昆明医学院第二附属医院

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1、NeurologyDepartmentofNeurology,The2ndaffiliatedhospital,kunmingMedicalcollege昆明医学院第二附属医院神经病学教研室熊静MotorNeurondisease运动神经元病MotorneurondiseaseDefinitionClinicfeaturesDiagnosis&DifferentiatingdiagnosisTreatmentPrognosisDefinition(1)Motorneurondisease(MND)isagroupofdiseasescharacterizedbyc

2、hronic,progressiveneuronaldegeneration.运动神经元病是一组慢性进行性神经元变性疾病。Definition(2)Thecauseisunknown.Thedegenerationpredominantlyinvolvesmotorneurons,includinguppermotorneuronsandlowermotorneurons.病因不清。变性主要侵犯上、下运动神经元。AnteriorhornPyramidalcellsMotornucleiPyramidaltractsClinicalmanifestations（分型）

3、UppermotorneurondeficitPrimaryLateralSclerosisLowermotorneurondeficitProgressivespinalmuscularatrophyMixedupperandlowermotorneurondeficitAmyotrophiclateralsclerosis(ALS)ProgressiveBulbarPalsy原发性侧索硬化进行性脊肌萎缩肌萎缩侧索硬化进行性延髓麻痹Clinicfeatures-ALS（1）Age:30-60yearsoldOnsetisinsidiousCourseischron

4、icandprogressive好发于30-60岁，起病隐袭，呈慢性进行性病程。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呈典型失神经支配改变Imagingstudy,CSF,EvokepotentialsareusuallynormalDiagnosis&DifferentialDiagnosisNosensorydeficitW

7、eaknessandatrophyoftheaffectedmusclesSignsofmotorneuronlesionsElectromyographyThekeysTreatmentNoeffectivetreatmentSymptomatictherapyandsupportivemeasuresPrognosisPrognosisisverypoor.Mostcommonlyfrompulmonaryinfectionsandweaknessofrespiratorymuscle.SummaryMotorneurondiseaseischaracter