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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_186_библиотеки_им_акад_М_И_Перельмана
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Digitalsubtractioncatheterangiographyshowingatypicalflame-shapedocclusionoftheinternalcarotid
artery.(ReprintedfromCastilloM.NeuroradiologyCompanion.4thed.WoltersKluwer;2011.)
CerebralVenousSinusThrombosis
Thrombosisofthecerebralveinsorduralvenoussinusesisrelativelyrarebutanimportantdo-not-miss
diagnosis.Theduralvenoussinuses(alsoreferredtoascerebralvenoussinuses)arevenouschannels
locatedwithinthedurathatdrainbothbloodandCSFfromthebrainintotheinternaljugularvein.
Consequently,thrombosiscanobstructdrainageofbothblood(predisposingtoischemicandhemorrhagic
stroke)andCSF(whichcanresultinelevatedintracranialpressure).
Themajorcerebralveinsandsinuses.
Womenareaffectedmoreoftenthanmen.Riskfactorsincludebothgeneticandacquiredthrombophilia
(mostnotablypregnancy-associatedthrombophilia;theriskofcerebralvenoussinusthrombosis(CVST)
ismorethantentimeselevatedduringlatepregnancyandtheearlypostpartumperiod),headinjury,and
infectioninvolvingtheears,sinuses,mouth,andthroat.
Symptomsvarywidelyandincludeheadache(oftenwithfeaturesofintracranialidiopathic
hypertension;seepage115),seizures,encephalopathy,andfocalsymptomsdependentonthelocationof
thethrombosedvein.
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CerebralVenousThrombosiswithVaccine-InducedImmuneThrombotic
Thrombocytopenia
Theuseoftheadenovirus-basedcoronavirusvaccineswasbrieflysuspendedinthespringof
2021givenreportsofbothcerebralandsplanchnicvenousthrombosisassociatedwith
thrombocytopeniaandantibodiesdirectedagainstplateletfactor4(PF4).Currently,onlya
handfulofcaseshavebeenreportedamongthetensofmillionsofvaccinatedpatients,and
thebenefitsofthevaccineshavebeendeterminedtogreatlyoutweightherisks.However,
earlyrecognitionofthesignsandsymptomsofvenousthrombosisinpatientsrecently
vaccinatedwiththeadenovirus-basedvaccinesremainscritical,andshouldprompttesting
foranti-PF4antibodiesandtreatmentwithnonheparin(argatroban,fondaparinux)
anticoagulation.
MRorCTvenogramsarethemostsensitivetechniquesfordiagnosingvenoussinusthrombosis.
Occasionally,CTwilldemonstratedirectvisualizationoftheclot.Infarctsduetovenoussinusthrombosis
tendtoinvolvemultiplearterialterritories,becausetheydonotrespectthearterialvasculardistributions.
Treatmentiswithanticoagulation,whichiscontinuedanywherefromseveralmonths(inthecaseofa
provokedthrombosis)tolifelong(inpatientswithseveregeneticthrombophilia).Importantly,the
presenceofvenoushemorrhageisnotacontraindicationtoanticoagulation.
(A)The“densetriangle”signonnoncontrastCTscandemonstratingahyperdenseclotwithinthesuperior
sagittalsinus.(B)The“emptydelta”signonpostcontrastMRIinthesamepatient,demonstratingafilling
defectinthesamelocation.Thereisalsoassociatedleptomeningealenhancementlikelyduetovenous
congestion.(ReprintedfromPopeTLJr,HarrisJHJr.Harris&Harris’TheRadiologyofEmergency
Medicine.5thed.WoltersKluwer;2012.)
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ReversibleCerebralVasoconstrictionSyndrome(RCVS)
RCVSisasyndromethatischaracterizedbyreversibleandmultifocalconstrictionoftheintracranial
cerebralarteries.Itismorecommoninwomenthanmen,andriskfactorsincludepregnancy,migraine,
andtheuseofvasoconstrictiveandothermedications(includingtriptans,selectiveserotoninreuptake
inhibitorsandvariousimmunosuppressants)andillicitdrugs(cocaineandmethamphetamine).
Recurrent,excruciatingthunderclapheadachesoverthespanofdaystoweeks,withorwithout
associatedfocaldeficits(duetoedema,orischemicorhemorrhagicstrokescausedbyvasospasm),are
themosttypicalpresentation.
Brainimagingisoftennormalespeciallyearlyon,butcanshowsmallischemicinfarcts(typicallyin
watersheddistributions)aswellasintracerebralandsubarachnoidhemorrhage.Thepresenceofdiffuse,
segmentalvasoconstrictioncausinga“beaded”appearanceonCTorMRangiographyisclassicand,in
therightclinicalcontext,diagnostic,butthesescanscanbenormalearlyonaswell.Iftheinitialscanis
negativeandclinicalsuspicionishigh,it’sagoodideatorepeatthescanafewdayslater.
Anangiogramshowingmultifocalarterialnarrowingconsistentwithreversiblecerebralvasoconstriction
syndrome(RCVS).(CourtesyofJonathanHoward.)
ThereisnoproventreatmentforRCVS,althoughanecdotalevidencesuggestscalciumchannel
blockersmaybehelpful.Steroidshaveactuallybeenshowntoworsenoutcomesandshouldbeavoided.
Ifacausativeagentispresent(suchasatriptan,SSRI,orcocaine),itshouldbestopped.Mostpatients
recoverspontaneouslywithinweekstomonths.
Vasculitis
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CNSvasculitisreferstodiffuseinflammationandbreakdownofthebloodvesselsinthebrainand/or
spinalcord.Theoverwhelmingmajorityofcasesaresecondarytoanotherprocess,suchasasystemic
autoimmuneorinflammatorydisease,infectionorneoplasm.Whennosecondaryetiologyisfound,the
conditionisreferredtoasprimaryangiitisoftheCNS(PACNS).PACNSisrare,mostoftenseeninolder
men,andtypicallylacksthesystemicsymptomsassociatedwithmostothervasculitides(suchasfever
andweightloss).
ExamplesofSecondaryCausesofCNSVasculitis
Systemicvasculitides Largevesselvasculitis(giantcellarteritis,takayasuarteritis)
Antineutrophilcytoplasmicantibody(ANCA)-associatedsmallvesselvasculitis(granulomatosiswithpolyangiitis)
Immunecomplex-associatedsmallvesselvasculitis(cryoglobulinemicvasculitis)
Autoimmune disease Sjögrensyndrome
Infection Viral(HIV,varicella-zostervirus[VZV],cytomegalovirus[CMV])
Bacterial(TB,lyme,syphilis)
Fungal(aspergillus,cryptococcus)
Parasitic(cysticercosis,malaria)
DrugInduced Cocaine
Methamphetamines
Neopla stic Lymphoma
Paraneoplastic
Ingeneral,thepresentationofCNSvasculitisisnonspecificandinsidious.Headaches,encephalopathy,
andseizuresarecommon.Bothischemicandhemorrhagicstrokescanoccurduetovascularbreakdown.
Tip-offsonMRItoanunderlyingvasculiticetiologyincludeacombinationofbothischemicand
hemorrhagiclesions,smallembolic-appearingischemicstrokesaffectingbothcorticalanddeepgray
matterstructures(duetoinvolvementofthesmallandmediumvessels)andstrokesinoddlocations(e.g.,
thecorpuscallosum).
Establishingthediagnosisinvolvesconfirmingvasculiticchangesonangiography,whichwillshow
segmentalnarrowingwithabeadedappearance.Soundfamiliar?DistinguishingvasculitisfromRCVS
canbedifficult,butitiscriticalgiventhevastlydifferenttreatmentprotocols.CSFanalysisisoften
crucial:youshouldexpectaninflammatoryprofilewithelevatedproteinandwhitecellsinpatientswith
vasculitis,whereasinRCVSnormalCSFistherule.AbatteryofbothserumandCSFstudiesshouldbe
senttohelpdeterminethepresenceofasecondaryetiology,includingvariousinflammatory,autoimmune,
andneoplasticmarkers.
Treatmentiswithsteroidsandshouldbeinitiatedearly,regardlessofknownorunknownsecondary
vasculiticetiology.Evidenceregardingtheoptimaldurationoftherapyislimited,anddecisionsaremost
oftenmadeonacase-by-casebasis.
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Follow-uponYourPatient:Laura’sdeficitsaresubtlebutultimatelydeemeddisablingand,
intheabsenceofanycontraindications,sheisgiventPA.HerCTangiogramisnormal,
withoutevidenceofanintervenableLVOoranysignificantstenosis.Herexamination
improvespost-tPA,andseveralhourslateronlyverymildwristweaknesspersists.HerMRI
showsanembolic-appearingright-sidedfrontoparietalinfarct.Givenherageandlackof
cardiovascularriskfactors,youorderatransesophagealecho,whichrevealsalargepatent
foramenovale(PFO).5LowerextremityDopplers(tolookfordeepveinthrombosis)are
negative,butherhypercoagulabilitypanelreturnspositiveforfactorVLeiden.Sheisstarted
onanticoagulationanddischargedhome,andafteronlyaweekortwoofphysicaltherapy,
she’sbackonthecourt.
Younowknow:
The basics of cerebrovascular anatomy and can localize many of the most common stroke
syndromes.
HowtoclassifyischemicstrokesubtypesviatheTOASTclassification.
Whentogiveand—justasimportant—whennottogivetPAforacuteischemicstroke.
Whentoconsiderendovascularthrombectomyforacuteischemicstroke.
Howtomanageacuteintracerebralandsubarachnoidhemorrhage.
How to recognize and treat several other important cerebrovascular disorders, including
cervicocephalicarterialdissection,RCVS,cerebralvenoussinusthrombosis,andCNSvasculitis.
Citedstatisticsherearefrom:
ViraniSS,AlonsoA,BenjaminEJ.Heartdiseaseandstrokestatistics–2020update:areportfromthe
AmericanHeartAssociation.Circulation.2020;141:e139-e596.
https://www.ahajournals.org/doi/10.1161/CIR.0000000000000757TheNationalInstituteofNeurological
DisordersandStrokert-PAStrokeStudyGroup.Tissueplasminogenactivatorforacuteischemicstroke.
NEnglJMed.1995;333:1581-1588.
1
ThisnumberisforadultsintheUnitedStates,anditchangesbasedongeographiclocationandage;inchildren,forinstance,hemorrhagic
strokeismuchmorecommonthanischemicstroke.
2
Wewillnotbetalkinganymoreabouttheexternalcarotidartery,butincaseyouareinterested,itsuppliesbloodtothefaceandneck,notthe
brain.Externalcarotidarterydiseasedoesnotcausestroke.
3
Thisistrueasofthiswriting,butongoingresearchiscurrentlyinvestigatingtherisksandbenefitsofgivingtPApriortothrombectomy.
4
Tenecteplase(TNK)isanotherthrombolyticthatcanbeused.ItissimilartotPAbuthasahigherfibrinspecificityandalongerhalf-life;its
timewindowandcontraindicationsarethesame.Ongoingresearchisevaluatingitsefficacyforacutestroke.
5
TherecentpublicationofseverallargeclinicaltrialshasresultedinfurtherexpansionofthetPAwindowincarefullyselectedpatient
populationswithspecificimagingcharacteristicssuggestiveofsignificantsalvageablebraintissue.Perhapsmostexcitingly,patientswith
“wake-up”strokes(i.e.,patientswhofallasleep“normal”andwakeupthefollowingmorningwithneurologicdeficits)arenowpotentialtPA
candidatesiftheirimagingmeetsspecificcriteria.
5
Thisisbeyondthescopeofthisbook,butincaseyouareinterested,theliteraturehasgonebackandforthonthebenefitsandrisksofPFO
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closureinpatientswithstroke.Differentphysiciansandinstitutionshavedifferentpreferences,butinthecaseofourpatient,mostwouldlikely
deferclosure;becausesheispositiveforfactorVLeidenandnowhasahistoryofthromboembolism,shewillbeonlifelonganticoagulation
regardless,thusobviatingtheneedforclosure.
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3Headache
Inthischapter,youwilllearn:
1. Howtorecognizeandtreatcommonheadachedisorders
2. Whentoworry(andwhattodo)aboutpotentiallyalarmingheadacheetiologies
3. Thedifferentialdiagnosisofthunderclapheadaches
4. Howtomanageacutemigraineinanurgentsetting
5. Howtomanageheadacheduringpregnancy
CASE3
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YourPatient:Anna,anotherwisehealthy28-year-oldfirst-yearsurgicalresident,comesto
seeyoubecauseofworseningheadaches.Shedescribesalonghistoryofpredominantly
right-sided,throbbingheadachesthatusedtooccuraboutonceamonthandresolvedwith
nonsteroidalanti-inflammatorydrugs(NSAIDs).Butinthepastfewmonths,shehasbeen
gettingtheseheadachesmuchmorefrequently,experiencingthreethispastweekalone.
NSAIDstaketheedgeoffbutnolongerfullyrelievethepain.Shehasahardtimeworking
whenshehasaheadache;justlookingatacomputerscreenhurtshereyes,andtryingto
thinkfeelslike“runninginaswimmingpool,”slowandexceedinglyeffortful.What’sthenext
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stepinyourmanagement?
Headacheisoneofthemostcommonreasonswhichsendpeopletoseekmedicalattention.Primary
headachedisorders,suchasmigraine,tension-type,andclusterheadaches,arebyfarthemostfrequently
encountered.MorethanonemillionpatientsvisitemergencyroomsintheUnitedStateseveryyearfor
migrainesalone.However,secondaryheadachedisorders—headachescausedbyotherunderlying,
sometimesworrisome,occasionallylife-threateningpathologiessuchasinfections,bleeds,andtumors—
compriseasignificantminority.Distinguishingbetweenprimaryandsecondaryheadachesisthemost
importantandoftenthemostchallengingstepinmanagingpatientswithheadache,solet’sstartthere.
HeadacheRedFlags
Primaryheadachedisorderscanbesevereandevendisabling,buttheyarenotfatal,unlikesome
secondaryheadachedisorders.Sowhendoweworry?Whatsetsoffalarmbellsforapotentialsecondary
etiology?ThemnemonicSNOOPishelpfulhere.Asourknowledgebasehasincreased,thismnemonic
hasgonethroughmultipleiterations(mostrecentlySNNOOPPPPPPPPPP—wearenotkidding),butfor
thesakeofsanity(yoursandours)andsimplicity,wewilluseSNOOP2.
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