重癥疾病性神經(jīng)肌肉?。ㄓ⑽恼n件）

CRITICALILLNESS

NEUROMYOPATHY

CRITICALILLNESS

NEUROMY1AbbreviationsCIPcriticalillnesspolyneuropathyCIMcriticalillnessmyopathyCMAPcompoundmusclactionpotentialsSNAPsensorynerveactionpotentialEMGelectromyogramSIRSsystematicinflammatoryresponsesyndromeAbbreviations2HISTORICALREVIEWIn1955observedapolyneuropathyaftershockorcardiacarrestIn1961described“coma-polyneuropathies”In1971describedapolyneuropathyinpatientswithburnsin1977severepolyneuropathyaboutsepticpatientsHISTORICALREVIEW3

By1983theterm“criticalillnesspolyneuropathy”（CIP）was

appliedRecentlythetermed“criticalillnessmyopathy”（CIM）was

applied

4

StudiesaboutAetiologyvariouslyThevariousfactorsassociatedwiththeSIRSCIPandCIM(Fig.

1)AsimplifieddepictionoftheoreticalmechanismsofdysfunctioninCIPandCIM.(Fig.2)Disorderofmicrocirculation（Fig.3)StudiesaboutAetiologyvari5

AdaptedwithpermissionfromBolton.Figure.1Adaptedwithpermissionfrom6AdaptedwithpermissionfromBolton25.Figure.

2AdaptedwithpermissionfromBo7Figure.3

Schematic,theoreticalpresentationofdisturbancesinthemicrocirculationtovariousorgans,includingbrain,peripheralnerve,andmuscle,inSIRS.Figure.3Schematic,theor8重癥疾病性神經(jīng)肌肉?。ㄓ⑽膒pt課件）9Incidence

50%–70%SIRS20%–50%ICU重癥疾病性神經(jīng)肌肉?。ㄓ⑽膒pt課件）10

?Weaknessoflimbandrespiratorymuscle?Tendonreflexesabsentordecrease?Distallosstopain,temperature,andvibrationClinicalFeaturesClinicalFeatures11ThediagnosticcriteriaforCIPareshowninfollowingTableDiagnosisDiagnosis12

DiagnosticcriteriaforCIPThepatientiscriticallyill(sepsisandmultipleorganfailure,SIRS)DifficultyweaningpatientfromventilatorafternonneuromuscularcausessuchasheartandlungdiseasehavebeenexcludedPossiblelimbweaknessElectrophysiologicevidenceofaxonalmotorandsensorypolyneuropathy

13DeclineintheCMAPamplitudefirstly(Fig.4)DclineintheSNAPamplitudeMotorunitpotentialsmaybereducedinnumberSingle-fiberEMGindicatedysfunctionofterminalmotoraxonsElectrophysiologicFeaturesElectrophysiologicFeatures14

Measurementofcompoundthenarmuscleactionpotentialsattheonsetofsepsis(A)and3weekslater(B).FIG.4

Measurementofcompound15Peripheralaxonaldegeneration.ModeratelossofdorsalrootganglioncellsCentralchromatolysisofanteriorhorncellsNoinflammationintheperipheralnervoussystemMorphologicFeaturesMorphologicFeatures16

MusclebiopsyAcuteandchronicdenervationOccasionalmyopathicchangesMusclebiopsy17

Pathologyofcriticalillnesspolyneuropathy.Thereischromatolysisofanteriorhorncells(A);severeaxonaldegenerationinthiscross-sectionofsuperficialperipheralnerve(B)andlongitudinalsectionofdeepperonealnerve(C);andacuteandchronicdenervationofintercostalmuscle(D)Pathologyofcritical18AxonalvariantsofGuillain–Barre′syndromeDevelopearlierOftenassociatedwithCJinfectionAbnormalcerebralspinalfluidDifferentialDiagnosisDifferentialDiagnosis19

TransientneuromuscularblockadeRepetitivenervestimulationMeasurementofanti-MuSK(musclespecificreceptortyrosinekinase)antibodiesTransientneuromuscularblock20TreatmentofsepsisandmultipleorgandysfunctionsyndromeManagementofdifficultyinweaningfromtheventilatorAttemptsatdirecttreatmentofCIP(stillunproven)PhysiotherapyandrehabilitationTreatmentTreatmentofsepsisandmultip21

TwonewerresearchapproachesarebeingexploredIntensiveinsulintherapyTheadministrationofrecombinanthumanactivatedproteinC

22RecoverydependsonthedistanceRecoveryforweeksinmildcasesandmonthsinseverecasesSlowingofnerveconductionmayhaveapoorprognosisPrognosisRecoverydependsonthedistan23重癥疾病性神經(jīng)肌肉?。ㄓ⑽膒pt課件）24IncidenceAtleastone-thirdofICUpatients（treatedforstatusasthmaticus）In7%ofpatientsaftertransplantationIncidence25

ClinicalFeaturesMajorfeatureisflaccidweaknessTendonreflexesdepressedOphthalmoplegiamaybepresentMyalgiasareuncommonClinicalFeatures26

DiagnosticcriteriaofCIM

●SNAPamplitudes80%ofthelowerlimitofnormal●NeedleEMGwithshort-duration,low-amplitudeMUPswithearlyornormalfullrecruitment,withorwithoutfibrillationpotentials●AbsenceofadecrementalresponseonrepetitivenervestimulationDiagnosisDiagnosis27

●Musclehistopathologicfindingsofmyopathywithmyosinloss●CMAPamplitudes80%ofthelowerlimitofnormalintwoormorenerveswithoutconductionblock●Elevatedserumcreatinekinase(CK）●Demonstrationofmuscleinexcitability*Foradefinitediagnosisofcriticalillnessmyopathy,patientsshouldhaveallofthefirstfivefeatures.

28NerveconductionstudiesLow-amplitudeCMAPsLongdurationCMAPsNormalSNAPsPhrenicnerveconductionnormallatenciesdiaphragmCMAPamplitudesreduceElectrophysiologicFeaturesNerveconductionstudiesElectr29

EMGFibrillationpotentialsandpositivesharpMotorunitpotentialslowamplitudeandshortdurationElectricalinexcitabilitybydirectneedlestimulation

30Featuresofthehistopathologyinthickfilamentmyosinloss(Fig.5)Electronmicroscopyrevealsselectivelossofthick(myosin)filaments(Fig.6)InflammatorychangesareconspicuouslyabsentMorphologicFeaturesFeaturesofthehistopathology31

Figure.5

Musclehistopathologyinacriticallyillpatientwiththickfilamentmyosinloss.(originalmagnification,100)(courtesyofDr.AndrewEngel).

32

Figure.6

ElectronmicroscopyofmuscleinCIM.(originalmagnification,44,000)(courtesyofDr.AndrewEngel).

336.DifferentialDiagnosis

CIPDirectneedlestimulationofthemuscleElectricalinexcitabilityinCIMThereisaresponseinCIP

(Fig.7)Se