Headaches:Neuro-endovasculardiagnos4candtreatmentop4ons.

“PseudotumorCerebri”EdisonValle,M.D.

Disclosures

Introduc4on

•  Pseudotumorcerebri:à1904àpa4entswithsignsandsymptomsofincreasedintracranialpressurebutnotumor.–  DandyCriteria:normalbrainimaging(ventriculograms).

•  Otherterms:meningi4sserosa,o44chydrocephalus,toxichydrocephalus,hypertensivemeningealhydrops,pseudoabscess,papilledemaofundeterminede4ology,andintracranialpressurewithoutbraintumor.

•  1955àbenignintracranialhypertensionà1969àIdiopathicintracranialhypertension(IIH)

GiridharanN,PatelSK,OjugbeliA,NouriA,ShiraniP,GrossmanAW,ChengJ,ZuccarelloM,Pres4giacomoJ,Understandingthecomplexpathophysiologyofidiopathicintracranialhypertensionandtheevolvingroleofvenoussinussten4ng:acomprehensivereviewoftheliterature.NeurosurgFocus45(1):E10,2018

•  Recently:primaryintracranialhypertension(PIH)andsecondaryintracranialhypertension(SIH).

•  IIHandPIHareuseindis4nctlyàisadiagnosisofexclusion.

Epidemiology

•  Incidence0.5-2/100,000.•  25–36yearsofageinaverage.•  Morecommoninwomen.•  Obesityincreasesrisk20-fold.

Clinicalpresenta4on.

•  GeneralizedHeadaches+visualchanges.– HA:pressure-like,severeuponawakening,accompaniedbynausea,photophobiaand/orphonophobia,neckpain,4nnitus.

– Transient/permanentvisionloss:doublevision,reduc4oninvisualacuity,VFdeficits.

•  Signs:ElevatedICPàpapilledema.•  DiagnosisisDxofexclusion.

Pathophysiology

•  Specula4vereasons.•  IncreasedCSFproduc4on,decreasedCSFabsorp4on,increasedvenoussinuspressure,obesity,changesinvitaminAmetabolism,hormonalinbalance.

GiridharanN,PatelSK,OjugbeliA,NouriA,ShiraniP,GrossmanAW,ChengJ,ZuccarelloM,Pres4giacomoJ,Understandingthecomplexpathophysiologyofidiopathicintracranialhypertensionandtheevolvingroleofvenoussinussten4ng:acomprehensivereviewoftheliterature.NeurosurgFocus45(1):E10,2018

Treatment•  Firstline:medicaltherapyandweightloss.•  Medicaltherapy:Carbonicanhydraseinhibitor(acetazolamide)à

classIBevidence.–  Improvedvisualfields–  Decreasedpapilledema–  Improvedqualityoflife.–  DecreasedCSFpressure.–  Othermedica4ons:furosemide,topiramate.

•  Acetazolamide:adverseevents:electrolytedisturbances,nephrolithiasis,paresthesias,dysgeusia,nausea/vomin4ng,diarrhea,fa4gue.

•  Secondline(failedmedicaltherapy):CSFdiversion,op4cnervesheathfenestra4on,bariatricsurgery,VSSàthereisnoRTCforthisdata.

WallM:Idiopathicintracranialhypertension.NeurolClin28:593–617,2010WallM:Updateonidiopathicintracranialhypertension.NeurolClin35:45–57,2017

ONF

CSFdiversion

•  7.6%majorcomplica4ons.•  33%minorcomplica4on•  Revisionrate43%.•  95%improvedHAby1month•  HArecurrence:– 6months:63%– 1year:77%– 2years:79%.

SajSRSr,LeishangthemL,ChaudryMI:Meta-analysisofCSFdiversionproceduresandduralvenoussinussten4nginthesejngofmedicallyrefractoryidiopathicintracranialhypertension.AJNRAmJNeuroradiol36:1899–1904,2015

VSS

•  10-90%oftheIIH,dependingonseries.•  78%ofpa4entshadreliefinHA.•  86.5%hadimprovementonvision.•  94%resolu4onofpapilledema.•  93%improvementof4nnitus.•  Complica4onrate5.4%.•  Followupsà6%developadjacentstenosis.

KanagalingamS,SubramanianPS.UpdateonIdiopathicIntracranialHypertension.CurrTreatOp4onsNeurol(2018)20:24

VSS

•  78%con4nuetohaveHA•  33%con4nuevisualdisturbances.•  0%hadauditorybruitorpapilledema.•  6%hadnewonset4nnitus.•  89%hadimprovementofsymptoms.

CappuzzoJM,HessRM,MorrisonJF,DaviesJM,SnyderKV,LevyEI,SiddiquiAH.Transversevenoussten4ngforthetreatmentofidiopathicintracranialhypertension,orpeudotumorcerebri.NeurosurgFocus45(1):E11,2018

ShuntVSVSS

•  Shun4ngàgoodshort-termsuccess(classIIA)àclosetohalfofpa4entshaverecurrenceofHAat36mo.

•  CostofshuntismuchgreaterthanVSSà$15kVS13K.

•  Alsodelayedcostàshuntrevisionmuchhigherratethansten4ng.àcostper100casesforshuntwasalmosttwicethecostforVSS.

AhmedRM,ZmudzkiF,ParkerGD,OwlerBK,HalmagyiGM:Transversesinussten4ngforpseudotumorcerebri:acostcomparisonwithCSFshun4ng.AJNRAmJNeuroradiol35:952–958,2014

KanagalingamS,SubramanianPS.UpdateonIdiopathicIntracranialHypertension.CurrTreatOp4onsNeurol(2018)20:24

Totopitoff…

•  Reversiblesinusstenosis.– McGonigaletal.Neurology2004;62:514-515– Baryshniketal.Neurology2004;62:1444-1446– HigginsJNPetal.Neurology2004;62:1907-1908

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