运动神经元病ppt课件

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1、ClinicalfeaturesofalsandotherformsofmotorneurondiseaseChenYan运动神经元病（motorneurondiseaseMND）：是一组病因未明的选择性侵犯脊髓前角细胞、脑干后组运动神经元、皮质锥体细胞及锥体束的慢性进行性神经变性疾病。SPECTRUMOFMOTORNEURONDISEASEAmyotrophiclateralsclerosis(ALS）Progressivemuscularatrophy（PMA）Primarylateralsclerosis（PLS）Progressivebulbar

2、palsy（PBP）FlailarmsyndromeFlaillegsyndromeALS-plussyndromeALSisthemostcommonformofmotorneurondiseaseandincludesupperandlowermotorneuronpathology.PMAisaprogressivelowermotorneurondisorder.SomeexpertsbelieveitrepresentsaformofALS.PLSisaprogressiveisolateduppermotorneurondisorder.Co

3、mparedwithALS,itischaracterizedbyslowerprogression,lackofweightloss,andabsenceoflowermotorneuronfindingsonexaminationorelectromyographyinthefirstfouryearsaftersymptomonset.PBPisaprogressiveupperandlowermotorneurondisorderofcranialmuscles.Thisconditionmayoccasionallystayisolatedto

4、thebulbarsegment,butmorecommonly,upperandlowermotorneuronsignsandsymptomsspreadtoinvolveothersegments.InitialpresentationandpatientcomplaintsAsymmetriclimbweaknessisthemostcommonpresentationofALS(80percent).Upperextremityonsetismostoftenheraldedbyhandweaknessbutmaybeginintheshoul

5、dergirdlemuscles.LowerextremityonsetofALSmostoftenbeginswithweaknessoffootdorsiflexion(footdrop),whileproximalpelvicgirdleonsetislesscommon.UppermotorneuronsymptomsLowermotorneuronsymptomsCognitivesymptomsAutonomicsymptomsParkinsonismandsupranucleargazepalsySensorysymptomsClinica

6、lpatternsofprogressionCognitiveimpairment,typicallyrelatedtofrontotemporalexecutivedysfunction,mayprecedeorfollowtheonsetofuppermotorneuronand/orlowermotorneurondysfunctioninpatientswithALS.FrontotemporaldementiamaybeassociatedwithALSin15to50percentofcases.Cognitivedysfunctiondoe

7、snotexcludethediagnosisofALS.CognitivesymptomsAutonomicsymptoms,parkinsonism,supranucleargazeparesis,and/orsensorylossmayoccuraspartofanALS-plussyndrome.Inpatientswithunilateralarm(leg)onsetthemostcommonpatternofspreadistothecontralateralarm(leg),thentotheipsilateralleg(arm),then

8、tothecontralateralremainingleg(arm),andt