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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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IfyouknowthemnemonicSNOOP2,youwon’thavetosnooparoundtoquicklyidentifypatientswhose
headacherequiresurgentattention.
SystemicSymptoms(S):Whenheadacheisassociatedwithfatigue,weightloss,orfever,youmust
considerunderlyingsystemicorinfectiousetiologies.Inflammationofthemeninges(meningitis),the
brainparenchyma (encephalitis), or theintracranial vessels (vasculitis) are important do-not-miss
diagnoses.
Neurologic Signs and Symptoms (N). Headache associated with focal neurologic deficits is
alwaysaredflag.Ischemicstroke,intracerebralhemorrhage,andmalignancy(alongwithmanyother
vascular,neoplastic,infectiousandinflammatoryetiologies)mustbeconsidered.Butitisimportant
—and perhaps surprising—to note that migraine is actually the most common etiology when
headachepresentswithnewfocalfindings.Moreonthatlater.
Onset(O).Thunderclapheadaches—headachesthatreachmaximalintensitywithin60secondsof
onset—don’t always reflect an underlying catastrophe. Sometimes they are just really bad
headaches. That said, they must always be taken seriously and emergently evaluated to rule out
subarachnoidhemorrhage(SAH).OnceSAHhasbeenexcluded,thedifferentialremainsbroadand
includes other potentially life-threatening disorders such as reversible cerebral vasoconstriction
syndrome(RCVS,seepage87),andcerebralvenoussinusthrombosis(CVST,seepage85).
Box3.1ThunderclapHeadache:DifferentialDiagnosis
VascularCauses Non-VascularCauses
Subarachnoidhemorrhage Spontaneousintracranialhypotension
Intracerebralhemorrhage Colloidcystofthethirdventricle
Vertebralorcervicalarterydissection Meningitis
Cerebralvenoussinusthrombosis Primaryexerciseheadache
Reversiblecerebralvasoconstrictionsyndrome Primarysexualheadache
Hypertensiveemergency Primarycoughheadache
Pituitaryapoplexy Primarythunderclapheadache
Cardiaccephalalgia
Don’tworry,youwillsoonlearnallyouneedtoknowaboutthediagnosesabove.
OlderAge(O).Becausemostprimaryheadachedisorderspresentinyoungerpatients,anew-onset
headacheinapatientolderthan50raisesaredflagforunderlyingpathology,includingneoplasms,
infections,andinflammatorydisorderssuchasgiantcellarteritis(GCA,seepage112).
Positional(P). Headachesthatworsenwhen thepatient liesdownorstands upraiseconcern for
abnormalitiesinintracranialpressure(ICP).
PatternChange(P).If you remembernothing else fromthischapter,remember this:people who
get headaches will get headaches. Almost any illness, whether neurologic or systemic, including
tumors, infections, anemia, thyroid disease, and so many others, can present as worsening of a
preexisting headache syndrome in patients already diagnosed with a primary headache disorder.
Therefore, any change in the pattern of the patient’s “usual” headache (frequency, severity, or
character) should always be taken seriously and should prompt close monitoring and further
evaluationforpotentialsecondaryetiologies.
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PrimaryHeadacheDisorders
Migrainesandtension-typeheadachesarebyfarandawaythemostcommoncausesofheadache.Ifyou
masterthepresentationandmanagementofthesetwoconditions,youwillbehelpingcountlesspatients
avoidneedlessimagingandmisguidedtherapies.
Migraine
Oddsare,ifyouyourselfdon’tsufferfrommigraineheadaches,youknowmanypeoplewhodo.The
classicmigraine—asinthecaseofAnnaabove—isaunilateral,throbbing,andoftendisablingheadache
thatisassociatedwithnausea,oftenvomiting,andsensitivitytolightandsound(photo-andphonophobia,
respectively).
Themoreup-to-datewayofthinkingaboutmigraine,however,isasitsownphenotype,meaninga
constellationofsymptoms(whichusually,butnotalways,includesanactualheadache)thatalsohas:
apredilectionforaspecificdemographic(femalesintheir20sand30s),
astronggeneticcomponent(heritabilityisestimatedtobebetween30%and60%),and
a predisposition to other medical conditions (such as hypercoagulability and stroke; these
connectionsarestillbeingactivelysortedoutandstudied).
Etiology.Whatcausesmigraine?Aprimaryvascularetiology,longbelievedtoberesponsible,isno
longertheacceptedtheory.Instead,thepathophysiologicbasisisthoughttobeanelectricalphenomenon
calledcorticalspreadingdepression,inwhichawaveofneuronaldepolarizationresultsinbrief
neuronalactivationandvasodilationfollowedbymoresustainedneuronalhypoactivityand
vasoconstriction.Thisself-propagatingwaveactivatestrigeminalsensoryafferentnervefiberswhichin
turncausethereleaseofvasoactiveandproinflammatorymediators(includingcalcitonin-gene-related
peptide[CGRP],atargetofnewermigrainetherapies)inthepain-sensitivemeninges.Whydoesthis
occurinsomepeopleandnotinothers?Westilldon’tknow.
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Inmigraineheadaches,awaveofcorticaldepolarizationpropagatesatapproximately3mmperminute
andisthoughttobethepathophysiologicbasisofmigraine.
ClinicalFeatures.Migrainestypicallylastanywherefrom4to72hours,andcanbedividedintofour
phases:
1. Prodrome. This can begin hours todays prior to the onset of the headache and consists of nonpainful symptomssuch as fatigue,
yawning,irritability,foodcravings,andfrequenturination.Peoplewithmigrainetypicallyknowtheirprodromalsymptomswell.
2. Aura. Migraine auras are focal, fully reversible neurologic symptoms that come on gradually, progress over several minutes, and
typically resolve within an hour. Not all people with migraine experience an aura; migraine with aura is much less common than
migraine without. The symptoms ofanaura are classically“positive” (e.g., colorful visual phenomena or tingling of an arm or leg)
followedbysymptomsthatare“negative”(e.g.,avisualfieldcutornumbnessofanarmorleg);pathophysiologicallythismakessense
because the wave of cortical spreading depression induces a transient neuronal activation followed by a more sustained period of
hypoactivity. However, virtuallyany focal neurologic symptomyou can thinkof, including word-finding difficulty,vertigo,andmotor
paralysis, canpresentas anaura. The aura isoften, butnot always,followedbya headache. Whenthere isnoheadache—inother
words, when we skip phase 3 (see next page)—the patient can be diagnosed (in the most recent iteration of the International
Classification of Headache Disorders) with “aura without headache.” Importantly, this is still considered a migraine. As youmight
suspect,these atypical migraines can be difficult to recognize, and can only be diagnosed after careful exclusion of other possible
etiologies.
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Box3.2MigrainewithAuraversusMigrainewithoutAura:ImportantClinicalImplications
Thisdis tinctionisanim portantonebecaus emigrainewithauraappearstopredis posetoanumberofconditions—suchas is chemicstroke
andvenousthromboembolism—thatmigrainewithoutauradoesnot.Thereas onsunderlyingthes eassociations arenotunderstood.Smoking
and estrogen-containing therapy(in contraceptive pills or horm one replacem ent treatments, for instance) seem to increas e these ris ks in
patients with m igraine with aura.Although not abs olutelycontraindicated in women withmigraine with aura, estrogen-containing horm onal
therapyshouldbepres cribedwithcautionandinthelowes tpossibledos e.Theabsoluteris kofstrokeandthromboembolicdis easeiss m all
inpatientswithm igraine withaura,particularlyinwomen whodon’ts m okeandwhotakelow-doseformulations ofestrogen,butthebenefits
and risks of thes e m edications s hould always be dis cussed prior to initiation (and, as always, s m oking cessation s hould be strongly
encouraged!).
Low-dos econtraceptivepills,althoughnotabs olutelycontraindicated,shouldbeprescribedwithcautioninpatients
withahistoryofmigrainewithaura.
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FortificationSpectra.Als oknownasteichopsia,this is acom m onformofvisualaura.Itappearsasashim m eringand
oftenbrightlycoloredsetofjaggedlines thatgraduallys preadacros s thevisualfield.Thenamecom es from its
resem blancetothebattlementsorwalls ofoldfortress es.
3. Headachephase.ThePOUNDmnemonicishelpfulforrememberingthetypicalcharacteristicsofmigraineheadache.Thepresence
of4 outof 5ofthese features can accurately predict the diagnosis of migraine and often eliminates the need for furtherdiagnostic
workuporimaging:
Pulsatile(i.e.,throbbing)qua lity
One-da y duration
Unilatera llocation
Nause aorvom iting
Disablingintensity
Othersymptomssuchasphotophobiaandphonophobia,neckpain(whichoftenleadstotheincorrectdiagnosisoftension-ty p eheadacheoroccipitalneuralgia,see
pages104and108),andotherfeaturesthatoftenleadtothemistakendiagnosisofasinusinfection—suchasnasalcongestion,runnynose,facialpain,andtearing—
arealsofrequently p resent.
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Mnem onics as ide,migraineheadacheshurt!
4. Postdrome. This term describes the period between headache resolution and when the patient feels 100% back to normal. These
symptomsareoftenthesameastheprodromalsymptoms,andcanalsolastforhourstodays.
Box3.3MRIChangesAssociatedWithMigraine
Somewherebetween10%and40%ofmigraineurshavewhatarecolloquiallyknownas
“migrainespots”ontheirMRI:smallnonspecificwhitematterlesionsthat,asfaraswe
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know,meanabsolutelynothingintermsofmigraineprognosisortheriskoffutureneurologic
problems.Webringthemupfortwoimportantreasons:
1. They can look similarto thewhite matterlesions caused byischemic vasculardisease or multiple sclerosis (MS).
Differentiatingtheseetiologiesdependslargelyonclinicalcontext(andknowingthetypical lesionlocationsofMS;see
page242).
2. They are one of manyreasons tothink before youscanevery patient with a headache.Although these lesions are
ultimatelyalmostalwaysdistinguishablefromdemyelinatingdiseasessuchasMS,theycanbeeasilyconfusedbylessexperiencedphysicians,andcanthenleadtounnecessaryandexpensivediagnostic evaluation.Perhapseven more
importantly,manypatientswithheadacheareunderstandablyanxiousabouttheirsymptoms,andtellingthemthattheir
brainsare,ineventheslightestway,“abnormal”doesnothingtoeasetheirconcern.
Whitematterlesions—migrainespots—seenontheMRIofapatientwithmigraines.(Courtesyof
JonathanHoward.)
Treatment
LifestyleManagement.Migrainetreatmentbeginswithlifestylemodifications,oftenreferredtoas
“headachehygiene.”ThemnemonicSEEDS(sleep,exercise,eat,diary,andstress)canhelpyou
rememberthebasics.Stabilityiskey:patientsshouldstrivetoattainaregularamountofsleepeachnight,
exercisefrequently,notskipmeals,maintainadequatehydration,andavoidtheirmigrainetriggers(red
wine,agedcheesesandsucralosearecommonexamples).Caffeineintakeshouldbelimitedoratleast
maintainedatastablelevelofconsumption.Keepingtrackof“headachedays”inadiarycanbeusefulto
monitorthepatient’sresponsetolifestyleandpharmacologicinterventions.
Box3.4“HeadacheDays”
Thebestwaytotracktheimpactoflifestylechangesonheadachesisbyaskingaboutand
recording“headachedays”(i.e.,daysduringwhichthepatientexperiencedheadache),as
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opposedtoheadachesthemselves.Manypatientshaveheadachesthatlastmultipledaysat
atime,andthus,whentheyareaskedhowmanyheadachesthey’vehadoverthecourseof
amonth,theymaysayonlytwoorthree—whichdoesn’tsoundsobad—wheninfactthey’ve
beensymptomaticforsignificantlylonger.
Unfortunately,asinthecaseofourfirst-yearsurgicalresident,Anna,whohasbeenwakingupat4am
topreroundonherpatientsandpullingregular24-hourovernightcallsinthehospital,lifestylestabilityis
notalwayspossible.Whenlifestylemodificationsaren’tsufficient,weturntomedications.
Migrainemedicationsaredividedintotwotypes:acutemedications(alsocalledrescueorabortive
medications),whicharetakenasneededtoacutelytreataheadache,andpreventive(alsocalled
prophylactic)medications,whicharetakendailytoraisethethresholdfordevelopingaheadacheand
decreasethenumberofheadachesovertime.
AcuteTreatment.AcetaminophenandNSAIDsarethemostcommonlyusedinitialtherapiesfor
migraine.Triptansareconsideredsecondlineandarefrequentlyprescribedforpatientswhodonot
respondtoanti-inflammatories,cannottoleratethem,orrequireincreasinglyhighdoses(whichcanbe
harmfultotheliver,kidneys,andgastrointestinal[GI]tractandcanpotentiallytriggermedication-overuse
headache;seepage111).
Triptanswerethefirstmigraine-specificmedications,andremainamongthemostwidelyusedabortive
treatments.Theyactasserotoninreceptoragonists(specificallyonthe5-hydroxytryptaminereceptors,
HT1B,and5HT1D:the“B”receptorvasoconstricts—youcanremember“B”forBloodvessels—andthe
“D”receptorinhibitsthetrigeminalnervebranchesresponsibleforpaintransmission—“D”forDamn
nerve).Interestingly,stimulationofthesereceptorsalsoseemstoinhibitreleaseofCGRP(seepage102)
andotherproinflammatorycytokines.
Triptans,whichcomeinoral,nasalspray,andinjectableformulations,aremosteffectivewhentakenat
theonsetofheadachepainandoftenworkbestwhencombinedwithanNSAID.Becauseoftheir
vasoconstrictingproperties,theyarecontraindicatedinpatientswithsignificantvasculardisease,
includingcoronaryarterydiseaseandperipheralvasculardisease,andparticularlyshouldbeavoidedin
patientswithapriorhistoryofstrokeormyocardialinfarction.
TwosmallmoleculeCGRPantagonists(ubrogepantandrimegepant)havealsobeenapprovedforacute
migrainetreatment.Currently,thesearemostoftenusedinpatientswithaninsufficientresponseor
contraindicationtotriptans.Unliketriptans,whichshouldbelimitedto2–3perweektoavoidpotential
reboundheadaches,thesemedicationscanbetakeneverydayifneeded.
Antiemetics,suchasmetoclopramideandchlorpromazine(manyofthesealsohaveantimigraine
properties),andmusclerelaxantssuchastizanidinearealsousedtotreatmigraine.
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Molecularmodelofsumatriptan,thefirsttriptanthatwasavailableforclinicaluse.
PreventiveTreatment.Thereisalonglistofmedicationsthatcanworkasmigraineprophylaxis,noneof
which—upuntiltheadventoftheCGRPinhibitors(seeBox3.5)—weredevelopedspecificallyfor
migraine.Theefficacyofpreventivedrugsisnot100%:overall,approximately50%ofpatientswill
experiencea50%reductioninheadachefrequencywithanyofthesedrugs,andthus,expectationsmustbe
managedaccordingly.Thechoiceofmedicationistypicallymadebasedonthesideeffectprofile.
Nospecificnumberofheadaches“qualifies”apatientforaprophylacticmedication,althoughthe
AmericanHeadacheSocietysuggestsconsideringprophylactictreatmentforpatientswithfourormore
debilitatingheadachedayspermonth(or6ormoreifnotdebilitating).Butthebestthingtodoistolisten
toyourpatients,anddecideifthedisabilitycausedbytheirheadachedisordermeritstherisksofstarting
adailymedication.Commonlyusedmedicationsarelistedonthefollowingpage,groupedbytheir
originalintendeduse.
InthecaseofourpatientAnna,prophylaxisshouldbeofferedbecauseofherincreasingheadache
frequency(likelyduetostressandlackofsleep,variablesshecannotcontrolfornow)andthenegative
impactthatherheadachesarehavingonherday-to-daylife.Thespecificmedicationchoiceshouldbea
jointdecisionmadewithherparticularhistoryandpreferencesinmind.
Thetablebelowsummarizessomeofthemorecommonly-usedmedications;itisnotacomprehensive
list.Notethatnoneofthesemedicationsareproventobecompletelysafeinpregnantwomenand,assuch,
arealmostalwaystaperedoffpriortopregnancyplanning.
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