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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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
ChangesontheCSFvolumewill
induceCBVvariationatthe
venousside(sincetheveinsaremoreelastic/compressible
andhavelowerpressure
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• CSFphysiologyandpressuremeasurements:
Backgrou
nd
• CSFopeningpressure
– Hypertension:>250mmH2O(children>280mmH2O)
– Hypotension:<60mmH2O(horizontal
orlateraldecubitus;sitting)
» Aliquorrhoea(negativeornon-measurablepressure)
» SittingthepatientthereisanincreaseoftheCSFpressureinnormalpatientsàinintracranialhypotensiontheCSFpressuremaintains<60mmH2O
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
From:LancetNeurol2015;14:655–68
• CSFphysiologyandpressuremeasurements:
Backgrou
nd
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
FromCapelCetalT.Heldt(ed.),IntracranialPressure&NeuromonitoringXVI,ActaNeurochirurgicaSupplement,Vol.126,2018
Arterialflow
Venousflow
ArterioVenous(arterial+venous)
flow
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• CSFphysiologyandpressuremeasurements:
Backgrou
nd
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
FromCapelCetalT.Heldt(ed.),IntracranialPressure&NeuromonitoringXVI,ActaNeurochirurgicaSupplement,Vol.126,2018
Arterialflow
Venousflow
ArterioVenous(arterial+venous)
flow
ArterioVenous(arterial+venous)
flow
CSFflow
• CSFphysiologyandpressuremeasurements:
Backgrou
nd
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
FromCapelCetalT.Heldt(ed.),IntracranialPressure&NeuromonitoringXVI,ActaNeurochirurgicaSupplement,Vol.126,2018
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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Summary
• Intracranialhypertension:
• Background• EtiologyandPhysiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Etiology
• IntracranialCSFpressurechanges:
• Causes:
• Hydrocephalus
• Intracranialmass
• Brain“oedema”;toxic;inflammatory;infectiouslesions
• Venousoutflowobstruction
• IdiopathicFrom:LancetNeurol2015;14:655–68
ICHypertension
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Diagno
sticcriteria
• IdiopathicIntracranialhypertension(IIH):
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
FromFriedmanDetalNeurology2013
Diagno
sticcriteria
• IdiopathicIntracranialhypertension(IIH):– Diagnosticcriteria
HeadachePapilledemaHighCSFopeningpressureCSFnormal
From:LancetNeurol2015;14:655–68
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Diagno
sticcriteria
• IdiopathicIntracranialhypertension(IIH):
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
FromFriedmanDetalNeurology2013
Reviseddiagnosticcriteriaforthepseudotumorcerebrisyndromeinadultsandchildren2013
Etiology
• IdiopathicIntracranialhypertension(IIH):
• 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
• Intracranialhypertension:
• Background• EtiologyandPhysiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
R
L
• IdiopathicIntracranialhypertension(IIH):
Summary
• Intracranialhypertension:
• Background• EtiologyandPhysiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
fromHartmannAJPetalJCN2016
fromDucrusAetalLancetN2015
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”andenlargmentofMeckelcave,
foramenovale,» “Slit”ventricles» Tonsillarherniation
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
fromTawfikKetalOto&N2017
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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”andenlargmentofMeckelcave,
foramenovale,» “Slit”ventricles» Tonsillarherniation
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings
• IdiopathicIntracranialhypertension(IIH):
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
• 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
• IdiopathicIntracranialhypertension(IIH):
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
• Duralsinusstenosis
QualitativeScore(FarbR2003)
Quantitative(CAD-ZurD2017):higherdifference(IIH/C)onminimalcrosssectionarea
FromFarbRetalNeurology2003
1
0
2
4
12
1 1
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Imagingfin
dings
• IdiopathicIntracranialhypertension(IIH):
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
• Duralsinusstenosis
QualitativeScore(FarbR2003)
Quantitative(CAD-ZurD2017):higherdifference(IIH/C)onminimalcrosssectionarea
FromZuretalBrainandbehavior2017
Summary
• Intracranialhypertension:
• Background• Physiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Treatm
ent
• IdiopathicIntracranialhypertension(IIH):– Treatment
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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Treatm
ent
• IdiopathicIntracranialhypertension(IIH):– Treatment
Emergent
Preventvisualloss!!!
• Papilloedema + severe headache + notsevere visual lossà ventricular / lumbarCSFshunting
• Papilloedema + severe visual lossà opticnervesheathfenestration
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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%
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Brain-Degenerative:Intracranialhypertension,hypotensionandCSF-leaks
PedroVilelaLisbon-Portugal
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Summary
• Intracranialhypotension:
• Background• EtiologyandPhysiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Etiology
• IntracranialCSFpressurechanges:
• Causes:
• Hypovolemia
• Overshunting
• CSFleaks(traumaticand“spontaneous”)
From:LancetNeurol2015;14:655–68
ICHypotension
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Etiology
• Intracranialhypotension:
• 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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Physiopa
thology
• Intracranialhypotension:
• Physiopathology: CSFleak
LossofCSFVolume
NegativeintracranialpressureVolumecompensation:éBloodVol
Monroe-Kelliehypothesis:constantintracranialvolume(blood+CSF+brain)
ExponentialrelationshipVOLUME–PRESSUREThewithdrawalofapproximately10%oftheCSFvolumeà
cause>40%decreaseinthe(alreadynegative)vertexCSFpressure
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Physiopa
thology
Negativeintracranialpressure
Descent(sagging)ofthebrain
Tractiononpain
sensitivestructures
Venousrupture
Tractionofnerveroots/
cranialnerves
SubduralhematomasPain Nerve
palsies
Encephalopathy;Lossofconsciousness;Coma
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Negativeintracranialpressure
Dilatationofpain-
sensitivevenous
structures
Physiopa
thology
Pain
Pituitaryhyperemia
Descent(sagging)ofthebrain
Tractiononpain
sensitivestructures
Venousrupture
SubduralhematomasPain
VolumecompensationMonroe-Kelliehypothesis:constant
intracranialvolume(blood+CSF+brain)
Increaseofthevascularcomponent
VenousDilatation(intracranial&spinal)
DurahasnoBBB
Subduralcollections
Increasedvenouspressure
Extravasationoffluidthroughtheinnermost
durallayer
Tractionofnerveroots/
cranialnerves
Nervepalsies
Encephalopathy;Lossofconsciousness;Coma
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Summary
• Intracranialhypotension:
• Background• EtiologyandPhysiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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
From:LancetNeurol2015;14:655–68
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Summary
• Intracranialhypotension:
• Background• EtiologyandPhysiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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
• Intracranialhypotension:
• PrimaryImagingfindingsàdetectionoftheCSFleak(s)
– Myelography(Myelo-CT)» Delayedacquisitions(3-4h)forslow/lowvolumeleaks» Rapidacquisitionsforfast/highvolumeleaksàDSA
– Myelo-MRI» Withoutcontrast» Withintratecalgadolinium
– Radionuclidemyelo/cisternography
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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”)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:NuclearM
edicine
• Intracranialhypotension:– NuclearMedicine(radioisotope)Cisternography
CSFleakidentification
Indirectsign:earlypassagetourinarybladder/kidneysoftheradioisotope
Indirectsign:absence/paucityofradioisotopeoverthecerebralconvexities
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI
• Intracranialhypotension:
• SecondaryImagingfindingsàbrainandspinalchangesinducedbytheCSFleak(s)–CSFlowvolume/pressure
– Intracranialfindings
– Spinalfindings
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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!
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI Negativeintracranialpressure
Descent(sagging)ofthebrain
Tractiononpain
sensitivestructures
Venousrupture
SubduralhematomasPain
Tractionofnerveroots/
cranialnerves
Nervepalsies
• Effacementofperichiasmaticprepontinecisterns
• Pituitarystalkandponsflattening• Bowingoftheopticchiasm• Cerebellartonsilsdescend
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI Negativeintracranialpressure
Dilatationofpain-
sensitivevenous
structures
VolumecompensationMonroe-Kelliehypothesis:constant
intracranialvolume(blood+CSF+brain)
Increaseofthevascularcomponent
Pain
Pituitaryhyperemia
Descent(sagging)ofthebrain
Tractiononpain
sensitivestructures
Venousrupture
SubduralhematomasPain
Subduralcollections
VenousDilation(intracranial&spinal)
Increasedvenouspressure
Extravasationoffluidthroughtheinnermost
durallayer
Tractionofnerveroots/
cranialnerves
Nervepalsies
meningealvesselsàcontrast
enhancement
• Effacementofperichiasmaticprepontinecisterns
• Pituitarystalkandponsflattening• Bowingoftheopticchiasm• Cerebellartonsilsdescend
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI
Subduralfluidcollections
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI
• Intracranialhypotension:– Clinicalpresentation
from:TanaCetal;Neurology.2014TeachingNeuroImages:Adangerouscomplicationofspontaneousintracranialhypotension.
SubduralhematomasSubduralfluidcollections
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI
• Intracranialhypotension:– SecondaryimagingfindingsàCT/MRI
• Diffuseduralthickening/enhancement(pachymeninges)à85%– Diffuse/crescent-shapedduralthickening
» ThinandLinear–notnodular» Noleptomeningealinvolvement» Supraandinfratentorial
– IsointensetobrainonT1&HyperintensetobrainonT2/FLAIR– Diffuseintensecontrast-enhancement
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI
• Intracranialhypotension:– SecondaryimagingfindingsàCT/MRI
• Braindownwarddisplacement à40-50%– Saggingofthemidbrain(descendbelowthelevelofthedorsumsellae)– Reductionofthecerebralpeduncles/ponsangle– Caudaldisplacementofthecerebellartonsils(25-75%)– Temporallobesherniationthoughttheincisura
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI
• Intracranialhypotension:– SecondaryimagingfindingsàCT/MRI
• Braindownwarddisplacement à40-50%– Saggingofthemidbrain(descendbelowthelevelofthedorsumsellae)– Reductionofthecerebralpeduncles/ponsangle– Caudaldisplacementofthecerebellartonsils(25-75%)– Temporallobesherniationthoughttheincisura
SIH Posttreatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI
• Intracranialhypotension:– SecondaryimagingfindingsàCT/MRI
• Other– Engorgementofthevenousstructures(cavernoussinus;otherduralsinus;corticalveins;spinalveins–epiduralplexuses)
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
PosttreatmentSIH
Imagingfin
dings:CT/MRI
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI
• Intracranialhypotension:– SecondaryimagingfindingsàCT/MRI
• Other– Sellaturcica:Enlargementofthepituitarygland(convexsuperiorborder)–reversiblepituitaryhyperemia
PosttreatmentSIH
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI
• Intracranialhypotension:SpinalFindings• Extra-arachnoidfluidcollections
Causeunknown:directleakageofCSFintotheepiduralspace/effusion/transudationofintravascularfluidfromhyperemicmeningesintoepidural/subduralspaceNonfocal,extendingovermultiplespinallevels
• DuralthickeningandenhancementFromduralvasodilationandengorgement
• Dilationoftheanteriorepiduralveins/venousplexus
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Imagingfin
dings:CT/MRI
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Imagingfin
dings:CT/MRI
07-2009 10-2010
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Summary
• Intracranialhypotension:
• Background• Physiopathology• Incidence;prevalence;clinicalpresentation• Imagingfindings• Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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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
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Treatm
ent
• Intracranialhypotension:– Treatment
2ndlineEpiduralbloodpatch
Refractoryafter~5days
2ndlineRepeatedEpiduralbloodpatch-nontarget(2to3timeswithanintervalof5days)
Refractoryafter~5days
Othertherapeuticoptions:precisesiteoftheCSKleakTargetBloodpatch/Fibrinsealant/Surgery
3rdline
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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Treatm
ent
• Intracranialhypotension:– Treatment
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
Brain-Degenerative:Intracranialhypertension,hypotensionandCSF-leaks
PedroVilelaLisbon-Portugal
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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.
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks
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.
Brain-Degenerative: Intracranial hypertension, hypotensionandCSF-leaks