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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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MRIofapatientwithintracranialidiopathichypertension;notetheemptysella(arrow).(Source:Dr.Daniel
T.Ginat,MD.)
Withoutanyintervention,thenaturalhistoryofpseudotumorcerebriisoneofslowsymptomatic
progression.Treatmentisthereforerecommended.Conservativemanagementwithcloseobservation,
weightloss,andotherriskfactormodificationsarefirst-linetreatmentsinpatientswithoutevidenceof
visionloss.Iftheheadachedoesnotimproveorifthereisevidenceofearlyvisionloss,carbonicanhydraseinhibitorssuchasacetazolamideortopiramateareused(thesedrugsdecreaseCSF
production).Ifthereisprogressivevisionloss,proceduraloptionsincludeopticnervesheathfenestration
(torelievepressureonthenerve)andplacementofaventriculoperitonealshunt(todivertCSFfromthe
brainintotheabdomenforbetterabsorption).Thereisnoindicationforseriallumbarpunctures,which
cancausesignificantdiscomfortandofferonlytemporaryrelief;theymay,however,beappropriateasa
temporizingmeasurepriortosurgeryorinpregnantwomenwhowishtoavoidmedicationsduring
pregnancy.
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PosteriorReversibleEncephalopathySyndrome(PRES)
Althoughthepreciseincidenceofposteriorreversibleencephalopathysyndrome(PRES)isunknown,itis
beingincreasinglyreportedinthemedicalliterature.Thisisonesyndromeyoureallyneedtoknowabout.
TheeasiestwaytothinkaboutPRESisasaconstellationofclinicalandradiographicfeaturesresulting
fromacutevasogenicedema(i.e.,fromtheextracellularaccumulationofintravascularfluiddueto
disruptionoftheblood-brainbarrier).Thenameisn’tthebest,becausePRESisnotexclusivelyposterior
(i.e.,involvingtheparieto-occipitalregion),maynotbereversible,anddoesnotalwayscause
encephalopathy.Solet’sgothroughitcarefully.
PREScanbecausedbytwothings:
Thefirstisarapidriseinbloodpressure.Thebrainisnormallyabletoautoregulateitselfsuchthat
cerebralbloodflowremainsstableoverabroadrangeofmeanarterialbloodpressures.Thereisan
upperlimittothisprocess, however,andwhenitisexceeded,cerebral bloodflowincreases.The
resultingelevatedpressurescancausefluidtoextravasatefromthecerebralbloodvesselsintothe
brainparenchyma,resultinginvasogenicedema.
Thesecondisimmunosuppressivemedication,suchascyclosporineandtacrolimus.Thepostulated
mechanismhereisdirectendothelial toxicitycausedbythemedicationitself,resultingincapillary
leakageanddisruptionoftheblood-brainbarrier,whichcanoccurevenaftermonthsofexposureto
theoffendingmedication.ToxiclevelsofthesemedicationsarenotrequiredtocausePRES.
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MRIwithclassicPRESfeatures(symmetric,confluentwhitematteredema;whitearrows).(Modifiedfrom
PulaJH,EggenbergerE.Posteriorreversibleencephalopathysyndrome.CurrOpinOphthalmol.
2008;19:479-484.)
Symptomsincludeheadache(oftenconstantandunresponsivetopainmedication),visualdisturbances
(duetopreferentialposteriorcerebralinvolvementcausingvisualfieldcuts,hallucinations,cortical
blindness,andsoforth),seizures,andencephalopathy(mostoftenlethargyandconfusion).
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Imagingshowssymmetric,confluentwhitematteredemathatoften(butnotexclusively)involvesthe
posteriorparieto-occipitalregions.ThisisbestseenonMRIbutcanbepickeduponCTinseverecases.
Imagingisessential,becausetherearenoreliableclinicalguidelinesfordiagnosis,andasyoumight
imaginethedifferentialdiagnosisofthissymptomcomplexisbroad(includingstrokesyndromes,
malignancies,andencephalopathies).AnMRIconsistentwithPRESintherightclinicalsetting(elevated
bloodpressureortheuseofimmunosuppressants)shouldmakeyoufeelconfidentinthediagnosis.
Treatmentissymptomatic,withbloodpressurecontrol,seizurecontrol,and,ifindicatedandfeasible,
withdrawalordosereductionoftheoffendingmedication.Mostpatientsrecoverwell,butneurologic
sequelae(suchasmotordeficitsandepilepsy)anddeathcanoccur.
CardiacCephalalgia
Cardiaccephalalgiareferstoheadacheduetomyocardialischemia.Theheadacheitselfcanclosely
resembleamigrainebutisvariableinlocation,intensity,andduration.Itcanbe—butdoesnothavetobe
—associatedwithchestpain,butisalwaysexacerbatedbyexertionandrelievedwithnitroglycerinor
withcardiacstentingandcoronaryarterybypassgraft(CABG)whentheseinterventionsareappropriate.
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Donotmissthediagnosisofcardiaccephalgia.
ThisisanuncommonbutDONOTMISSdiagnosisandshouldbeconsideredinolderpatientswith
new-onsetheadacheandsignificantcardiovascularriskfactors.Itiswisetoobtainaresting
electrocardiogram(ECG)andastresstestinsuchpatients,particularlypriortoprescribinganytriptans.
Post-traumaticHeadache
Ifheadacheonsetoccurswithin7daysofaheadinjury,orwithin7daysofregainingconsciousness
followingaheadinjury,theheadacheisconsideredtobepost-traumatic.Riskfactorsincludeyounger
age,priorheadachehistory,and,paradoxically,milderdegreesofheadtrauma(i.e.,traumaassociated
withonlytransientamnesiaandbriefifanylossofconsciousness).Theheadachecharacteristicsare
variableandcanresemblebothmigraineandtensiontype.Thekeytodiagnosis,therefore,istoelicita
historyofantecedenttrauma.Lightheadedness,mildcognitiveslowing,andinsomniaarecommon.
Considerationshouldbegiventoimagingthesepatientstoruleoutanunderlyingsubduralbleedor
hemorrhagiccontusion.
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Treatmentissymptomatic.Iftheheadachesoundslikeamigraine,treatthemigraine;ifitsoundslikea
tension-typeheadache,treatthetension-typeheadache.Amitriptylineprophylaxisworkswellforbothand
isthedrugthat’sbeenbeststudiedinthispatientpopulation.Mostpatientswillfullyrecoverwithinafew
months,althoughtherarepatientmayhavesymptomsthatneverresolve.
Ahistoryoftraumaisthekeytodiagnosingpost-traumaticheadache.
Box3.12HeadacheinPregnancy
Headacheinpregnancyisawholedifferentballgame.Pregnancyputswomenatriskfora
plethoraofconditionsthatcanpresentwithheadache,somelife-threatening,andthusany
neworworseningheadachethatoccursduringpregnancymustbetakenseriously.Itcan
seemlikethediagnosticpossibilitiesareendless,butlet’sbreakthemdowninto5
categories.ThesearetheDONOTMISSpotentialcausesofheadacheinpregnantwomen.
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Therearemanycausesofheadacheinpregnancyandyouneedtoknowthemall.
1. CerebrovascularDisorders:Pregnancyisa hypercoagulable stateandthusincreasestheriskofvascular disorders
suchas venoussinusthrombosis andacuteischemicorhemorrhagicstroke.Theriskofsubarachnoidhemorrhage,
PRES, and reversible cerebral vasoconstriction syndrome (see Chapter 2) are also increased during pregnancy,
especiallyinthethirdtrimesterandpostpartumperiod.
2. Space-OccupyingLesions: Previously unrecognized Chiari malformations (see Box 3.11), colloid cysts (see page
400), and other tumors canmake themselves knownduring labor due to increased pressure caused bysustained
Valsalvamaneuvers.Meningiomasalsohaveatendencytogrowrapidlyduringpregnancy.Themechanismbehindthis
is unclear, but changes in blood flow dynamics, as well as hormone-mediated cellular proliferation, have been
hypothesized.
3. Disorders Relatedto HighBloodPressure: Gestational hypertension can cause both preeclampsia/eclampsiaand
PRES.
4. DisordersRelatedtoChangesinIntracranialPressure:Idiopathicintracranialhypertensionoftenworsensinpregnancy
due to relatively rapid weight gain. Epidural anesthesia also creates a risk for post-dural puncture (low-pressure)
headache,aswellaspneumocephalus(entryofairintothebrain).
5. PituitaryApoplexy:Thiscanpresentasasuddenandsevereheadacheduetohemorrhageintoapreexistingpituitary
lesionorenlargedpituitarygland.
Thegoodnewsisthatwecanscreenfornearlyallofthesewiththreeimagingstudies(none
ofwhichrequiregadolinium,andallofwhichcanbedoneinjustonetripdowntothe
scanner):
MRI (to lookat thebrain parenchyma,to ruleoutPRES,ischemicstroke, bleeds, and
underlyingstructurallesions)
MRA (to lookat theintracranial arteries, to rule out dissection and reversiblecerebral
vasoconstrictionsyndrome)
MRV(tolookatthevenoussinuses,toruleoutvenoussinusthrombosis)
Allofthissaid,notallpregnantpatientswithheadacheneedtobeimaged.Forinstance,ifa
pregnantpatientwithahistoryofmigrainepresentswithaslightlymoresevereheadache
thanisnormalforher,butonewhichisotherwiseidenticaltohernormalmigraines,you’re
probablyoknotorderinganyimagingstudies.Butthebartoscanpregnantpatientsis,for
obviousreasons,significantlylowerthannon-pregnantpatients,andifyou’reatallunsure,
scan.
Assumingthescansarenegativeandyouhaveruledoutanypotentialsecondaryor
dangerousetiologies,optionsforfirst-linetreatmentforheadacheinpregnancyinclude
acetaminophenandmetoclopramide.
Interestingly,patientswithmigrainetendtohavefewerheadachesduringpregnancy,
particularlyduringthesecondandthirdtrimesters,thancomparedtotheirpre-pregnancy
baseline.Itisalsonotuncommontodevelopnewmigraineorevennewaurasymptoms
duringpregnancy.
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TheGeneralApproachtotheHeadachePatient
We’vejustgonethroughapanoplyofheadachetypesanditallmayseemmorethanalittleoverwhelming.
Butinmanyinstances,thediagnosiswillbeobviouswithinminutesoflisteningtoyourpatientdescribe
hisorhersymptoms.
Nomatterwhat,however,don’tbetooquicktojumptoadiagnosis.Inparticular,knowyourredflags
—SNOOP2—cold.Thesearethediagnosesyoudon’teverwanttomiss.Inadditiontoyourhistoryand
examination,thereareonlyafewtoolsyouwillneed—imaging,CSFanalysis,andsomelaboratory
studies.Don’tordertheseindiscriminately.Theyareexpensive,time-consuming,andanxiety-provoking,
andwithyournewfoundclinicalacumen,frequentlyunnecessary.
Herearesomequickexamplesthatwillillustratethegeneralapproachtothepatientwithheadache:
PatientApresentstoyourofficeforthefirsttimetoestablishcare.Onyourreviewofsystems,she
reportsthatshegets“normalheadaches”onceinawhilewhenshe’stired,dehydrated,orstressedout.
Sheusuallydoesn’thavetotakeanymedicationbutoccasionallyshe’lltakeanibuprofenandthepain
goesaway.Herexaminationisnormal,andyoutellherthatshehastensionheadaches.Ibuprofenisan
appropriatetreatment,aslongassherestrictsitsusetolessthantwiceperweek.
Aquickclinicalpearl:ifaheadachebringsapatienttoyourofficeor,especially,totheED,itisinall
likelihoodnotatensionheadache.Theseareconsidered“normal”bymostpatientswhogetthemand,by
definition,arenotdebilitating.PatientAlikelywouldnothavecometoseeyouforherheadache;shejust
happenedtomentionit(sinceyouasked).
PatientBpresentswithinfrequentbutsevereheadachesforwhichshe’shadtomissadayortwoof
work.Herheadachesareusually,butnotalways,left-sidedandassociatedwithnauseaandlight
sensitivity.Hermothergetssimilarheadaches.Shefeelsbetterwhensheliesdowninadarkroom,and
ibuprofenoracetaminophenhelpbutoftendonotfullyresolvethepain.Herexaminationisnormal,and
youdiagnoseherwithepisodicmigrainewithoutaura.Givenhernormalexamination,familyhistory,
andclassicmigrainehistory,thereisnoneedforimaging.Youprescribesumatriptantotakeatheadache
onsetandtellhershecantakeitincombinationwithanNSAIDformaximaleffect.Youaskhertokeepa
headachediaryforyoutoassessatyournextvisitanddiscusstheimportanceofregularexerciseand
maintainingaregularsleepschedule.
PatientCcomestotheEDafter5daysofexcruciatingheadaches.Hetellsyouthathe’shad“normal”
headachesinthepast,butnothinglikethis.Theseheadachesareright-sided,sharp,andsoseverethathe
tellsyouhewouldratherdiethancontinuetoexperiencethem.Theyseemtoalwayscomeonjustafterhe
eatsdinner,lastaboutanhour,andareassociatedwithrighteyetearingandadroopyeyelid(ptosis).He
feelsfinerightnowandhisexaminationisnormal.Althoughhishistoryisconsistentwithcluster
headache,obtaininganMRIwithoutcontrastwhileintheEDisreasonable,giventhesuddenchangein
headachecharacteristicsandreportedfocaldeficit.Hisscanisnormal,andyoudischargehimona
steroidtaper,withaprescriptionforsubcutaneoussumatriptanandclosefollow-upinyouroffice.
PatientDcomestoyourofficewithathrobbing,right-sidedheadachethat’sbeengoingonfor5days.
Thepainisnoticeablyworseatnightbutneverfullygoesaway.Shehasn’thadmuchofanappetitebut
doesreportthatforthepast2weeksshehasfeltadeepacheinherjawwhenshechews.Sheisillappearing,butherexamination,includinghervision,isnormal,withtheexceptionofmoderatetenderness
topalpationoverherrighttemporalartery.YousendoffanESRandCRP,bothofwhichreturnelevated.
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YoutellheryoususpectshehasGCA,startheronempiricsteroidsandreferherforurgenttemporal
arterybiopsy.
PatientEpresentstotheEDwithasudden-onsetheadachethatbeganabout12hoursearlier.Shegets
headachesfrequentlybutsaysthisoneistheworstheadacheshe’severhadandthatshe’snowseeing
doublewhenevershelookstotheleft.Onexamination,youimmediatelynoteptosisofherrighteye,then
discoverthatherrightpupilisdilatedandshecannotadductherrighteyeacrossmidline.HerheadCT,
doneonarrivaltotheED,showsnoblood,butthisdoesnotreassureyou:asyounowknow(seeChapter
2),thesensitivityofCTforasubarachnoidbleeddramaticallydecreasesmorethan6hoursaftersymptom
onset.YoutelltheEDthatsheneedsanurgentlumbarpuncture,whichdemonstratessignificantlyelevated
redbloodcellsthatdon’tdilute.HerCTangiogramshowsarupturedposteriorcommunicatingartery
aneurysm(likelycompressingherthirdcranialnerve,causingthefocaldeficitsyoufoundon
examination),andsheiswhiskedofftotheoperatingroomforendovascularcoilingofheraneurysm.
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