myasthenia gravis：重症肌无力

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1、MyastheniaGravisMikeWillcutMyastheniaWhat?My-as-thee-nee-ahGrr-a-visAutoimmunediseaseHereditarygeneticmutationCausesmuscleweaknessWherehaveIheardthat?ContraindicationforVersedFentanylPrecautionforsuxPathophysiologyAutoImmuneAntibodiesattackAchReceptorsDecreasedc

2、ellexcitementElectricalthresholdNoactionpotentialWeaknessCellwithMGThismightbeontheQuizHowdoesMGaffectthebody?A–BlocksmotorreceptorscausingweaknessB-IncreasesstimulusthresholdC-DecreasesstimulusthresholdD-ReducesamountofAchreleasedcausingweaknessClassification1:

3、Eyemuscleweakness(EMW)2:EMWandothermildmuscleweaknessPredominantlylimboraxialmusclesPredominantlybulbarorrespiratorymuscles3:EMWandothermoderateweaknessPredominantlylimboraxialmusclesPredominantlybulbarorrespiratorymuscles4:EMWandothersevereweaknessPredominantly

4、limboraxialmusclesPredominantlybulbarorrespiratorymuscles5:IntubationneededtomaintainairwayWho’satRiskFemales20-40Males60-80ThosewithanotherAIDiseasePenicillamineusers(rheumatoidmed)EnlargedThymusorthymoma20in100,000or2in1million(discrepancy)DiseasesTypicallyAss

5、ociatedWithMGDiabetesType1RheumatoidArthritisLupusDemyelinationCNSDiseaseOtherAutoimmunediseasesWhatwillwesee?HallmarkS/SEyeliddrooping(ptosis)Impairedspeech(dysarthria)DifficultySwallowing(dysphagia)DoubleVision(dipoplia)Easilyfatigued,quickrecoverywithrestWadd

6、linggaitPhysicalExamTestformusclefatigueabilityLookupandtothesidefor30secIfsupine,lookatfeetfor1minArmsstretchedforwardfor1min10deepkneebendsorsquatsLookforptosisandaskaboutdiplopiaMyastheniaCrisisRequiresimmediateassistanceShortnessofbreathExtrememuscleweakness

7、GeneralfatigueablilityRespiratorymuscleparalysisMGinPregnancyManymothersgetworse1sttrimesterandbetter2ndand3rdtrimester.Upto10%ofinfantsborntomotherswithMGwillhaveTransientNeonatalMyasthenia.Weakcry,poormuscletone,difficultybreathingetc…InterventionsDependingons

8、everityRSISupportivecareTriggersMaybetriggeredby:InfectionStressFeverAdversereactiontomedicationsHospitalTestsBloodtestsTestsforspecificantibodiesOnly50%accurateCTChe