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1、NeurologyDepartmentofNeurology,The2ndaffiliatedhospital,kunmingMedicalcollege昆明醫(yī)學(xué)院第二附屬醫(yī)院 神經(jīng)病學(xué)教研室熊靜MotorNeurondisease運(yùn)動(dòng)神經(jīng)元病MotorneurondiseaseDefinitionClinicfeaturesDiagnosis&DifferentiatingdiagnosisTreatmentPrognosisDefinition(1)Motorneurondisease(MND)isagroupofdiseasescharacterizedbyc
2、hronic,progressiveneuronaldegeneration.運(yùn)動(dòng)神經(jīng)元病是一組慢性進(jìn)行性神經(jīng)元變性疾病。Definition(2)Thecauseisunknown.Thedegenerationpredominantlyinvolvesmotorneurons,includinguppermotorneuronsandlowermotorneurons.病因不清。變性主要侵犯上、下運(yùn)動(dòng)神經(jīng)元。AnteriorhornPyramidalcellsMotornucleiPyramidaltractsClinicalmanifestations（分型）
3、UppermotorneurondeficitPrimaryLateralSclerosisLowermotorneurondeficitProgressivespinalmuscularatrophyMixedupperandlowermotorneurondeficitAmyotrophiclateralsclerosis(ALS)ProgressiveBulbarPalsy原發(fā)性側(cè)索硬化進(jìn)行性脊肌萎縮肌萎縮側(cè)索硬化進(jìn)行性延髓麻痹Clinicfeatures-ALS（1）Age:30-60yearsoldOnsetisinsidiousCourseischron
4、icandprogressive好發(fā)于30-60歲，起病隱襲，呈慢性進(jìn)行性病程。Clinicfeatures-ALS（2）Weaknessofupperextremities;AtrophyandfasciculationsWeaknessoflowerextremities;IncreasedmuscletoneIncreasedtendonreflexes;PositiveBabinskisignClinicfeatures-ALS（2）Weaknessoflowerextremities;IncreasedmuscletoneIncreasedtendonre
5、flexes;PositiveBabinskisignWeaknessofupperextremities;AtrophyandfasciculationsDifficultyinswallowingandspeaking；Droopingofthepalate；AtrophyandfasciculationoftongueClinicfeatures-ALS（2）Weaknessoflowerextremities;IncreasedmuscletoneIncreasedtendonreflexes;PositiveBabinskisignWeaknessofup
6、perextremities;AtrophyandfasciculationsDifficultyinswallowingandspeaking;Droopingofthepalate；AtrophyandfasciculationoftongueInvestigativeStudyElectromyographyshowsneuropathiclesion呈典型失神經(jīng)支配改變Imagingstudy,CSF,EvokepotentialsareusuallynormalDiagnosis&DifferentialDiagnosisNosensorydeficitW
7、eaknessandatrophyoftheaffectedmusclesSignsofmotorneuronlesionsElectromyographyThekeysTreatmentNoeffectivetreatmentSymptomatictherapyandsupportivemeasuresPrognosisPrognosisisverypoor.Mostcommonlyfrompulmonaryinfectionsandweaknessofrespiratorymuscle.SummaryMotorneurondiseaseischaracter
