Neuro Dr. Nibal

“ Acute Flaccid Paralysis ”

Total Lec: 45

Acute flaccid paralysis

Dr Nebal Waill

Definition

• AcuteFlaccidParalysis(AFP)occurswhenthereisrapidevolutionofmotorweakness(<than4days),withalossoftoneintheparalysedlimb.Thisexcludesweaknessduetotraumaandspasticparalysis.

• AFPisamedicalemergencyasunnecessarydelayscanresultindeathanddisability

• ThelistofunderlyingcausesofAFPisbroad,andthereissubstantialvariationbyage,ethnicity,andgeographicarea.

• Intheabsenceofwildvirus-inducedpoliomyelitis,theacutedemyelinatingformofGuillain-Barresyndrome(AIDP)accountsforatleast50percentofAFPcasesgloballyfollowedinfrequencybyparalyticnon-polioenterovirusinfection,themotoraxonalformofGuillain-Barresyndrome(AMAN),traumaticneuritis,andacutetransversemyelitis.

Background

•1916-Guillian,BarreandStrohldescribed2Frenchsoldierswithmotorweakness,areflexia,and“albuniocytologicaldissociation”inthecerebrospinalfluid.Theyrecognizedtheperipheralnatureoftheillness.

Epidemiology

• AnnualincidenceofGBS=1-3/100000personsannually.• Rareininfants.• Male&femalehavesimilarrisk• Anyagebutmostfrequentat4-9years

GBS subtypes

1. Sporadic AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy)

2. AMSAN (Acute Motor and Sensory Axonal Neuropathy) 3. AMAN (Acute Motor Axonal Neuropathy ) 4. MFS ( Miller Fisher Syndrome )

Pathology

• Bothmotorandsensoryfibersareaffected

• AIDP→segmentaldemyelination

↘axonaldegeneration(lessextensive)

• Segmentaldemyelinationoccursatalllevelsofperipheralnervoussystem

↘Anterior+posteriorroots

Sympatheticchainandganglia

Peripheralnerves

• CNSalterationsaresecondarytoaxonaldegenerationandaffect

↘Anteriorhorncellsinspinalgreymatter

Neuronsofmotorcranialnervesnucleiinbrainstem

Antecedent Events

C.Jujeniaccountsfor1/3ofGBSbecauseofmimicrybetweengangliosidesandlipopolysaccharidesofthebacteria

Clinical features

1. AIDP

• Infection(GIT:Campylobacter,Respiratory:Mycoplasma)within2weeksofonset

• Weakness(lowerextremitiesthenascenduptothetrunkthenupperlimbsandbulbarweakness.

– Maystartinthearmsandmovedownward

– Maybegininthearmsandlegsatthesametime

– Mayoccurinthenervesoftheheadonly

– Inmildcases,weaknessorparalysismaynotoccur

• Thisweaknessissymmytrical(minorsidesdifferencesmayoccure),proximalanddistal

• 9%isasymmetrical

• progressslowlyoverdaysorweeksOrabruptandrapid

• Childbecomesirritable.

• Parasthesiamayoccur,89%painaccompanyweakness

• 50%bulbarinvolvement.

• Facialnerveinvolved.alsoVI,III,XII,IX,X

• Someshowviralmeningitisormeningoencephalitis.

• Papillodema(unexplainedpathogenesis)

• Respiratorymuscules:reducedvitalcapacityCO2retentioneveninabsenceofrespiratorysymptoms

Featuresrequiredfordiagnosis

1. Progressivemotorweaknessofmorethanonelimb.

2. Areflexiaorhyporeflexia(lossofanklejerksanddiminishedkneeandbicepsreflexeswillsufficeifotherfeaturesareconsistentwiththediagnosis.

� Featruessupportiveofdiagnosis

1. Progression:weaknessmaydeveloprapidlybutceasetoprogressafter4wk.Roughly50%willplateauwithin2wks,80%by3wks,and90%by4wks.

2. Relativesymmetry.

3. Mildsensorysymptomsandsigns.

4. Cranialnerveinvolvementslikefacialweaknessdevelopsinabout50%ofpatients.

5. Autonomicdysfunction.

6. Absenceoffeverattheonsetofneurologicalsymptoms.

7. Recoverywithoutspecifictherapy,begins2-4wksafterprogressionceases,occasionallydelayedformonths.

� Featurescastingdoubtonthediagnosis

1. Markedpersistentasymmetryinmotorfunction.

2. persistentbowelorbladderdysfunctionatonsetofsymptoms.

3. Discretesensorylevel.

4. Progressivephaselongerthan4wks.

5. CSFpleocytosis(>50wcc/mm3).

6. Completeophthalmoplegia(internalorexternal).

Clinical Phases

Guillian-barrecanbedividedintofivedistinctclinicalphases:

• Phase1-first24hrfrompresentation

• Phase2-diseaseprogression

• Phase3-plateauphase

• Phase4-initialrecovery

• Phase5-rehabilitation

Dx

1. Clinical

2. CSF

3. Electrophysiologic

4. MRI

Investigation

MRIofthebrainandspinalcord

Shouldbeconsideredinallpatients,usuallydoneif:

1. Thepresentationisacuteorrapidlyprogressive

2. Therearepredominantlysensorysymptoms(includingbackpain)

3. Thereispredominantsphincterdisturbanceofpresentation

4. Thereisaclearsensoryormarkedmotorlevel

Lumberpuncture

• ElevatedCSFproteinwithoutpleocytosisisasupportivediagnosticfinding,howevertheCSFmaybenormalwithinsevendaysofonsetofsymptoms

• Proteinlevel:elevated(>45mg/dl)afterthefirstweekofsymptoms,peak4-5wks

• WCC<10/mm3,occasionallyupto50mm3

• Glucoselevelnormal

Neurophysiology

• NormalnerveconductionstudiesinthefirstweekdoesnotexcludethediagnosisofGBS

• InAIDPnerveconductionimpairment=conductionblock,decreasecompoundactionpotentialamplitude

• SincethemediandurationofexcretionofCampylobacterinstoolsofinfectedpersonsisonly16daysandbecauseofthe1-to3-weeklagtimebetweeninfectionandtheonsetofGBS,manyGBSpatientswithprecedingCampylobacterinfectionmighthavefalselynegativestoolcultures.

• multiplestoolsamples(orrectalswabs)shouldbeobtainedfromGBSpatients

immediatelyuponadmissiontothehospital,preferably3overa3-dayperiod.

Otherinvestigations

• Fullbloodcount,bloodculture(ifpyrexial)

• Ureaandelectrolytes(hypokalemia)

• Creatinekinase(myositis)

• Chestx-ray,ECG

• Abdominalx-ray(palpablebladder,constipation)

Treatment

1. Admission

2. IVIG

3. Plasmaexchange

4. Supportivetreatment

5. Rehabilitation

• SymptomatictreatmentisanessentialpartofthemanagementofGBS.• Childrenshouldadmittedtothepediatricintensivecareunitiftheyhaveone

ormoreofthefollowing:

1. Flaccidtetraparesis

2. Severerapidlyprogressivecourse

3. Reducedvitalcapacityatorbelow20ml/kg

4. Bulbarpalsywithsymptoms

5. Autonomiccardiovascularinstabilitythatispersistenthypertensionorlabilebloodpressureorarrhythmias.

Plasmaexchange

• PlasmapheresishasremainedthegoldstandardtreatmentforGBSoverthelast20years.

• Shouldbeusedwithin4weeksofonsetofneuropaticsymptomsinnon-ambulatorypatients

• Shouldbeusedwithin2weeksofonsetofneuropathicsymptomsinambulatorypatients

• Plasmapheresisisgenerallysafeinchildrenwhoweigh10kgormore.Aseriesofexchangewithacumulativetotalofapproximately250ml/kgvolumeexchangeorroughlyatriplevolumeexchange.

• DisadvantagesofPlasmapheresisincludeitsrarecomplications,suchassepsis,riskofacquiringviralinfectionssuchashepatitisandHIV.

IVIGtreatmenthasadvantagesoverplasmapheresisbecause

• itiseasiertoadminister,• hassignificantlyfewercomplications,• andismorecomfortableforthepatient.

SideeffectsofIVIG

• expandstheplasmavolumesoitmustbeadministeredwithcautioninpatientswithcongestiveheartfailureandrenalinsufficiency

• fever,myalgia,headache,nausea,andvomiting,butthese"influenza-like"symptomsareself-limiting.

• asepticmeningitis,neutropenia,andhypertension

• Anaphylaxis

• Thromboembolicevents

• riskofserioushepatitisCinfectiontransmissionhasbeenreduced

• IVIGshouldbeusedwithin2weeks

• Corticosteroidnotrecommended

• SequentialtreatmentwithPEthenIVIGnotrecommended

• PE&IVIGrecommendedfortheseveredisease

Painmanagement

� Painofdiscomfortispresentin50-80%ofchildrenwithGBSatthetimeofpresentation.

Managingpainby:

• Opioids

• Nonsteroidalanti-inflammatorydrugs(ibuprofen)

• Anti-epilepticdrugs(carbamazepine,gabapentine)

• Tricyclicantiderpessants(amitryptine)

Preventionofpain:

• Airmatresses

• Turningpatientsandcarfulpositioningoflimbs

• Continuationofenteralfeeding,effectiveantacidsasomeprazole

• Preventingconstipation

• Preventandtreaturinaryretention.

Supportivetreatmentdirectedto:

1. Hypertension,hypotension

2. Cardiacarrhythmia

3. Pulmonaryembolism(Prophylaxisfordeepvenousthrombosisshouldbeprovidedbecausepatientsfrequentlyareimmobilizedformanyweeks).

4. Bladderandbowel

5. Psychologicalsupport

6. Nutrition,fluid,electrolytes

7. Pain

8. Skin

9. Cornea

10. Joints

11. Infection

12. communication

prognosis

� 40%→bedbound

� 15%→requireventilation

� 90-95%→completerecoverywithin6-12months

� Remainderambulatorywithminorresidualdeficit

� 4%→mortalityrate

• AntecedentsC.jujeniinfectioncorrelateswithpoorPx

Causesofdeath:

1. Autonomic(bradycardia,tachycardia,hypertension)

2. Respiratoryfailure

3. Pulmonaryembolism

4. Complicationofventilation

5. Cardiovascularcollapse

Chronicrelapsingorchronicunremitting(7%)

Featuressuggestiverelapsingare:

1. Severelyweak

2. Flaccidtetraplagia

3. Bulbarandrespiratorymuscleinvolvement

� Oneormorerelapsesover2mo.-years=CIDP

CongenitalGBS

� Weakness,areflexia,hypotonia.

� CSFandelectrophysicalstudiessuggestiveofGBS.

� Notreatment,gradualimprovement.

PoliomyelitisPolioviruses

• RNAviruses,Picornaviridaefamily,enterovirus

• 3geneticallydistinctserotypes

• SpreadfromintestinaltracttoCNS

• 90–95%inapperantinfections

• Transmission:humanistheonlyreservoir

• Fecal–oralroute

• Isolatedfromstoolfor2weeksbeforeparalysistoseveralweeksafteronsetofsymptoms

Pathogenesis

• Wildtypeandvaccinestrains

• GainhostentrythroughGIT

• Passtotheregionallymphnodes

• Goestothebloodcausingviremia

• WildtypeaccesstheCNSthroughperipheralnerves

• Incubationperiod8-12days

Clinical manifestions

Wildtypefollowoneofthefollowingcourses:

1. 90-95%inapparentinfection(nodisease&nosequelae

2. 5%inabortivedisease(influenza–likesyndrome1-2wkafterinfection,fever,malaise,anorexia,headache+/-vomiting)thenrecoverycomplete

3. Non-paralyticpoliomyelitis• 1%• Signsofabortivetype,fleetingparalysisofbladderandconstipation.• Thisisfirstphase(minor)thensymptoms-freeperiodthenmajorphase• O/E:nuchalrigidity,changesinthedeepandsuperficialreflexes

(impendingparalysis).Nosensorydefects4. Paralyticpoliomyelitis

• 0.1%• Spinaltype:majorphase,sensory(paresthesia,hypersthesia),motor(

fasiculationandspasms)progressto• Asymmetricparalysisofoneleg,then1arm• DTRinitiallyactivethendiminishedandabsent• Variablecourse:someprogress,somerecover

5. Bulbartype• +/-spinalcordinvolvement• Nasalvoiceorcry• Difficultyinswallowing• Accumulatedpharyngealsecretion• Absenceofeffectivecoughing• Nasalregurgitation• Deviationofpalate,uvula,tongue• Involvementofvitalcentersinthemedulla• Paralysisofvocalcords…hoarseness,aphonia• Sometimesculminateintoascendingparalysis(Landrytype)

6. polioencephalitis• Rare• Seizures,coma,spasticparalsysis,increasedreflexes• Respiratoryinsufficiency

7. Paralyticpoliowithrespiratoryinsufficiency• Anxiousexpression• Inabilitytospeakwithoutfrequentpauses• IncreasedRR• Movementofalanasi,accessorymuscles• Inabilitytocoughorsniff

• Paradoxicalabdominalmovement• Relativeimmobilityofintercostalspace

Diagnosis

• Shouldbeconsideredinanyunimmunizedorincompletelyimmunizedchildwithparalyticdisease

• Oranychildwithparalyticdiseaseoccurring7-14daysafterreceivingtheoralvaccine

• Stool:Isolatethevirusin2stoolspecimencollectedwith24–48hrapart

• Canisolatepoliovirusin80–90%inthefirstweekandlessthan20%within3-4wk

• CSF:normalinminordisease

• Cells20-200/mm3initiallythenreduced

• Protein:increasetoreach50-100mg/dlby2ndweek

• CSFserology:seroconversionor4foldsriseinantibodytiters

Treatment

• Nospecifictreatment

• Supportive:Limitprogression,preventskeletaldeformities,preparechildandfamilyforprolongedtreatment

Abortivepoliomyelitis

• Analgesics,sedatives

• Attractivediet

• Bedrestuntiltemperaturenormalize

• Avoidanceofexertionforensuing2wks

• Carefulneurologicandmusculoskeletalexamination

Nonparalyticpoliomyelitis

• Sameasabortive

• Reliefmuscletightness

• Analgesics

• Hotpacksfor15-30minevery2-4hr

• Hottubbaths

• Firmbed

• Footboardorsplinttokeepfeetatrightangletolegs

• Latergentlephysicaltherapy

Paralyticpoliomyelitis

• Hospitalization

• Physicalrest

• Suitablebodyalignmenttopreventdeformity

• Changepositionevery3-6hr

• Activeandpassivemovementindicatedaspaindisappear

• Moisthotpacks

• Opiatesandsedatives

• Treatconstipation

• Parasympatheticstimulantforbladderparalysis

• Adequatedietaryandfluidintake

• Orthopedist,physiatristshouldseethem

Bulbar

• Maintainairway

• Avoidriskofinhalation

• Gravitydrainageofaccumulatedsaliva

• Nursedinlateralorsemi-proneposition

• Aspiratorswithrigidtipsfororalpharyngealsecretionorflexiblecatheterfornasopharyngeal

• Fluidandelectrolytesequilibrium

• Bloodpressuretakenatleasttwice

• Impairedventilationsignsshouldbenoticedtodecidetracheostomy

Prognosis

• Inapparent,abortiveandasepticmeningitis=goodoutcome

• Paralytic=dependsontheextentandseverityofCNSinvolvement,recoveryphaselast6months

• Severebulbar=MR60%

• 30–40%ofpersonssurvivedparalyticpolio…mayexperiencemusclepainandexacerbationofexisitingweaknessafter30-40yr

Prevention

• Vaccination

• Hygienicmearures

Thank you