·94·经科杂志2003年4月第36卷第2in』!!：多系统萎缩44例的临床与神经电生理特点分析王含崔丽英【摘要】目的探讨临床和神经电生理检查对于多系统萎缩(MSA)的诊断价值。方法对44例MSA患者分组分析其临床特点、肌电图(EMG)、神经传导速度(NCV)、躯体感觉诱发电位(SEP)、运动诱发电位(MEP)、脑干听觉诱发电位(BAEP)及视觉诱发电位(VEP)。并比较临床表现和电生理检查结果在各组间的差异。结果本组患者以自主神经功能障碍和小脑『生共济失调的异常率最高(88．6％)。帕金森综合征出现率在各组间差异有显著意义(P值分别为0．027、0．007、0．025)，卧立位血压异常在拟诊组和可能组间差异有显著意义(76．5％、18．2％)。EMG和NCV的异常率为38．1％，各组间出现率有不同。各种诱发电位的异常出现率在分组比较中无显著性差异。结论仅凭临床表现无法对MSA进行分组。各项神经电生理检查中以BAEP的阳性率最高。EMG和NCV结果在各组之间的差异提示该项检查对于MSA的分组可能有一定的帮助。【关键词】多系统萎缩；肌电描记术；体感诱发电位；电生理学Clinicalandelectroneurophysiologicalstudyon44casesofmultiplesystematrophyWANGHan，CUILi·ying．DepartmentofNeurology，PekingUnionCollegeHospital，PekingUnionMedicalCollege，ChineseAcademyofMedicalSciences．Beijing．10073oChina【Abstract】ObjectiveToidentifytheclinicalandneur叩hysiologicalfeaturesofmuhiplesystematrophy(MSA)andexplorediagnosticvaluesusingvarioustechniquesinelectro·neurophysiology．MethodsForty—foursubjectsdiagnosedasmuhiplesystematrophy(MSA)during1983to2001inPUMCHospitalwerestudiedretrospectivelyabouttheirclinica1features．routineelectromyography(EMG)，nerveconductivevelocity(NCV)，somatosensoryevokedpotential(SEP)，motor·~vokedpotential(MEP)，brainstemauditoryevokedpotential(BAEP)andvisualevokedpotential(VEP)．Theclinicalfeaturesandtheelectroneurophysiologicresultswereanalyzedindifferentsubgroups(probable·MSAandpossible·MSA，OPCAandnon．OPCA．classified．MSAandunclassified．MSA)．ResultsMostpatientswerefoundtobesuferedfromautonomicfailure(88．6％)andcerebelladysfunctionf88．6％)．rhepyramidalsignwasseenin72．7％ofpatients．Noremarkablestatisticaldiferenceswerefoundinalloftheabovethreedomains．TheincidenceofParkinsonismwas36．4％ofallthepatients．whichshowedsignificantdifferencesbetweenprobableandpossible．MSA(54．5％vs18．2％)，non—OPCAand()PCA(50％vs7．1％)．unclassified．MSAandclassified．MSA(52％vs15．8％)．EMGwasabnormalirI7patients(38．=【％)，allofthemwereneurogenicimpairments．TheratesofabnormalEMGwerevarieddiferentlybetweenprobableandpossible．MSA(54．5％vs22．2％)，non．OPCAandOPCA(53．8％vs14．3％)，unclassified．MSAandclassified．MSA(58．3％vs12．5％)．butwithoutstatisticaldiferences．TheabnormalratesinBAEP，SEP．VEPandMEPwere56．7％，28％，23．1％and20．0％，respectively，andtherewerenosignificantdiferencesbetweensubgroups．ConclusionsItshouldbehardtodivideMSAintogroupsonlybytheclinicalmanifestations．AllEPswereabnormalinsomeextent．ThemostsensitivetestisBAEPincurrentstudy．buttherewerenodiferencesamongthesubgroupsintheratesofabnor~aa1EPs．ThedifterenceofEMGandNCVbetweenthegroupsshowedthatthetechniquemightbehelpfulirIthediagnosisofMSA．【Keywords】Multiplesystematrophy；Electromyography；Evokedpotentials；Electrophysiology多系统萎缩(MSA)是一组病因不清的进行性作者单位：100730中国医学科学院中国协和医科大学北京协和医院神经科．论著．神经系统变性疾病。传统的观念认为该病主要包括：黑质纹状体变性(SND)、Shy—Drager综合征(SDS)、散发性橄榄脑桥小脑萎缩(sOPCA)。由于少突胶质细胞中高密度的嗜银性胞质...
