gbs inservice presentation
TRANSCRIPT
Prognos'cFactorsEffec'ngLongTermOutcomeinPa'entswithGuillain-Barre
Syndrome
Ka@yCsete,SPTDukeUniversity
Outline
ì Introduc'on
ì Treatmentprocess
ì Prognos'cfactors
ì Rehabilita'onandphysicalac'vity
Introduction
ì Body’simmunesystema@ackspartofthePNS
ì Caneffectanyoneatanyageandbothgenders(male>female)
ì Effectseverybetween1and4in100,000people
ì Prognosiscanincludelongtermeffectofpersistentfa'gueanddisabilitywhileambula'ngeitherwithorwithoutanassis'vedevice.Worstcasescenariosmayresultindeath–rare.
Fullline=menDottedline=women
Causes
ì Nospecificknowncause
ì MostcommonlyoccursfollowingrespiratoryorGIviralinfec'on,rarelyaYersurgeryorvaccina'on
ì Viruschangesnatureofimmunesystemì Recognizecellsasforeignbodiesì Lessdiscrimina'ngonwhichcellsitrecognizesasitsown
Presentation
ì Symmetricalweakness,decreasedsensa'on,painandothersymptomsinlowerextremi'eswhichcanspreadtoupperextremi'esandtrunk
ì Mostseverecasesresultinwholebodyparalysisì Pa'entsputonven'lator
ì Symptomprogressionì Hours,days,orweeksì Reachespointofgreatestweaknesswithin4weeksofonset
DifferentialDiagnosis
ì Tohelpiden'fyGBSì Fairlysymmetricalpresenta'onì Rateofsymptomprogressionvariesgreatlyì DiminishedDTRì NCVcanhelpiden'fyì SpinaltapwillresultinincreasedproteininCSF
DifferentialDiagnosiscont.
ì Acutemyelopathy
ì Vasculi'cneuropathy
ì Myastheniagravis
ì Botulism
ì WestNileencephalomyeli's
ì Lymeneuroborreliosis
ì Tickparalysis
ì Acuteintermi@entporphyria
ì Poliomyeli's
ì Cri'calillnessmyopathyandpolyneuropathy
Burns,TedM.
PEvsIVIgtreatment
ì PEshortensdura'onofdisabilitycomparedtostandardsuppor'vetreatment
ì IVIgpoten'allysaferandmoreconvenientì Notuniversallyacceptedduetonotedcon'nueddisease
progressionorrelapse
ì IVIgimmediatelyfollowingPEtreatmentì Topreventreboundsynthesis
Hughesetal.
Results
ì Majoroutcomecriterion:meandisability-gradeimprovementaYer4weeksì Nosignificantdifferencebetweenthethreegroups
ì Secondaryoutcomecriteria:'metorecovertheabilitytowalkunaided,'mefromrandomiza'ontodiscon'nua'onofven'la'on,meangradeateachfollowupweekì Nosignificantdifferencebetweenthethreegroups
ì Otheroutcomes:median'metohospitaldischarge,median'metoreturntoworkì Nosignificantdifferencebetweenthethreegroups
ProspectiveStudy
ì 108pa'entswerefollowedeverydayinhospital,atday30,atdischarge,6monthsaYerdischarge,1year,and2year
ì Hughesscaleusedtoevaluatepa'entsthroughoutì 0=healthyì 1=minorsignsorsymptomsofneuropathybutcapableof
manualworkì 2=abletowalkwithoutsupportì 3=abletowalkwithsupportofADì 4=confinedtobedorchairboundì 5=requiringassistedven'la'onì 6=dead
Chio,Aetal.
Longtermoutcome
ì At6monthfollow-up:64%hadagoodrecovery(grade</=2)
ì At1yearfollow-up:75%hadagoodrecovery
ì At2yearfollow-up:80%hadagoodrecovery
**7pa'entsdiedwithin30daysofacuteneurologicalsymptomonset,allassociatedwithven'lator-associatedpneumonia
Chio,Aetal.
LongTermOutcomecont.
ì Variablesthatsignificantlyrelatedtopoorrecoveryì Grade>/=3atpeakneurologicdeficits(44%)ì Respiratoryimpairment(40.8%)ì Age>/=50(meanage51.2)ì AxonalormixedEMG(24.2%each)ì Infec'onpriortodiagnosis(58.3%)ì Autonomicinvolvement(22.5%)
ì CVS,sphinctermuscles,respiratorysystemmostaffected
Longtermoutcomeafterventilation
ì 105pa'entswithGBSì 54requiredven'la'on,followedat1yearandmaxrecoveryì 51non-ven'latedpa'entsalsofollowed
ì 1yearfollow-upoftheven'latedpa'ents:ì 52%(28pa'ents)regainedindependentambula'onì 7%(4)s'llven'latedì Remainderofpa'entsrangefrom3-4onHughesscale
ì 1yearfollow-upofnon-ven'latedpa'ents:ì 72%hada2orloweronHughesscale=“goodrecovery”ì 28%hada3-4onHughesscale=“poorrecovery”
Fletcher,Detal.
Follow-upatmaxrecoveryì Outofallpa'ents75%had“good”outcomeand25%had
“poor”outcome
ì Forthosewhorequiredven'la'on..ì 61%(33)hadgoodoutcome,39%(21)hadapooroutcomeì MortalityrelatedGBSaccountedfor20%ì Ofthesurvivors(42):79%(33)regainedindependentambula'on,
14%(6)couldambulatewithassistance,and7%(3)hadseveredisabilityì Allpa'entsleYwithseveredisabilitywereven'latedformorethan
6monthsì Onven'latorforlongerthan4monthsresultedinpooroutcome
Longtermoutcomeafterventilation
ì Predictorsofpoormaximaloutcomeì Olderageì Upperlimbparalysisatpeakdisabilityì Increasedven'la'on'meì Unexcitablenervesonini'alelectrophysiologicstudiesì Delayed'metopeakonset
GeneralCareofGBS
ì Mul'disciplinarycarerequired
ì Monitorpulmonaryfunc'on,autonomicdysfunc'on,infec'onpreven'on,DVT
ì Managementofpain
ì PhysicaltherapyandOccupa'onaltherapy
ì Psychologicalsupport
ConsiderationsinRehabilitation
ì Fa'gue–occursin60-80%pa'ents
ì Approachinthesamewayasotherpa'entswithneurologicaldisease–likelytobeatincreasedriskofcomplica'onssecondarytoimmobiliza'onandweakness
ì Muscleweaknessanddecreasedmobility–ROMtoavoidcontracture&strengtheningexercisesì Avoidoverworkingmusclegroups
ì Sensoryreintegra'onandrepe''veexercisestoimprovecoordina'on
ì Ortho'cstomaximizefunc'onBurns,TedM.
BenefitsofPhysicalActivity
ì 16severelyfa'guedpa'entstookplacein12weekbicycletrainingprogramwith3sessionsperweek
ì Preandpostassessmentof5domains:physicalfitness,self-repor'ngfa'gue,actualmobility,perceivedphysicalfunc'oning,andperceivedmentalfunc'oning
ì Results
Bussmann,JB.etal.
Conclusion
ì Treatmenttrialsmayunderes'matefinaloutcomeiffollow-upislimitedtoshorterdura'ons
ì Educatepa'entsprolongedrecoveryperiod
ì Physicalac'vityisbeneficialtopa'entsonvariousdomains,notonlyimprovingoverallfitnessbutalsomentalfunc'oning
ì Pa'entsbenefitfrommul'disciplinarycare
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