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Ростовский Государственный Медицинский Университет
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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_186_библиотеки_им_акад_М_И_Перельмана

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2. SecondaryTN.ThisreferstoTNcausedbyanunderlyingdisease,suchasherpeszosterinfectionandmultiplesclerosis(MS)lesions involvingthetrigeminalnerverootentryzoneinthepons.Lesscommonaretumorslocatedatthecerebellopontineangle,arteriovenous malformations,andaneurysms.
3.  Idiopathic TN. Whentheworkup forTN isentirelynormal,it isreferredtoasidiopathic.Notethatcontactbetweena bloodvessel andthetrigeminalnerverootisacommonfindinginhealthyindividuals;ifthisisseenonMRIbutwithoutmorphologicalchangesin thetrigeminalnerve,TNisconsideredidiopathic.
MRIandMRangiography(MRA)isrecommendedforallpatientsinwhomyouneedtoruleouta secondarycause,buteveninpatientsathighrisktheyieldisrelativelylow(asecondarycausewillbe foundinnomorethan15%to20%ofcases).
CarbamazepineandoxcarbazepinearecommonlyusedtreatmentsforclassicalandidiopathicTN. Oxcarbazepinehasfewersideeffects,butthereislessevidencesupportingitsefficacy.Lamotrigine, topiramate,valproicacid,andgabapentinarealsoused.Unfortunately,theresponsetomedicationoften decreasesovertime.Surgicalintervention(eithermicrovasculardecompressionorgammaknifetherapy) forclassicalTNissecond-linetherapy,whenfeasible.SecondaryTNismanagedbytreatingthe underlyingcause.
Box3.8TrigeminalNeuralgiaVersusSUNCT/SUNA
SUNCT/SUNA TN
Cranialnerve5(CN5)divisionmostoftenaffected V1 V2andV3 Presenceofautonomicfeatures Yes No Presenceofrefractoryperiod No Yes
OccipitalNeuralgia.Occipitalneuralgiaischaracterizedbysharp,paroxysmalattacksofpainlocalized tothegreateroccipitalnerve(GON),lesseroccipitalnerve(LON),orthirdoccipitalnerve.Painisoften unilateralbutcanbebilateralandisfeltintheneckrisinguptotheposteriorscalp.Mostpatientswith neckpainwillnothaveoccipitalneuralgia,butyoushouldconsiderthispossibilitywhentheattacksare short,sharp,andsevere(unlikethefar-more-commoncervicalstrainandsprain,whichismorepersistent andusuallypositional).Thediagnosisrequirestendernessorallodyniaoverthesymptomaticnerve,as wellaseliminationofpainwithanerveblockovertheaffectedarea(whichisalsothetreatmentof choice).Mostoften,occipitalneuralgiaisduetoentrapmentoftheGONalongitspathfromtheC2 vertebraetothetrapeziusaponeurosis.Secondarycausesincludeinfection(suchasherpeszoster)and neoplasm.
https://t.me/med1917
Irritationofthegreater,third,andlesseroccipitalnerves.
Box3.9LessCommon(ButImportant!)PrimaryHeadacheDisorders
Thesearediagnosestotuckinyourbackpocket,readytopulloutonlywhennecessary. Usuallythehistorywillgivethediagnosisaway,butnomatterhowgoodthehistory,these arediagnosesofexclusion:youmustalwaysconsiderandoftenevaluateyourpatientfor othersecondarycauses.
PrimaryStabbingHeadache:Short,irregularjabsofpainthatusuallylast1to2seconds, withoutanyassociatedmigrainousorautonomicfeatures.Thelocationofthepaincanbe fixedoritcanchange.Thistypeofheadacheismostcommoninchildren.Treatmentis usuallyunnecessary,butindomethacinisthefirst-lineoptioninadults.
NummularHeadache:Thispresentswitheitherepisodicorcontinuouspainconfinedtoa coin-shapedareaonthehead(nummularactuallymeanscoin-shaped).Thisdiagnosis alwayswarrantsaCTorMRItoruleoutunderlyingcranialbonelesions.Acetaminophenor NSAIDsarefirst-linetreatment.
https://t.me/med1917
Distributionofpaininapatientwithnummularheadache.
PrimaryHeadacheAssociatedwithSexualActivity(previouslyknownasOrgasmic Headache):Exactlywhatitsoundslike,thistypeofheadacheishypothesizedtobedueto
briefvasospasmduringorgasm.Theheadachefeaturesarevariable:suddenorgradual onset,unilateralorbilateral,lastingminutestohours.Subarachnoidhemorrhage,reversible cerebralvasoconstrictionsyndrome,arterialdissection,andothervasculardisordersmustbe excluded;imagingisalmostalwaysnecessary.Indomethacin30to60minutesbeforesexual activityisthepreferredtreatment.
PrimaryExerciseHeadache:Apulsatingheadachethatisconsistentlyprecipitatedby sustainedexercise.Workupissimilartotheabove.Thecourseistypicallyself-limited(it resolveswithinafewmonths).Treatmentinvolvestemporaryabstentionfromexerciseor,if thatisn’tpossible,indomethacintakenimmediatelypriortoworkingout.
https://t.me/med1917
PrimaryCoughHeadache:Asudden-onsetheadachethatisconsistentlyprovokedby coughing.Itisparticularlyimportanttoruleoutstructurallesionssuchasposteriorfossa tumorsorChiari1malformations(seeBox3.11).Oncepotentialunderlyingsecondary etiologieshavebeenexcluded,treatmentiswithindomethacin—andtreatingthecough.
EpisodicVersusChronicHeadache
Somepatientsdevelopapersistentheadachepatternthattheysimplycan’tshake.Chronicmigrainecan bediagnosedwhenapatientreportsaheadachethatispresent15ormoredayspermonthformorethan threeconsecutivemonths.Inaddition,theheadachemustmeettheacutemigrainecriteriaonatleasteight ofthosedays.Episodicmigraineisanythinglessfrequentthanthis.Patientswithchronicmigrainehave generallytransitioned—forunknownreasons—fromepisodictochronicmigraine.
Otherformsofchronicheadacheinclude:
Chronic tension-type headache: Like chronic migraine,this mustbe present15 ormoredaysper monthformorethan3months. Newdailypersistentheadache(NDPH):NDPHisaheadachethatbeginsonedayoutofnowhere, thenjustdoesn’tgoaway.Patientswilloftenbeabletotellyouexactlywhattheyweredoingwhen theheadachebegan(usuallysomethingbenignlikegardeningorwatchingTV).Unfortunately,NDPH isnotoriouslydifficulttotreat. Medicationoveruseheadache(MOH,seebelow)Hemicraniacontinua(discussedaboveonpage106)
Thedistinctionbetweenepisodicandchronicheadachemattersmostintermsoftreatment.Oncea headachedisorderhas“transformed”fromepisodicintochronic,itbecomesmuchmoredifficulttotreat —youcanthinkofitastryingtoputoutaforestfireasopposedtoblowingoutacandle.Botoxandthe CGRPmonoclonalantibodiesarecurrentlytheonlyFoodandDrugAdministration(FDA)-approved treatmentsforchronicmigraine.Preventivetreatment(seepage101)canbebeneficialforthesepatients.
SecondaryHeadacheDisorders
WehavenowarrivedatthecategoryofheadachesforwhichtheSNOOP2redflagsweredevised.Notall oftheseareemergenciesbutsomeare.Severalofthemostseriousanddangeroussecondarycausesof headache—subarachnoidhemorrhage(seepage81),encephalitis,meningitis,andbrainmalignancies— arediscussedelsewhereinthistext.Herewewillfocusonsomeothersecondaryetiologiesofheadache youmustknowabout.
MedicationOveruseHeadache(MOH)
https://t.me/med1917
Headachemedicationscomeinallsortsofshapesandcolorsandmanycan—ifoverused—cause reboundheadachesknownasmedicationoveruseheadaches.
MOHisnotanemergency.Itisdefinedasaheadacheoccurring15ormoredayspermonthduetoregular overuseofsymptomaticheadachemedication(s)formorethan3months.Itisalmostalways superimposedontopofanotherheadachedisorderandoften,butnotalways,resolveswithgradual removaloftheoffendingmedication.Thehighest-riskmedicationsareopioids,butalbital-containing analgesicsandaspirin-paracetamol-caffeinecombinationpills,butNSAIDs,acetaminophen,andtriptans havebeenimplicatedaswell.
ThevalidityofMOHasitsownclinicalentityisbeingactivelydebated.Manyarguethatthisdiagnosis placesblameexclusivelyonthepatientwhen,inreality,thesymptomsmayinsteadbeaconsequenceof theprovider’sinabilitytoadequatelytreatthepatient’spain.
Box3.10
ApartfromthosepainmedicationsthatcancauseMOH,therearenumerousotherdrugs thatcancauseheadacheasasideeffectoftheiruseforanothercondition.Foremostamong thesearehormonalcontraceptives,beta-adrenergicagonists,stimulants(e.g., amphetamines),nitrates(almostuniversally),andphosphodiesteraseinhibitors(usedtotreat erectiledysfunction).Otherdrugsandsubstancescancauseheadachesduringwithdrawal, suchascaffeineandmanyantidepressants.
GiantCellArteritis(GCA)
Alsoknownastemporalarteritis,GCAisamedium-to-largevesselvasculitisthataffectstheaortaand mostofitsmajorbranches.Diffusevascularinflammationcanleadtoscarring,stenosis,andeventual
https://t.me/med1917
occlusion.GCAisseenalmostexclusivelyinpatientsolderthan50,withapeakincidencebetween70 and80yearsofage.Womenareaffected2to3timesmoreoftenthanmen.Thisdiagnosisshouldalways beconsideredinpatientsover50whopresentwithnew-onsetheadaches.
Theheadacheitselfcanbeunilateralorbilateralandisoftenbutnotexclusivelytemporal.Associated featuresmayinclude:
Systemicsymptomssuchasfever,fatigue,weightloss,andmyalgias; Tendernesstopalpationoverthetemporalartery; Jawclaudication(painandfatiguewithchewingduetoinvolvementofthemaxillaryartery); Polymyalgia rheumatica (whichpresentswith muscle pain,weakness, and stiffness predominantly affectingtheshoulders);and Themostconcerningsymptom—visualloss,typicallyduetoischemiaoftheretinaoropticnerve.
https://t.me/med1917
Aninflamedtemporalarteryinapatientwithgiantcellarteritis(GCA).
Inflammatorymarkers,includingtheerythrocytesedimentationrate(ESR)andc-reactiveprotein (CRP),aregenerallyelevated(theCRPhasasensitivityof>95%),butnormalvalues—whileuncommon —donotexcludethediagnosis.Nevertheless,ifyoususpectthediseaseinanyadultover50,checkan ESRorCRP.
Temporalarterybiopsyremainsthegoldstandardfordiagnosisbutcanbefalselynegativebecausethe inflammationisnotuniformbutrather“skips,”leavingsomeareasofthetemporalarteryunaffected. Bilateralbiopsyimprovesthediagnosticyield.Althoughthediagnosiscanbehardtomake,maintaininga lowthresholdtoscreenforandempiricallytreatGCAisessential,because15%to20%ofpatientswill ultimatelysufferfromrapidandoftenirreversiblevisionlossifnotpromptlytreated.Itisimportantto stressthislastpoint—ifyoususpectyourpatienthasGCAbasedonyourclinicalassessment,start treatmentatonce;donotwaitforthebiopsyresults.High-dosesteroidsarefirst-linetreatment.
https://t.me/med1917
SpontaneousIntracranialHypotension(SIH)
Alsoknownas“low-pressure”headaches,thesearecausedbycerebrospinalfluid(CSF)leakagethrough atearinthedura.Thereisoftenanobviousprecipitatingevent(e.g.,alumbarpuncturethatpokesahole inthedura,epiduralanesthesiathatever-so-slightlymissesitstarget,amotorvehicleaccidentorsports injury)oranunderlyingconnectivetissuedisease(suchasEhlers-DanlosorMarfansyndrome)that predisposestoaflimsydurathatisathighriskoftearing.
Theheadacheisclassically“orthostatic”inthatitworsensonstandingandresolvesonlyingdown,but thisfeaturecanresolvewithtime.WorseningwithanyValsalvamaneuveriscommon,aresultofelevated venouspressurethatforcesincreasedCSFleakagethroughthetear.Otherfeaturescanincludetinnitus (typicallynonpulsatile),nocturnalawakenings,neckpain,andmigrainousfeaturessuchasphotophobia, phonophobia,andnausea.
SIHisdiagnosedbasedontheseclinicalfeaturesinconjunctionwitheitherspecificimagingfindings ordirectevidenceoflowCSFpressureobtainedvialumbarpuncture.However,recentevidencesuggests thatlowCSFpressureisactuallyrelativelyuncommoninthesepatients,andthatlowCSFvolumeis moreimportant;regardless,theutilityofalumbarpunctureisnowdebatable.MRIofthebrain(yes,the brain,eventhoughthesiteoftheleakisusuallyatthelevelofthespinalcord)isusuallythefirstimaging testthatisobtainedandisabnormalapproximately75%ofthetime.
PotentialabnormalitiesonabrainMRIarenumerousandaresummedupbythemnemonicSEEPS:
Subduralfluidcollections Enhancementofthedura(sometimesreferredtoasthepachymeninges) Engorgementofvenoussinuses Pituitaryhyperemia Saggingofthebrainandcerebellartonsildisplacement
MRIofapatientwithspontaneousintracranialhypotension(SIH)shows(A)enhancementofthedura(red arrows),(B)pituitaryhyperemia(bluearrow),and(C)mildsaggingofthebrain(yellowarrow).(Modified fromLouisED,MayerSA,NobleJM.Merritt’sNeurology,14thed.WoltersKluwer,2021.)
Spinalimaging(eitherwithtraditionalMRIorCTmyelography,aninvasiveimagingtechniquein whichcontrastisinjectedintotheCSFspace)isalsooftenobtainedinordertohelpvisualizethetear.
Dependingupontheseverityofthesymptoms,treatmentcanbeginconservativelywithbedrest,
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caffeine(e.g.,2to3cupsofcoffee2to3timesaday;itmayworkviaitsactionasanarterial vasoconstrictor),hydration,andtime.Ifthisdoesn’twork,you’llneedtoarrangeforanepiduralblood patch,aprocedurethatinvolvesepiduralinjectionofautologousbloodtotamponadetheleakand hopefullyrepairthetear.
Box3.11ChiariMalformations
Chiarimalformationsareanatomicabnormalitiescharacterizedbythedownward displacementofthecerebellum,eitheralone(Chiari1)ortogetherwiththelowerbrainstem (Chiari2),belowtheforamenmagnumandintothespinalcanal.
Chiari1malformations(whicharemostrelevanttothischapter)areoftenasymptomatic,but incertaincasescancauseheadaches,mostoftencharacterizedbyprominentoccipitalpain andnecksoreness.Lowercranialnervepalsiescausingdysarthria,nystagmus,hoarseness, and/orsleepapneacanalsooccur,aswellassensorylossandevenscoliosisdueto syringomyelia(i.e.,theformationofafluid-filledcystinthespinalcord,commonlyfoundin associationwithChiari1malformations).Ingeneral,symptomsdon’tpresentuntilyoung adulthood.
SpontaneousintracranialhypotensioncancauseasecondaryChiari1(i.e.,brainsagdueto lowCSFvolume)(seeimageConpage114).
Chiari2malformationsareusuallydiagnosedprenatally,sincetheyarealmostalways associatedwithamyelomeningocele(aneuraltubedefectcharacterizedbyprotrusionofa sectionofspinalcordanditsmeningealcoveringthroughthechild’sback).Symptomscan includeweakness,dysphagiaandapneaduetomedullarycompression.Progressive hydrocephalus(duetoobstructionofCSFoutflow)isacommoncomplication.
Theneedforsurgery(usuallywithposteriorfossadecompressionorashunttotreat hydrocephalus)dependsontheextentofcerebellarandbrainstemdisplacementandthe degreeofneurologicimpairment.
PseudotumorCerebri
Pseudotumorcerebriischaracterizedbyaconstellationofsignsandsymptomsthataretheresultof elevatedICP,whichdevelopsbecauseofCSFbuild-upandsubsequentventricularexpansion(otherwise knownashydrocephalus)thatputspressureonthesurroundingbraintissue.Itcanthereforemimicsome ofthefeaturesofabraintumor—hencethename.
Thebestwaytothinkaboutintracranialhypertensionistodivideitinto2categories,idiopathic (colloquiallythecategoryreferredtoaspseudotumorcerebri)andsecondary.
IdiopathicIntracranialHypertension.Idiopathicintracranialhypertension(IIH),asitsnameindicates, hasnoknowncause.Overweightwomenofchildbearingagearethemostcommonlyaffected.Risk factorsincluderecentweightgain,varioussystemicconditions(includinganemia,polycysticovary syndrome,andsystemiclupuserythematosus),andmedications(especiallytetracyclines,growthhormone, glucocorticoids,fluoroquinolones,vitaminAandvitaminAderivativessuchasisotretinoin).
SecondaryIntracranialHypertension.Secondaryintracranialhypertensionisduetoanyprocessthat
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causesexcessCSFaccumulationresultinginelevatedICP.Theculpritsinclude:
1. Anythingthatresultsinblock ageofCSFflowwithsubsequentCSFaccumulationwithintheventricles.Venoussinusthrombosisand jugularveinobstructionblockvenousoutflowfromthebrain,whichisthesameoutflowpathutilizedbytheCSF.Tumorsorothermass lesionscausingventricularoutflowobstruction(i.e.,obstructivehydrocephalus)canalsodothis.
2. AnythingthatresultsindecreasedCSFabsorption.Priormeningitisorsubarachnoidhemorrhagecanresultinscarringandadhesions ofthearachnoidgranulationsthatareresponsibleforCSFresorption.
3. AnythingthatresultsinincreasedCSFproduction.Uncommonbutnotunheardof,choroidplexuspapillomasaretumorsthatsitwithin theventriclesandproduceexcessCSF.
Regardlessofcause,theheadacheassociatedwithintracranialhypertensionispositional,butunlike SIH,itisworsenedbylyingdownandimprovedbystandingup.Migrainousfeaturesarecommon.Other morespecificclinicalfeaturesthatshouldsuggestthediagnosisinclude:
1. Pulsatiletinnitus
2. Transientvisualobscurations, whicharebrief episodesofvisionlossinoneorbotheyes characteristicallyprecipitatedbystanding up, and
3. Cranialnerve(CN6)palsy,thatis,impairedabductionoftheaffectedeye,a“falselocalizingsign”(inthatitcanreflectdysfunctionfar awayfromthelocationsuggestedbytheexamfinding);thesixthnervehasthelongestintracranialcourseofallthecranialnerves,and is,therefore,mostsusceptibletotheeffectsofelevationsinICP.SeeChapter18fordetailsonthecranialnerves.
Diagnosisrequirespapilledemaonexamination(opticnerveswellingduetoelevatedICP)andan elevatedopeningpressureobtainedvialumbarpuncture(over25mmHginadults,28mmHgin children).CSFanalysisisotherwisenormal.AnMRIofthebrainwithandwithoutcontrastandanMR venogram(MRV)mustbeobtainedtoruleoutsecondarycauses.Classicfeaturesofintracranial hypertensiononMRIincludeanemptysella(asaddle-shapeddepressioninthebaseoftheskullwhere thepituitarysits),flatteningoftheposteriorglobesandtorturous-appearing,enhancingopticnerves.The ventriclesandbrainparenchymashouldlooknormal.
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