头痛疾病的国际分类课件

头痛疾病的国际分类InternationalClassificationofHeadacheDisorders-secondeditionICHD-II背景偏头痛的患病率在欧美国家为1500－2000/10万人，发病率为10－15％；在中国，患病率为732.1/10万人，发病率为0.06％但实际是由于中国的诊断标准在许多下级医院不是很明确，许多医师对头痛分类仍然沿用不规范的用语，致使许多病例无法纳入统计。按照神经科医师在临床上接诊的情况，我国的头痛患病人数绝不会与欧美有如此大的差距。头痛疾病分类的历史最早的是60年代两个相似的头痛分类，列出了当时被认可的一些头痛疾患，只能算是描述，而不是诊断标准。1988年国际头痛协会IHS头痛分类委员会首次出版了“头痛疾患国际的分类（ICHD）”，立即被全世界广泛接受并应用于临床。虽然当时的诊断标准基于专家的意见，但随后的研究证明完全可靠有效。而且几乎不需要进一步改进。ICHD-I使得研究的进展，并导致了更完善的ICHD-II的提出。头痛疾病的国际分类4类原发性头痛、8类继发性头痛和另外的2类原发性头痛偏头痛紧张性头痛丛集性头痛及其它三叉自主神经性头痛其它原发性头痛继发性头痛归因于头和（或）颈部外伤的头痛归因于颅或颈部血管疾病的头痛归因于非血管性的颅疾病的头痛归因于某些物质或它的戒断的头痛归因于感染的头痛归因于代谢疾病的头痛归因于颅骨、颈、眼、耳、鼻、鼻窦、牙、口、或其它头面部结构疾病的面部痛归因于精神疾患的头痛颅神经痛，中枢性或原发性面部痛及其它头痛颅神经痛和中枢性疾病有关的面部痛颅神经痛，中枢性或原发性面部痛及其它头痛采用逐级分类法，共有四级8归因于物质或它的戒断的头痛8.1归因于急性物质使用或暴露的头痛

8.1.1一氧化氮前体诱导的头痛

8.1.1.1一氧化氮前体诱导的即刻头痛

8.1.1.2一氧化氮前体的迟发性头痛一、原发性头痛

Part1:TheprimaryheadachesMigrainePrevalenceLipton,2007One-yearperiodprevalenceofmigrainebyageandgenderAmericanMigrainePrevalenceandPreventionStudy1、偏头痛1.1无先兆的偏头痛1.2有先兆的偏头痛1.3儿童周期综合症为前驱的偏头痛1.4视网膜性偏头痛1.5偏头痛合并症1.6很可能的偏头痛1.1无先兆偏头痛的IHS诊断标准至少有满足标准B-D的5次发作每次持续4－72小时（未治疗或治疗无效）头痛至少有下列特征中的两项单侧痛搏动性痛中或重度疼痛因日常体力活动加重或避免此类活动（如走路或爬楼梯）头痛过程中至少伴随下列一项恶心和/或呕吐畏光和畏声不能归因于其它疾患1.1无先兆的偏头痛对小儿，持续1－72小时婴幼儿的畏光畏声可从其行为判断发作频率≥15天/月则诊断为慢性头痛1.2有先兆的偏头痛及其亚型先兆是局灶神经系统体征典型地发生在头痛之前或伴随头痛一起发生，或也可只有先兆而无头痛。先兆通常经5－20分钟发展起来，持续20－60分钟。视觉先兆最为普遍,其次是无力和失语。1.2有先兆的偏头痛1.2.1有偏头痛的典型先兆的头痛

头痛满足无先兆偏头痛的诊断标准1.2.2无偏头痛的典型先兆的头痛

伴随先兆的是轻至重度的紧张性头痛样偏头痛1.2.3无头痛的典型先兆1.2.4家族性偏瘫性偏头痛FHM

先兆必须包括某种程度的偏瘫，且至少有一个亲属有相同的发作1.2.5散发性偏瘫性偏头痛

无家族史1.2.6基底型偏头痛

表明后颅窝受累及，而不是基地动脉1.2有先兆的偏头痛的IHS诊断标准至少2次头痛发作符合B-E能完全逆转的视觉、感觉、或言语症状，但无运动障碍至少满足下列两项同向视觉症状包括阳性体征（如点状色斑或线形闪光幻觉），和/或阴性症状（视野缺损），和/或单侧感觉症状包括阳性体征（针刺感）和/或阴性体征（麻木感）至少一个症状渐渐发展≥5分钟和/或不同症状接连发生。每个症状持续5－60分钟满足无先兆偏头痛诊断标准B-D的头痛在有先兆时发生或在先兆发生后60分钟内发生不能归因于其它疾患1.3儿童周期综合症为前驱的偏头痛1.3.1周期性呕吐综合征1.3.2腹型偏头痛1.3.3良性发作性眩晕1.3.1周期性呕吐

至少５次发作符合标准Ｂ和Ｃ。周期性发作，个别患儿呈刻板性，强烈恶心和呕吐持续１小时至５天。发作期间呕吐至少４次／小时，或至少１小时。２次发作间期症状完全缓解。不能归因于其它疾病。

1.3.2腹型偏头痛

至少５次发作符合标准Ｂ～Ｄ腹部疼痛发作持续１～７２小时（未治疗或治疗不成功）。腹部疼痛具备以下所有特点①位于中线、脐周或难以定位②性质为钝痛或“微痛”③程度为中度或重度腹痛期间至少有以下２项①食欲减退②恶心③呕吐④苍白。不能归因于另一种疾病。

1.3.3儿童良性发作性眩晕

“无先兆多次严重眩晕发作，数分钟到数小时后自行缓解”至少应在５次以上。发作间期神经系统检查和听力、前庭功能正常，脑电图正常。

1.5偏头痛合并症1.5.1慢性偏头痛1.5.2偏头痛持续状态1.5.3无梗塞的持续先兆1.5.4偏头痛性梗塞1.5.5偏头痛诱发的癫痫1.5.1慢性偏头痛偏头痛≥15天/月，持续3个月以上，无药物滥用。如有药物滥用（急性抗偏头痛药物和/或混合止痛药≥10天/月）或普通止痛药服用15天/月，则诊断为药物滥用性头痛8.2，如停药后症状改善，则更支持该诊断。否则诊断为伴可能药物滥用的可能偏头痛。1.5.2偏头痛持续状态尽管经过治疗，头痛仍持续72小时以上。伴虚弱。如不伴虚弱，则诊断无先兆的可能偏头痛。1.5.3无梗塞的持续先兆先兆持续2周以上无梗塞的影像学证据1.5.4偏头痛性梗塞7天内1个或更多个偏头痛先兆不能完全恢复，和/或相关的缺血性梗死的神经影像学定位依据。与其它原因引起的中风的鉴别诊断：神经系统缺损症状必须与先前发作的偏头痛先兆极其相似中风发生在典型的偏头痛发作过程中必须排除其它中风的原因1.5.5偏头痛激发的癫痫癫痫在偏头痛先兆发生的一小时内发生眼肌麻痹型偏头痛

放入13颅神经痛和与中枢疾病有关的面痛中。诊断标准为至少２次发作满足Ｂ偏头痛样头痛发作的同时或４日内发生第3、４和（或）６对脑神经中一条或多条轻瘫适当的检查排除眼窝和后颅窝组织损伤。

THETREATMENT

APPROACHTO

MIGRAINEMigraine,DepressionandAnxietyPatientswithmigraine3timesmorelikelytodevelopdepressionPatientswithdepression3timesmorelikelytodevelopmigraine(Breslau,1991;Breslau,Davis,1993,Patel,2007)MalepatientswithPanicDisorder7timesmorelikelytohavemigraine(Stewart,1989,Sheftell,2007)40%patientswithanxietyreportedapaindisorderand7%patientswithpanicdisorderreportedtakingpainmedicationsdaily(Kuch1991)50%ofMigraineursexperienceanxiety(Devlen1994)PathophysiologyofMigraineAsdescribedbyGoadsby,migraineinvolvesa"dysfunctionofbrainstempathwaysthatnormallymodulatesensoryinput".Moreprecisely,migraineinvolvesabnormalsensorymodulation.

Boyd,2005TrigeminalNerveEndingNeurogenicinflammation:Followingstimulationofthetrigeminalnerve,neuroinflammatorypeptides,suchassubstanceP,CGRP,andneurokininA,arereleasedfromperivascularnervefibers,triggeringneurogenicinflammation,whicheventuallyleadstothepainofmigraine.Silberstein,1998RatesofMigraineSymptomsYoungandSilberstein,2006AuraVisual

Scotoma:scintillating;flashes,mosaicvision Illusion:fortification,shimmering,rotation, oscillation,metamorphopsia,macropsiaSensory

Paresthesias:oftenmigrating,lastingfor minutes,canbecomebilateralLanguage DysarthriaordysphasiaMotor

WeaknessDisturbedsensorium

Déjàvu,jamais-vuScintillatingScotomaClinicalPhasesofaMigraineAttackGraphcourtesyoftheMigraineAssociationofIrelandAcuteTreatmentofMigraineGoalsofAcuteTreatmentRapidtreatmentMinimizerecurrenceRestoreabilitytofunctionMinimizetheuseofbackupandrescuemedicationsOptimizeself-careReduceuseofresourcesCosteffectivenessMinimalornoadverseeventsTriptansSelectiveagonistof5-HT1Dand5-HT1BreceptorsBlocksplasmaextravasationfromcranialvesselsBlockseffectsofCalcitoninGene-RelatedPeptide(CGRP)NonspecificTreatmentsAcuteTreatmentinClinicorEmergencyDepartmentPreventiveTreatmentDecreaseOfficevisits51%DecreaseERvisits82%DecreaseCTandMRIscanwithmigrainediagnosis(75%and88%)AMSIIshowedonly5%ofmigraineursusepreventivetherapy25%ofMigraineurshave>3attacks/monthPhysiciansshouldoffertherapywhen>2attacks/month-AdrenergicBlockersMostwidelyusedpreventivemedicationclass50%effectiveinproducing>50%reductioninattackfrequencyAnalysisof74controlledtrialsconfirmeffectivenessofpropranolol(atenolol,metoprolol,timolol,nadololalsoeffective)Blockcentral-receptorsthatinterferewithvigilance-enhancingadrenergicpathwayInhibitnitricoxideproduction(propranolol)NotEffective--blockerswithintrinsicsympathomimeticactivity(acebutolol,alprenolol,oxprenolol,pindolol)CalciumChannelAntagonistsMechanismofActionBlock5-HTreleaseBlockcalciumdependentenzymesinvolvedinprostaglandinformationInterferewithpropagationofspreadingdepression45controlledtrials-highdropoutratesduetoSideEffectsVerapamilmostusefulNicardipineandnifedipinenotrecommendedAnticonvulsantsValproicAcidIncreasesbrainlevelsof-aminobutyricacid5studieswithstrongevidenceforefficacyweightgain,GIsymptoms,thrombocytopenia,hepatitis/pancreatitisTopiramateNointerferencewithbirthcontrolpillsat<200mg/dWeightloss(3.8%ofbaselinebodyweight)paresthesias(50%)treatedwithpotassiumGabapentinmixedresultsforeffectiveness,drowsinesssideeffectLamotrigineandPhenytoin:ineffectiveDrugDoseRouteAspirin500-650mgOralParacetamol500mg-4gOralMIGRAINE:ABORTIVETHERAPYNon-specifictreatmentIbuprofen200-300mgOralDiclofenac50-100mgOral/IMNaproxen500-750mgOralABORTIVETHERAPYFORMIGRAINEDrugDoseRouteErgotalkaloidsErgotamine1-2mg/d;max-6g/dOralDihydroergotamine0.75-1mgSC5-HTreceptor

agonistsSumatriptan25-300mg6mgOrallySCRizatriptan10mgOrallySpecifictreatmentDrugDose(mg)/dRouteDomperidone10-80mgOralMetoclopramide5-10mgOral/IVPromethazine50-125mgOral/IMChlorpromazine10-25mgOral/IVANTI-NAUSEANTDRUGSFORMIGRAINETREATMENTWHYTHENEEDFORPROPHYLAXIS?Abortivedrugsshouldnotbeusedmorethan2-3timesaweekLong-termprophylaxisimprovesqualityoflifebyreducingfrequencyandseverityofattacks80%ofmigraineursmayrequireprophylaxisWHENISPROPHYLAXISINDICATED?AccordingtotheUSHeadacheConsortiumGuidelines,indicationsforpreventivetreatmentinclude:Patientswhohaveveryfrequentheadaches(morethan2perweek)Attackdurationis>48hoursHeadacheseverityisextremeMigraineattacksareaccompaniedbyprolongedauraUnacceptableadverseeffectsoccurwithacutemigrainetreatmentContraindicationtoacutetreatmentMigrainesubstantiallyinterfereswiththepatient’sdailyroutine,despiteacutetreatmentSpecialcircumstancessuchashemiplegicmigraineorattackswithariskofpermanentneurologicinjuryPatientpreferenceDrugsDose(mg/d)BetablockersPropranolol40-320CalciumChannelBlockersFlunarizineVerapamil10-20120-480TCAsAmitriptyline10-20SSRIsFluoxetine20-60PREVENTIVETHERAPYFORMIGRAINEDrugsDose(mg/d)Anti-convulsantSodiumvalproate600-1200Anti-histaminicCyproheptadine4-8PREVENTIVETHERAPYFORMIGRAINE

(CONTD.)ROLEOFBETABLOCKERSINMIGRAINEPROPHYLAXIS‘Goldstandard’inmigraineprophylaxisEstablishedefficacyandsafetyinmigraineprophylaxisEspeciallypreferredifhypertensionoranxietyco-existROLEOFPROPRANOLOLINMIGRAINEPROPHYLAXISLIMITATIONSOFIMMEDIATE-RELEASEPROPRANOLOLShortt½of3-5hrsMultipledailydosingrequiredtomaintainadequatedegreeofbeta-receptorblockadethroughout24hrPoorpatientcompliancemaycompromiseefficacyADVANTAGESOFEXTENDED-RELEASEPREPARATIONOFPROPRANOLOLMigrainepatientsareasymptomaticbetweenattacksImportanttominimizenumberofdailydosesduringprophylactictreatmentOnce-dailyadministrationimprovescomplianceStabledrugconcentrationfor24hrsDOSAGEOFPROPRANOLOLStartingdose:40-80mgoncedailyMax.dose/day:240mgIfsatisfactoryresponseisnotobtainedwithin4-6weeks,afterreachingthemaximaldose,therapyshouldbediscontinuedTaperslowlytoavoidreboundheadacheandadrenergicsideeffectsMax.duration:9to12months2.Tension-typeheadacheDiagnosticcriteriaAtleast10episodesfulfillingfollowingcriteriaHeadachelasting30minsto7daysHas2atleast2ofthefollowingBilaterallocationPressing/tightening(non-pulsating)qualityMildormoderateintensityNotaggravatedbyphysicalactivitysuchaswalkingorclimbingstairsNonauseaorvomiting<2episodesofphotophobiaorphonophobiaNotattributabletoanotherdisorderCategoriesInfrequentepisodictensiontypeheadacheOccurs<1daypermonth(<12days/year)FrequentepisodictensiontypeheadacheOccurs>1and<15days/month(>12and<180days/year)ChronictensiontypeheadacheOccurs>15days/month(180ormoredays/year)CausesUncertain?ActivationofhyperexcitableperipheralafferentneuronsfromheadandneckmusclesAssociatedwithandaggravatedbymuscletendernessandpsychologicaltensionbutdonotcauseitAbnormalitiesincentralpainprocessingandgeneralisedincreasedpainsensitivityarefoundinsomeindividualsGeneticfactorsPeopleatriskPrevalencepeaksatage40-49inbothsexesMeanlifetimeprevalenceis46%Chronictensiontypeheadacheaffects3%ofgeneralpopulationFemaletomaleratiois4:5PrevalenceincreaseswitheducationallevelCanoccurinchildrenPresentationMildtomoderatebilateralpainSensationofmuscletightnessorpressureLastshourstodaysNotassociatedwithconstitutionalorneurologicalsymptomsPeoplewithchronictensionheadachemorelikelytoseekhelpoftenhaveahistoryofepisodicheadachebutdelayeduntilfrequencyanddisabilityarehighDifferentialdiagnosisMigraine–inchronicformcharacteristicfeaturesdisappearandpainislesssevereNeckproblems–muscletendernessoftensiontypeheadachemayinvolvetheneckMedicationoveruseheadache–considerinpatientstakingopioidorcombinationanalgesicsforanaverageof10days/monthExaminationandinvestigationExaminationNeurologicalexaminationManualpalpationofpericranialmuscles(frontal,temporal,masseter,pterygoid,sternomastoid,spleniusandtrapezius.FundoscopyforpapilloedemaInvestigationsIfneuroexaminationnormalnoneneededInvestigationNeuroimagingshouldbearrangedifAtypicalpatternofheadacheHistoryofseizuresNeurologicalsignsorsymptomsSymptomaticillness–acquiredimmunodeficiencysyndrome,tumoursorneurofibromatosisTreatmentInfrequentheadacheGoodresultsfromnonprescriptionmedicationMayneedreassuranceIfrequiredrugsonmorethan2-3days/weekthenmedicaltreatmentisindicatedtopreventmedicationmisuseheadacheTreatmentAcutetherapyforindividualattacksSimpleanalgesiaAspirin500–1000mgNSAIDSParacetamolmoreeffectivethanplacebolesseffectivethanNSAIDSCombinationdrugscontainingsimpleanalgesicsandcaffeinearehelpfulOpioidsorsedativesshouldnotbeusedasimpairalertnessandcancauseoveruseanddependenceTreatmentPreventivetreatmentConsiderwhenheadachesarefrequentoracuteattacksdon’trespondtoabortivetreatmentBestevidenceisforAmitriptyline75-150mg/day.Ithelpsbothpainandmuscletenderness.WorksbestwhenstartedatlowdoseandincreasedweeklyMirtazipine15-30mg/dayUnhelpfulSSRI’sBotuliniumtoxinTreatmentPreventivetreatmentShouldbeconsideredwhenatleast2headaches/monthasriskofchronicheadachegoesupexponentiallywhenfrequencyreaches1/weekasdoesseverityofpainBenefitorpreventivetreatmentisdiminishedwhenpatientsaresimultaneouslyoverusingabortivetreatments.WithdrawalofmedicationisadvisedbeforestartingpreventativetherapyTreatmentEducation,lifestyleandnon-pharmacologicaltreatmentLittleevidenceexiststosupportorrefutemostdietaryorlifestylerecommendationsfortensiontypeheadache.TreatmentReferralDiagnosisisunclearDoesnotrespondtotreatmentComplicatedbymedicationoveruseRequireneuroimagingPrognosis45%ofadultswithfrequentorchronictensiontypeheadachewillgointoremission39%willcarryonwithfrequentheadaches16%willcarryonwithchronicheadachePoorprognosisAssociatedwithPresenceofchronicheadacheatbaselineCo-existingmigraineNotbeingmarriedSleepproblemsGoodprognosisAssociatedwithOld