology.9thed.London:Williams&Wilkins,1995.8302833.(:2000212225)(:):265200();(),1996(PYM),56:5632(5711%),24(4219%)3065,(5619811)523,511:46,10;31,18,756CT2%,2%4mlPYM8mg,1ml1ml(2mgPYM),,1,PYM4mg1,41,14mg,:348(8517%),48(1413%)(1)48(8517%),(1)6(1017%),(1)2(316%),9614%56PYM1016mg,114mg,1,56:,5212/10,,,PYM,,,,,,,,(:2001202221)(:),,200210,,:3000800,300,,,:42:100710,200291061120024352ChinJNeurol,April2002,Vol35,No.2
本文标题:多系统萎缩的诊断与治疗进展
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