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CRITICALILLNESS
NEUROMYOPATHY
CRITICALILLNESS
NEUROMY1AbbreviationsCIPcriticalillnesspolyneuropathyCIMcriticalillnessmyopathyCMAPcompoundmusclactionpotentialsSNAPsensorynerveactionpotentialEMGelectromyogramSIRSsystematicinflammatoryresponsesyndromeAbbreviations2HISTORICALREVIEWIn1955observedapolyneuropathyaftershockorcardiacarrestIn1961described“coma-polyneuropathies”In1971describedapolyneuropathyinpatientswithburnsin1977severepolyneuropathyaboutsepticpatientsHISTORICALREVIEW3九年級化學全冊第7章應用廣泛的酸堿鹽74九年級化學全冊第7章應用廣泛的酸堿鹽75
AdaptedwithpermissionfromBolton.Figure.1Adaptedwithpermissionfrom6AdaptedwithpermissionfromBolton25.Figure.
2AdaptedwithpermissionfromBo7Figure.3
Schematic,theoreticalpresentationofdisturbancesinthemicrocirculationtovariousorgans,includingbrain,peripheralnerve,andmuscle,inSIRS.Figure.3Schematic,theor8九年級化學全冊第7章應用廣泛的酸堿鹽79Incidence
50%–70%SIRS20%–50%ICU九年級化學全冊第7章應用廣泛的酸堿鹽710
?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
22RecoverydependsonthedistanceRecoveryforweeksinmildcasesandmonthsinseverecasesSlowingofnerveconductionmayhaveapoorprognosisPrognosisRecoverydependsonthedistan23九年級化學全冊第7章應用廣泛的酸堿鹽724IncidenceAtleastone-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)SerumCKMusclebiop
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