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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
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