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ReversibleCerebral

VasoconstrictionSyndrome
PatMcCormick,MS4
ChicagoMedicalSchool&
UniversityofNorthCarolina

Outline

Definition
Epidemiology
ClinicalPresentation
Complications
Pathophysiology
SecondaryCauses
DifferentialDiagnosis
Reversiblecerebralvasoconstrictionsyndrome(RCVS)vs.Posterior
ReversibleEncephalopathysyndrome(PRES)

Imaging
Treatment&Prognosis

Definition
Severeheadacheswithorwithoutseizures
orneurologicdeficitsandconstrictionof
cerebralarterieswhichresolves
spontaneouslywithin13months

SynonymsorIncludedDisorders

Isolatedbenigncerebralvasculitisorangiopathy
CallFlemingsyndrome
CNSpseudovasculitis
Benignangiopathyofthecentralnervoussystem
Postpartumangiopathy
Migrainousvasospasm
Migraineangiitis
Idiopathicthunderclapheadachewithreversiblevasospasm
Druginducedcerebralvasculopathy
Fatalvasospasminmigrainousinfarction

Epidemiology
Females>males(210:1)
Meanageofonset=45y.o.
Incidenceunknownprobablyunder
diagnozedespeciallypurelycepahalalgicform
Upto60%aresecondary

ClinicalPresentation
Headache(secondary)thunderclapvariety,
peakswithinoneminuteandveryintense
Onlysymptomin75%
Multipleover14weekperiodisalmost
pathognomonic
Usuallyposteriorandbilateral
Nausea/vomiting,photophobia,phonophobia

Focalneurologicdeficitsandseizuresinminority
ofpatients

Complications

LocalizedcorticalSAH(2025%)
Ischemicorhemorrhagicstroke(510%)
PRES
Permanentsequelaeofausuallybenign
entity

Pathophysiology
Proposedmechanism:transientdisturbance
ofcerebralarterialvasculartonein
segmentalandmultifocalfashion
Leadstovariousareasofconstrictionand/or
dilatation
Eitheridiopathic(primary)orsecondary(25
60%)

SecondaryCauses
Vasoactivesympathomimeticorserotoninergicsubstances
Selectiveserotonicuptakeinhibitors,alphasympathomimetics
(nasaldecongestants),somedietpills
Illicitdrugs:cannabis,cocaine,ecstasy

Postpartumstate
Usually1stweekpostpartumafternormaldelivery
5070%associatedwithvasoconstrictorsusedtotreat
postpartumhemorrhageorinhibitlactation

Othercauses:hypercalcemia,pheochromocytoma,
exercise,andsexualintercourse

DifferentialDiagnosis
Aneurysmalsubarachnoidhemorrhage
Correlateswithsiteandseverityofvasospasm
Rareisolatedtoconvexity

Cerebralvasculitis,particularlyPACNS(Primary
angiitisofthecentralnervoussystem)

Moreinsidious,graduallyprogressiveheadache
MosthaveMRIabnormalities:multiple,smallinfarcts
CSFismarkedlyabnormal
Preferentiallyaffectssmalltomediumarterieswhereas
RCVSaffectsmediumtolargearteries

MoreDDxforThunderclapHeadache
Otherintracranialhemorrhages(cerebellarand
interventricular)
Cervicalandintracranialarterialdissections
Intracranialvenousthrombosis
Giantcellarteritis
Pituitaryapoplexy
Nonvasculardisorders:acutesinusitis,meningitis
andCSFhypotension

RCVSandPRES
Overlap:about10%ofcasesofRCVSare
associatedwithPRES,regardlessofcause
Sharesimilarclinicalfeatures
PREShascharacteristicfindingsonMRI
Symmetricalwhitematteredemainposteriorcerebral
hemispheres,particularlyparietooccipitalregions
HypoorisointenseonDWIandhyperintenseon
ADCmapdistinguishesitfromstrokeinmostpatients

Imaging
Imagingplaysavitalroleastheconditionis
definedinpartbythereversibilityofthe
cerebralvasoconstriction
Althoughrarelyused,cathetercerebral
angiographyisconsideredthegold
standard

NoncontrastCT
InuncomplicatedRCVS:usuallynormal
MayshowcorticalSAHorintracebral
hemorrhageincomplicatedcases
Shouldbefollowedbylumbarpunctureifnormal
toruleoutSAHandinflammatoryconditionslike
infectionorcerebralvasculitis

MRI
Usuallynormal
Mayshowevidenceofinfarctions,
especiallyinwatershedzones
MaylooklikePRES
ParenchymalhemorrhagesorcorticalSAH

AxialFLAIR&DWI(top&middleleft)
showhighsignalfromrightcerebellar
infarct.MRA(bottomleft)suggests
vasculitis.Lateral(center)ICAinjection&
frontal(right)vertebralarteryinjection
showtypicalsausagebeadingofRVCS.

PostpartumpatientshowsconvexitySAHonFLAIR
(left),smallbleedonT2*(center)&beadingof
arteries(right)especiallyintherightposterior
cerebralartery.

CTA/MRA/Angiography
Alternatingareasofconstrictionanddilatation
a.k.a.beadinginseveralvascularterritories
Maybeseeninlargetomediumsizedarteriesof
anteriororposteriorcirculation
Abnormalitiesmaybeabsentearlybutshowupon
repeatimaging,believedtostartdistallyandmove
centripetally
NOTspecificforRCVS
Resolutionwithin3monthsismostspecificfor
RCVS

PostpartumpatientshowsacuterightparietalhematomaonCT
(left),SAHonFLAIR(centerleft),PRESlikecerebellar
findings(centerright)&beading/thinningofarteriesonMRA
(right).

Prognosis
Highlydependentontheoccurrenceof
stroke(69%)
Otherwise,bydefinition,mostresolve
completelywithoutanysequelae

Treatment
Symptomatic(pain,seizures,bloodpressurecontrol)
Triggeravoidance(eitheractivityorvasoactive
substances)
Observation
Calciumchannelblockers
IVmagnesium
Shortcourseofsteroids?

Sources
CalabreseLH,DodickDW,SchwedtTJ,etal.Narrativereview:reversiblecerebral
vasoconstrictionsyndromes.AnnInternMed2007;146:3444.
DucrosA,BoukobzaM,PorcherR,etal.Theclinicalandradiologicalspectrumofreversible
cerebralvasoconstrictionsyndrome.Aprospectiveseriesof67patients.Brain2007;130:
3091101.
DucrosA,BousserM.Reversiblecerebralvasoconstrictionsydnome.PractNeurol2009;9:
256267.
KoopmanK,UyttenboogaartM,LuijckxGJ,etal.Pitfallsinthediagnosisofreversiblecerebral
vasoconstrictionsyndromeandprimaryangiitisofthecentralnervoussystem.EurJNeurol
2007;14:10857.
MoskowitzSI,CalabreseLH,WeilRJ.Benignangiopathyofthecentralnervoussystem
presentingwithintracerebralhemorrhage.SurgNeurol2007;67:5227.
WhyteCA,CalabreseLH.Reversiblecerebralvasoconstrictionsyndrome.Headache:the
journalofheadandfacepain.2009;49:597598.

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