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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