昆明醫(yī)學院第二附屬醫(yī)院

昆明醫(yī)學院第二附屬醫(yī)院

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時間:2019-05-11

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1、NeurologyDepartmentofNeurology,The2ndaffiliatedhospital,kunmingMedicalcollege昆明醫(yī)學院第二附屬醫(yī)院 神經(jīng)病學教研室熊靜MotorNeurondisease運動神經(jīng)元病MotorneurondiseaseDefinitionClinicfeaturesDiagnosis&DifferentiatingdiagnosisTreatmentPrognosisDefinition(1)Motorneurondisease(MND)isagroupofdiseasescharacterizedbyc

2、hronic,progressiveneuronaldegeneration.運動神經(jīng)元病是一組慢性進行性神經(jīng)元變性疾病。Definition(2)Thecauseisunknown.Thedegenerationpredominantlyinvolvesmotorneurons,includinguppermotorneuronsandlowermotorneurons.病因不清。變性主要侵犯上、下運動神經(jīng)元。AnteriorhornPyramidalcellsMotornucleiPyramidaltractsClinicalmanifestations(分型)

3、UppermotorneurondeficitPrimaryLateralSclerosisLowermotorneurondeficitProgressivespinalmuscularatrophyMixedupperandlowermotorneurondeficitAmyotrophiclateralsclerosis(ALS)ProgressiveBulbarPalsy原發(fā)性側(cè)索硬化進行性脊肌萎縮肌萎縮側(cè)索硬化進行性延髓麻痹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

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