brain-degenerative: intracranial hypertension, hypotension and … hyper... · 16/04/18 4 • csf...

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Brain-Degenerative:Intracranialhypertension,hypotensionandCSF-leaks

PedroVilelaLisbon-Portugal

Summary

Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks

•  CSFpressurediseases:

•  Background•  EtiologyandPhysiopathology•  Incidence;prevalence;clinicalpresentation•  Imagingfindings•  Treatment

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Backgrou

nd

•  CSFphysiologyandpressuremeasurements:

•  CSFvolume–  Production:0.35-0.6mL/min(500-600mL/day)

–  Volume:160mL»  ~25%àventricles»  ~75%àsubarachnoidspace(intracranialandspinal)

•  CSFpressure–  SimilartotheICP(intracranialpressure)–  Fluctuates(includingduringtheday)

»  Monro-KelliedoctrineCSFandCBVhavereciprocalchangesinordertomaintainanormalintracranialpressure(ICP)

–  Varies:60-250mmH2O(280mmH2Oinchildren)


Backgrou

nd


•  CSFvolume–  Production:0.35-0.6mL/min(500-600mL/day)

–  Volume:160mL»  ~25%àventricles»  ~75%àsubarachnoidspace(intracranialandspinal)

•  CSFpressure–  SimilartotheICP(intracranialpressure)–  Fluctuates(includingduringtheday)

»  Monro-KelliedoctrineCSFandCBVhavereciprocalchangesinordertomaintainanormalintracranialpressure(ICP)

–  Varies:60-250mmH2O(280mmH2Oinchildren)


ChangesontheCSFvolumewill

induceCBVvariationatthe

venousside(sincetheveinsaremoreelastic/compressible

andhavelowerpressure

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Backgrou

nd

•  CSFopeningpressure

–  Hypertension:>250mmH2O(children>280mmH2O)

–  Hypotension:<60mmH2O(horizontal

orlateraldecubitus;sitting)

»  Aliquorrhoea(negativeornon-measurablepressure)

»  SittingthepatientthereisanincreaseoftheCSFpressureinnormalpatientsàinintracranialhypotensiontheCSFpressuremaintains<60mmH2O


From:LancetNeurol2015;14:655–68


Backgrou

nd


FromCapelCetalT.Heldt(ed.),IntracranialPressure&NeuromonitoringXVI,ActaNeurochirurgicaSupplement,Vol.126,2018

Arterialflow

Venousflow

ArterioVenous(arterial+venous)

flow

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Backgrou

nd



Arterialflow

Venousflow


flow


flow

CSFflow


Backgrou

nd



Arterialflow

Venousflow

CSFandCerebralflowarecloselyrelated:

CSFflowsecondarily(andpassively)duetocerebralbloodvolumechanges

Systole

àincreaseinflowduetoarterialflowà  Notimmediately

compensatedbyvenous(out)flow(isdelayed90-100ms)

à  CompensatedbyimmediateCSFoutflow

CSFflow

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Backgrou

nd

•  IntracranialCSFpressurechanges:

•  Types: Low-pressure/High-pressure

•  Clinicalpresentation: Headaches

•  Causes: IdiopathicorSymptomatic(secondary)

–  Idiopathicintracranialhypotension àCSFleak–  Idiopathicintracranialhypertensionà(?)

•  Ifuntreatedàseverecomplications:–  blindness (CSFhypertension) –  coma (CSFhypoandhypertension)


Summary

•  Intracranialhypertension:



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Etiology


•  Causes:

•  Hydrocephalus

•  Intracranialmass

•  Brain“oedema”;toxic;inflammatory;infectiouslesions

•  Venousoutflowobstruction

•  IdiopathicFrom:LancetNeurol2015;14:655–68

ICHypertension


Diagno

sticcriteria

•  IdiopathicIntracranialhypertension(IIH):–  Pseudotumorcerebri –  Benignintracranialhypertension (...notalwaysbenign...)

•  Definition:–  ChronicelevationofICP

»  Headache+Papilledema+nolocalizingneurologicsigns+normalCSFcomposition

»  Nodefinablecause(nosecondarycauseofICPincrease)hydrocephalus,hypertensiveencephalopathy/PRESS,masslesion,traumaticbraininjury,infection,ischemicstroke,venousoutflowobstruction,venousthrombosis,metabolic/drugs(hypoxemia,hypercapnia,hypervitaminosisA,acuteliverfailure,...),fever,seizures

•  Imaging:R/Osecondarycausesofintracranialhypertension


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Diagno

sticcriteria

•  IdiopathicIntracranialhypertension(IIH):


FromFriedmanDetalNeurology2013

Diagno

sticcriteria

•  IdiopathicIntracranialhypertension(IIH):–  Diagnosticcriteria

HeadachePapilledemaHighCSFopeningpressureCSFnormal



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Diagno

sticcriteria



FromFriedmanDetalNeurology2013

Reviseddiagnosticcriteriaforthepseudotumorcerebrisyndromeinadultsandchildren2013

Etiology


•  Hypotheses»  Increasebrainwatercontent(andincreasebrainvolume)»  ExcessCSFproduction»  ReducedCSFabsorption»  Increasedintraabdominal/thoracicpressure(obesity)»  Increasevenouspressure

•  Venousstenosis(seemstobesecondarytoincreasedICP)

»  Inflammatory/Immunologic•  somecasesCSF:presenceofoligoclonalbands;increasecytokines

»  Cellular/moleculardysfunction:•  Dysregulationofaquaporin1and4receptors•  Dysregulationofmitochondria•  Dysregulationofsignalingleptins


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Summary




Prevalen

ce

•  IdiopathicIntracranialhypertension(IIH):•  Prevalence

–  Community-based:1per100000

–  Gender:adultsF>M(5-15:1)(men10%ofcases)»  Nogenderpredominanceinprepubertalchildren

–  Allages(pediatricàelderly):Peak3rddecadeoflife(20-42y/o)

–  Increasedprevalence»  Female»  Obesity(highBMI)–present>70%ofcases

•  BMIcorrelateswithCSFopeningpressure(?increasedintra-abdominal/thoracicpressure)

»  Sleepapnea»  VitaminAtoxicity»  Withdrawalofsteroidtherapy


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Clinicalpresentation

–  Clinicalpresentation

•  Severeheadache(>90%ofcases)–  Bilateral(mimicsmigraine)

»  Constantorfluctuating»  Aggravates:Valsalvamaneuver,coughing,bendingoveror

lying(increasestheintacranialpressure)

•  Visualcomplains(~50-75%)–  BilateralPapilloedema

»  blurredvision,photopsia,ortransientvisualobscuration»  absentinrarecases(speciallyinmalegender)–IIHwithout

papilloedemaàmorefrequentlyassociatedwithduralsinusstenosis

–  Opticnerveatrophy(bilateral)»  associatedtolongstandingpapilloedema

•  Otherassociatedsymptoms:»  Diplopia(unilateralorbilateralVIcranialnervepalsy)»  Pulsatiletinnitus»  Nausea,vomiting(~50%)»  Lesscommon:neckorbackpain,depression,mildcognitive

impairment


R

L


Summary




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Imagingfin

dings

–  Imagingfindings(MRI)–  Duralsinus(transversesinus)stenosis–

superiorto50%-(~93-97%)–  Eye

»  Posteriorglobeflattening-(~45-98%)»  Distentionoftheperiopticsubarachnoidspace–

nervesheath>5.5-6mm(~45-89%)»  Protrusionoftheopticpapillae(~3-59%)»  Enhancement/BrightDWIspotattheopticnerve

head(~2–50%)

–  Empty(partiallyempty)sella(~80-83%)»  protrusionofthesubarachnoidspaceinthecavum

sellae

–  Other»  “Meningocele”andenlargementofMeckelcave,

foramenovale,»  “Slit”ventricles»  Tonsillarherniation


fromHartmannAJPetalJCN2016

fromDucrusAetalLancetN2015

Imagingfin

dings





head(~2–50%)


sellae

–  Other»  “Meningocele”andenlargmentofMeckelcave,



fromTawfikKetalOto&N2017

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Imagingfin

dings





head(~2–50%)


sellae

–  Other»  “Meningocele”andenlargmentofMeckelcave,



fromHartmannAJPetalJCN2016

Imagingfin

dings

•  IdiopathicIntracranialhypertension(IIH):•  Duralsinusstenosis–mostfrequentimagingsign-

–  Duralsinusstenosisispresentin>90%ofcases(93–97%ofpatients)•  MorrisP2018:IHH94% Controls:3%•  FarbR2003: IHH93% Controls:6.7%

Controversial:causeorconsequenceofIIH(viciouscycle)•  Physiopathology:duralsinusstenosis/thrombosisàimpairedvenousoutflowà

reducedCSFabsorptionàincreasedICP•  Duralsinus“stenosis”andhypoplasia(>50%ofduralsinuslengthwithreduceddiameter)

arefrequentinasymptomaticpopulation•  IIHpatientshowreversibilityofTSS“stenosis”afterlumbarCSFdrainage(functional

stenosis)•  Nocorrelation:TSSgradeandopeningCSFpressure

TSS–transversesinusstenosis(morecommon)•  morecommonattheTS/SSjunction•  commonlybilateralstenosis(~85%ofpatients)•  triangularshape(inoppositiontotheroundsinusshape)


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dings



•  Duralsinusstenosis

QualitativeScore(FarbR2003)

Quantitative(CAD-ZurD2017):higherdifference(IIH/C)onminimalcrosssectionarea

FromFarbRetalNeurology2003

Classification:0/GAP:discontinuity(gap)oraplasticsegment;1/<25%:hypoplasiaorseverestenosiswithinasegmentoftheconduitestimatedaslessthan25%ofthecrosssectionaldiameterofthelumenofthedistalsuperiorsagittalsinus;2/25-50%:moderatelystenosedsegmentoftheconduit

(25–50%)3/50-75%:mildlynarrowedsegment(50–75%)

4/>75%:nosignificantnarrowingseen(75–100%)CombinedConduitScore(CCS)=sumofthe

lowestscores

CCS4

CCS3

CCS1

Imagingfin

dings






FromFarbRetalNeurology2003

1

0

2

4

12

1 1

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Imagingfin

dings






FromZuretalBrainandbehavior2017

Summary


•  Background•  Physiopathology•  Incidence;prevalence;clinicalpresentation•  Imagingfindings•  Treatment


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Treatm

ent

•  IdiopathicIntracranialhypertension(IIH):–  Treatment

1stline

Conservativetreatmentand

medicaltreatment

•  Weightloss(~0.5Kg/week)•  Diet:fluidrestrictionandalow-sodium

•  Acetazolamide•  carbonic anhydrase inhibitorà reduces CSF production and

ICP•  1–4gperday(in2-3doses)

•  Topiramate•  weakcarbonicanhydrase inhibitoràreducesCSFproduction

andICP•  20–120mgperday

•  Noindicationforsteroids


Treatm

ent


2stline

Refractorycases

•  Bariatricsurgery•  weight-reductionsurgery•  morbid obesity patients with failure weight-reduction with diet and

exercise

•  CSFshuntingprocedures•  ventricular / lumbar CSF derivation (lumbar / ventricular peritoneal

shunt)•  refractorypatientswithsevereheadaches

•  Opticnervesheathfenestration•  compressionofopticnervebycreationofawindow into thedural sheath,

donebilaterallyorsequentially(theothereyemightimproveafterthefirsteyeisoperatedon)

•  refractorypatientswithseverevisual(andmildheadaches)

•  EndovascularvenousPTA&Stenting•  PTA & Stenting of a stenotic venous sinus: bilateral or unilateral (in

casesthathaveacontralateralhypoplasicsinus)•  refractory patients with non-reversible (after lumbar puncture)

transverse sinus stenosis; and/or refractory to other surgicaltreatments


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Treatm

ent


Emergent

Preventvisualloss!!!

•  Papilloedema + severe headache + notsevere visual lossà ventricular / lumbarCSFshunting

•  Papilloedema + severe visual lossà opticnervesheathfenestration


Treatm

ent

•  IdiopathicIntracranialhypertension(IIH):•  Endovasculartreatment:

–  1stpublishedcase:Higgins2002

–  Objectives:évenousoutflowàêvenouspressureàéCSFoutflowandabsorption

–  Indications:

»  IHHwithrefractorypersistent/progressivepapilledema•  Underconservativetreatmentdiet+carbonicanhydraseinhibitors(ornon-

compliantorseveresideeffectswithmedicaltherapy)•  Afterfailedsurgicalprocedures

»  Pressuregradient•  Pressuregradient>5mmHgfavorableforstenting(absenceofpressure

gradientisnotacontraindicationforstenting•  Generalanesthesiamaymaceratethepressuregradient(evaluatedon

awake/conscioussedationprocedures)


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Treatm

ent

•  IdiopathicIntracranialhypertension(IIH):•  Endovasculartreatment:

–  Data»  Pufferetal2013(metaanalysis):143patients»  Aguilar-PérezMetal2017(caseseries):51patients

•  PTA&Stenting•  Headache improvement/resolution~84-88% failure~12-16%•  Papilledema improvement/resolution~88-97% failure~3-12%

•  Visualsymptoms: improvement/resolution~82-87% failure~13-18%

•  Longtermfollowup àretreatment~12%•  instentstenosis:~10%•  denovostenosis:14%




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Summary

•  Intracranialhypotension:



Etiology


•  Causes:

•  Hypovolemia

•  Overshunting

•  CSFleaks(traumaticand“spontaneous”)


ICHypotension


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Etiology


•  Cause:CSFleak–  Traumatic(morecommontype)–96%

»  Locationàskullbase»  80%trauma;16%iatrogenic/postsurgical

–  Spontaneous–4%»  Locationàoftenatspinelevel;rarelyfromskullbase

•  Morecommon:cervico-thoracicjunction;thoracic»  Cause:unknown

•  Duralweakness:duralattenuation;duraltears;meningealdiverticula•  VentralCSFleaksàspinaldegenerativediseases•  Connectivetissuediseases(MarfanS;Ehlers-DanlosS)


Physiopa

thology


•  Physiopathology: CSFleak

LossofCSFVolume

NegativeintracranialpressureVolumecompensation:éBloodVol

Monroe-Kelliehypothesis:constantintracranialvolume(blood+CSF+brain)

ExponentialrelationshipVOLUME–PRESSUREThewithdrawalofapproximately10%oftheCSFvolumeà

cause>40%decreaseinthe(alreadynegative)vertexCSFpressure


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Physiopa

thology

Negativeintracranialpressure

Descent(sagging)ofthebrain

Tractiononpain

sensitivestructures

Venousrupture

Tractionofnerveroots/

cranialnerves

SubduralhematomasPain Nerve

palsies

Encephalopathy;Lossofconsciousness;Coma


Negativeintracranialpressure

Dilatationofpain-

sensitivevenous

structures

Physiopa

thology

Pain

Pituitaryhyperemia


Tractiononpain

sensitivestructures

Venousrupture

SubduralhematomasPain

VolumecompensationMonroe-Kelliehypothesis:constant

intracranialvolume(blood+CSF+brain)

Increaseofthevascularcomponent

VenousDilatation(intracranial&spinal)

DurahasnoBBB

Subduralcollections

Increasedvenouspressure

Extravasationoffluidthroughtheinnermost

durallayer


cranialnerves

Nervepalsies

Encephalopathy;Lossofconsciousness;Coma


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Summary




Prevalen

ce

•  Intracranialhypotension:LowCSFvolumeheadaches

•  NotprimaryCSFlowpressurebutCSFlowvolume•  Otherterms:"CSFhypovolemia”;"CSFvolumedepletion”;"spontaneousCSFleaks”

•  Prevalence–  Community-based:2-5per100000(morecommonthanIdiopathic

IntracranialHypertension)–  Emergencyserviceannualincidence5per100000(~SAH)–  Probablyunderdiagnosed

–  Gender:F>M(2:1)–  Allages(pediatricàelderly):Peak4thdecadeoflife(35-42y/o)


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Clinicalpresentation

•  Intracranialhypotension:–  Clinicalpresentation

•  Severeheadache–  BilateralandOrthostatic

»  Starts~15minaftersupinepositionandrelievedafter~15-30minofrecumbence»  sometimesprecededbycervical/interscapularpain;overtimetheaggravationwith

orthostaticpositionmaybecomelessevident–  Aggravates:Valsalvamaneuver(increasestheCSFoutflow)

–  Thunderclapheadache(15%ofcases)–  Notalwaysbilateralandorthostatic:persistent,pulsatile,ect…headaches…even

paradoxicalposturalheadachesometimesmaybeencountered(headachespresentinrecumbenceandrelievedinanuprightposition)

•  Otherassociatedsymptoms(orthostaticinnature):nausea,vomiting,anorexia,neckpain,dizziness,changesinhearing,galactorrhea,facialnumbnessorweakness,orradicularsymptomsinvolvingtheupperlimb

•  Severesymptoms(rare):–  Encephalopathy;Lossofconsciousness;ComaRequireemergenttreatmentàintratechalSaline


Complications

•  Intracranialhypotension:•  IntracranialHypotensioncomplications:

–  RecurrenceofIChypotensionepisodes–  Evolutiontochronicity:chronic(IChypotension)headache–  Other:

»  Venousthrombosis(~1%ofcases)»  Subduralhematomasàbraincompression(unpredictableevolution,and

mayacutelyincreaseinsize)»  Cerebralinfarction(veryrare)

•  CSFleakscomplications:–  Spinalleak:“No”riskformeningitis(complication)–CSFisabsorbedby

theepiduralvenousplexus–  Skullbaseleak:riskformeningitis


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Diagno

sticcriteria

•  Intracranialhypotension:–  Diagnosticcriteria

HeadacheSigns/symptomsofdecreaseICpressureLowCSFopeningpressureImprovementafterepiduralbloodpatchAbnormalMRIfindingsCSFleakdemonstration



Summary




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Investigation

•  Intracranialhypotension:•  Imagingevaluation:

–  1stlinestudies:»  BrainorBrain&Spineevaluation:MRIissuperiortoCT»  CSFleakstudy:noninvasivemethod(MRI)

–  2ndlinestudies:invasivestudiesforrefractorycases(patientsthatfailed2or3–non-oriented-lumbarepiduralpatches)

•  Imagingfindings:–  Primary:CSFleakdetection

»  myeloCT»  myeloMR»  Othermethods:Myelo-DSA;Nuclearmedicinestudies

–  Secondary:intracranialandspinalchanges


Investigation

•  Intracranialhypotension:•  Imagingevaluation:

–  1stlinestudies:»  BrainorBrain&Spineevaluation:MRIissuperiortoCT»  CSFleakstudy:noninvasivemethod(MRI)

–  2ndlinestudies:invasivestudiesforrefractorycases(patientsthatfailed2or3–non-oriented-lumbarepiduralpatches)

•  Imagingfindings:–  Primary:CSFleakdetection

»  myeloCT»  myeloMR»  Othermethods:myelo-DSA;nuclearmedicinestudies

–  Secondary:intracranialandspinalchanges


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Imagingfin

dings


•  PrimaryImagingfindingsàdetectionoftheCSFleak(s)

–  Myelography(Myelo-CT)»  Delayedacquisitions(3-4h)forslow/lowvolumeleaks»  Rapidacquisitionsforfast/highvolumeleaksàDSA

–  Myelo-MRI»  Withoutcontrast»  Withintratecalgadolinium

–  Radionuclidemyelo/cisternography


Imagingfin

dings:CT/MRI

•  Intracranialhypotension:•  PrimaryImagingfindingsàdetectionoftheCSFleak(s)

–  Myelography(Myelo-CT)»  Delayedacquisitionsforslow/lowvolumeleaks»  Rapidacquisitionsforfast/highvolumeleaksàDSA


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Imagingfin

dings:CT/MRI

•  Intracranialhypotension:•  PrimaryImagingfindingsàdetectionoftheCSFleak(s)

–  Myelo-MR»  Withoutcontrast»  Withintratecalgadolinium(“offlabel”)


Imagingfin

dings:CT/MRI

•  Intracranialhypotension:–  IntrathecalgadoliniumMRImyelography

•  ITinjectionofGdcontrastisanoff-labeluse•  Reservedforhighlyselectedpatients

–  substantiallysymptomatic–  havehighclinicalsuspicionofCSFleak–  havedemonstratednoleakonNMC/CTM

FatsatcoronalT1afterICgadolinium(1ccofgadobutrolum)AlbesGetal.DetectionandtreatmentofspinalCSFleaksinidiopathicintracranialhypotension.Neuroradiology2012


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Imagingfin

dings:CT/MRI

•  Intracranialhypotension:–  Causes

osteophyte

Duraldiverticulum


Imagingfin

dings:NuclearM

edicine

•  Intracranialhypotension:Leakidentification–  NuclearMedicine(radioisotope)Cisternography

•  IVinjectionofIndium-111bylumbarpunctureàserialscanningduring 24-48hours–  DirectSign:CSFleakdemonstration–  IndirectSigns:

»  Paucityofradioisotopeatcerebralconvexities:24hànormally-isotopeprogressesintothecerebralconvexitieswhereitisabsorbedbythearachnoidvilli(inaCSFleakthereisnopassagetothecerebralconvexitiesthatleadstoadeficiencyofisotopeàmostcommonsign

»  Earlypassage(<4hcomparedto6-24h)tourinarybladder/kidneysoftheradioisotope

•  Drawbacks:–  variablesensitivity (60–90%);–  Falsenegatives:noactiveleak/leaksmallerthanimageresolution–  Lowspatialresolution/Multiplescans/Durationofthestudy–  Patientcomplianceandcollaboration(difficultinoutpatients)


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Imagingfin

dings:NuclearM

edicine

•  Intracranialhypotension:–  NuclearMedicine(radioisotope)Cisternography

CSFleakidentification

Indirectsign:earlypassagetourinarybladder/kidneysoftheradioisotope

Indirectsign:absence/paucityofradioisotopeoverthecerebralconvexities


Imagingfin

dings:CT/MRI


•  SecondaryImagingfindingsàbrainandspinalchangesinducedbytheCSFleak(s)–CSFlowvolume/pressure

–  Intracranialfindings

–  Spinalfindings


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Imagingfin

dings:CT/MRI

•  Intracranialhypotension:– Secondaryimagingfindings(SEEPS)–  5majorfindings:

•  Saggingofthebrain à~50–72%•  Subduralfluidcollections à~15–72%

–  Effusions“Hygromas”(common);hematomas

•  DiffuseduralmeningealEnhancement/thickening à~85%•  VenousEngorgement•  Pituitaryhyperemia

– MRImagingmaybenormalàupto20%ofcasesAbsenceofCT/MRimagingintracranialhypotensionfindings

doesnotexcludethediagnosis!


Imagingfin

dings:CT/MRI Negativeintracranialpressure


Tractiononpain

sensitivestructures

Venousrupture



cranialnerves

Nervepalsies

•  Effacementofperichiasmaticprepontinecisterns

•  Pituitarystalkandponsflattening•  Bowingoftheopticchiasm•  Cerebellartonsilsdescend


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Imagingfin

dings:CT/MRI Negativeintracranialpressure

Dilatationofpain-

sensitivevenous

structures

VolumecompensationMonroe-Kelliehypothesis:constant

intracranialvolume(blood+CSF+brain)

Increaseofthevascularcomponent

Pain

Pituitaryhyperemia


Tractiononpain

sensitivestructures

Venousrupture


Subduralcollections

VenousDilation(intracranial&spinal)

Increasedvenouspressure

Extravasationoffluidthroughtheinnermost

durallayer


cranialnerves

Nervepalsies

meningealvesselsàcontrast

enhancement

•  Effacementofperichiasmaticprepontinecisterns

•  Pituitarystalkandponsflattening•  Bowingoftheopticchiasm•  Cerebellartonsilsdescend


Imagingfin

dings:CT/MRI

•  Intracranialhypotension:–  SecondaryimagingfindingsàCT/MRI

•  SubduralFluidCollections/Hematomas à15-50%–  Type:Effusions(70-90%);Hematomas(10-30%)àdifferentetiology

•  Effusions/“Hygromas”àSUBDURALFLUIDCOLLECTIONS–  Causedbythepresenceofapressuregradient–  Subduraleffusionsaretypicallythin,crescentic,andlocatedeitherbeloworbetweenenhancing

membranes–  Generallyarenotseenintheabsenceofduralenhancement–  Maybebilateralandareusuallywithoutmasseffect–  Resolvewithindays-weeks

•  Hematomas–  CausedbytheruptureofthebridgingveinsastheCSFvolumedecreasesandthebrainsags,

pullingawayfromthedura–  Typicallyhavemasseffect–  ResolvewiththecorrectionoftheSIH(requirelongerfollowup–upto3months)


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Imagingfin

dings:CT/MRI

Subduralfluidcollections


Imagingfin

dings:CT/MRI

•  Intracranialhypotension:–  Clinicalpresentation

from:TanaCetal;Neurology.2014TeachingNeuroImages:Adangerouscomplicationofspontaneousintracranialhypotension.

SubduralhematomasSubduralfluidcollections


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Imagingfin

dings:CT/MRI


•  Diffuseduralthickening/enhancement(pachymeninges)à85%–  Diffuse/crescent-shapedduralthickening

»  ThinandLinear–notnodular»  Noleptomeningealinvolvement»  Supraandinfratentorial

–  IsointensetobrainonT1&HyperintensetobrainonT2/FLAIR–  Diffuseintensecontrast-enhancement


Imagingfin

dings:CT/MRI


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Imagingfin

dings:CT/MRI


•  Braindownwarddisplacement à40-50%–  Saggingofthemidbrain(descendbelowthelevelofthedorsumsellae)–  Reductionofthecerebralpeduncles/ponsangle–  Caudaldisplacementofthecerebellartonsils(25-75%)–  Temporallobesherniationthoughttheincisura


Imagingfin

dings:CT/MRI


•  Braindownwarddisplacement à40-50%–  Saggingofthemidbrain(descendbelowthelevelofthedorsumsellae)–  Reductionofthecerebralpeduncles/ponsangle–  Caudaldisplacementofthecerebellartonsils(25-75%)–  Temporallobesherniationthoughttheincisura

SIH Posttreatment


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Imagingfin

dings:CT/MRI


•  Other–  Engorgementofthevenousstructures(cavernoussinus;otherduralsinus;corticalveins;spinalveins–epiduralplexuses)


PosttreatmentSIH

Imagingfin

dings:CT/MRI


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Imagingfin

dings:CT/MRI


•  Other–  Sellaturcica:Enlargementofthepituitarygland(convexsuperiorborder)–reversiblepituitaryhyperemia

PosttreatmentSIH


Imagingfin

dings:CT/MRI

•  Intracranialhypotension:SpinalFindings•  Extra-arachnoidfluidcollections

Causeunknown:directleakageofCSFintotheepiduralspace/effusion/transudationofintravascularfluidfromhyperemicmeningesintoepidural/subduralspaceNonfocal,extendingovermultiplespinallevels

•  DuralthickeningandenhancementFromduralvasodilationandengorgement

•  Dilationoftheanteriorepiduralveins/venousplexus


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Imagingfin

dings:CT/MRI


Imagingfin

dings:CT/MRI

07-2009 10-2010


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Summary


•  Background•  Physiopathology•  Incidence;prevalence;clinicalpresentation•  Imagingfindings•  Treatment


Treatm

ent

•  Intracranialhypotension:–  Treatment

1stline(withoutidentified

CSFleak)

Symptomatictreatment:Bedrest;oralhydration

NSAI?Theophylline?

?Caffeine(increaseCSFproduction)??Compressiveabdominalbinders?

?VitaminA?

Positiveresults

~15-30%ofcases

Refractoryafter~1week

2ndlineEpiduralbloodpatch

(nontarget)

Positiveresults

~70%ofcases


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Treatm

ent

•  Intracranialhypotension:–  Treatment:EpiduralBloodPatch

•  Method:–  Patient:decubitusorTrendelenburgposition–  Autologousbloodinjectionattheepiduralspace

»  Location:•  Singleà lumbar•  Doubleà lowerlumbarandthoracolumbarjunction

»  Volume:10-80mLdependingonthesizeofepiduralspace•  1stà lowvolume(10-20mL)•  2ndà highervolume(>20mL)

–  Followedbybedrestduring24hindecubitus

•  Complications:–  Vasovagalsyncope;lumbarpain,hypertensionheadaches(rebound),arachnoiditis,asepticmeningitis,infection


Treatm

ent


2ndlineEpiduralbloodpatch

Refractoryafter~5days

2ndlineRepeatedEpiduralbloodpatch-nontarget(2to3timeswithanintervalof5days)

Refractoryafter~5days

Othertherapeuticoptions:precisesiteoftheCSKleakTargetBloodpatch/Fibrinsealant/Surgery

3rdline


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Treatm

ent





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References

•  Intracranialhypotension(selectedreferences)•  HeadachearisingfromidiopathicchangesinCSFpressure.DucrosA,BiousseV.LancetNeurol.2015Jun;14(6):

655-68.•  High-pressureheadaches,low-pressuresyndromes,andCSFleaks:diagnosisandmanagement.Graff-RadfordSB,

SchievinkWI.Headache.2014Feb;54(2):394-401•  MokriB.SpontaneousCSFleaks:lowCSFvolumesyndromes.NeurolClin.2014May;32(2):397-422.•  SpontaneousLowPressure,LowCSFVolumeHeadachesSpontaneousCSFLeaks.Mokri,Betal;

Headache.2013;53(7):1034-1053.•  SpontaneousSpinalCerebrospinalFluidLeaksandIntracranialHypotension.Schievink,WJAMA2006•  IntracranialHypotensionSyndrome:AComprehensiveReview.Paldino,Metal;NeurosurgFocus.2003•  YuhEL,DillonWP.Intracranialhypotensionandintracranialhypertension.NeuroimagingClinNAm.2010Nov;

20(4):597-617.


References

•  Intracranialhypertension(selectedreferences)•  FriedmanDI,LiuGT,DigreKB.Reviseddiagnosticcriteriaforthepseudotumorcerebrisyndromeinadultsand

children.Neurology.2013Sep24;81(13):1159-65•  BatemanGA,StevensSA,StimpsonJ.Amathematicalmodelofidiopathicintracranialhypertensionincorporating

increasedarterialinflowandvariablevenousoutflowcollapsibility.JNeurosurg.2009;110(3):446–56•  CapelC,BaronciniM,Gondry-JouetC,BouzerarR,CzosnykaM,CzosnykaZ,BalédentO.CerebrospinalFluidand

CerebralBloodFlowsinIdiopathicIntracranialHypertension.ActaNeurochirSuppl.2018•  BidotS,SaindaneAM,PeragalloJH,BruceBB,NewmanNJ,BiousseV.BrainImaginginIdiopathicIntracranial

Hypertension.JNeuroophthalmol.2015•  HartmannAJ,SoaresBP,BruceBB,SaindaneAM,NewmanNJ,BiousseV,PeragalloJH.ImagingFeaturesof

IdiopathicIntracranialHypertensioninChildren.JChildNeurol.2017Jan;32(1):120-126.•  ZurD,AnconinaR,KeslerA,LublinskyS,ToledanoR,ShelefI.Quantitativeimagingbiomarkersforduralsinus

patternsinidiopathicintracranialhypertension.BrainBehav.2017Jan3;7(2):e00613•  FarbRI,VanekI,ScottJN,MikulisDJ,WillinskyRA,TomlinsonG,terBruggeKG.Idiopathicintracranial

hypertension:theprevalenceandmorphologyofsinovenousstenosis.Neurology.2003May13;60(9):1418-24•  MorrisPP,BlackDF,PortJ,CampeauN.TransverseSinusStenosisIstheMostSensitiveMRImagingCorrelateof

IdiopathicIntracranialHypertension.AJNRAmJNeuroradiol.2017Mar;38(3):471-477•  Aguilar-PérezM,Martinez-MorenoR,KurreW,WendlC,BäznerH,GanslandtO,UnsöldR,HenkesH.

Endovasculartreatmentofidiopathicintracranialhypertension:retrospectiveanalysisofimmediateandlong-termresultsin51patients.Neuroradiology.2017Mar;59(3):277-287.

•  PufferRC,MustafaW,LanzinoG.Venoussinusstentingforidiopathicintracranialhypertension:areviewoftheliterature.JNeurointervSurg.2013Sep1;5(5):483-6.


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