背景n偏头痛的患病率在欧美国家为15002000/10万人，发病率为1015；在中国，患病率为732.1/10万人，发病率为0.06n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。n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n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。n1988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。nICHD-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n原发性头痛原发性头痛1.偏头痛2.紧张性头痛3.丛集性头痛及其它三叉自主神经性头痛4.其它原发性头痛n继发性头痛继发性头痛5.归因于头和（或）颈部外伤的头痛6.归因于颅或颈部血管疾病的头痛7.归因于非血管性的颅疾病的头痛8.归因于某些物质或它的戒断的头痛9.归因于感染的头痛10.归因于代谢疾病的头痛11.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12.归因于精神疾患的头痛n颅神经痛，中枢性或原发性面部痛及其它头痛颅神经痛，中枢性或原发性面部痛及其它头痛13.颅神经痛和中枢性疾病有关的面部痛14.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 Part 1: The primary headachesMigraine PrevalenceLipton,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A.至少有满足标准B-D的5次发作B.每次持续472小时（未治疗或治疗无效）C.头痛至少有下列特征中的两项1.单侧痛2.搏动性痛3.中或重度疼痛4.因日常体力活动加重或避免此类活动（如走路或爬楼梯）D.头痛过程中至少伴随下列一项1.恶心和/或呕吐2.畏光和畏声E.不能归因于其它疾患1.1 无先兆的偏头痛n对小儿，持续172小时n婴幼儿的畏光畏声可从其行为判断n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n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。n先兆通常经520分钟发展起来，持续2060分钟。n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A.至少2次头痛发作符合B-EB.能完全逆转的视觉、感觉、或言语症状，但无运动障碍C.至少满足下列两项1.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）2.至少一个症状渐渐发展5分钟和/或不同症状接连发生。3.每个症状持续560分钟 D.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E.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 A.至少次发作符合标准和。B.周期性发作，个别患儿呈刻板性，强烈恶心和呕吐持续小时至天。C.发作期间呕吐至少次小时，或至少小时。D.次发作间期症状完全缓解。E.不能归因于其它疾病。 1.3.2 腹型偏头痛 A.至少次发作符合标准B.腹部疼痛发作持续小时（未治疗或治疗不成功）。C.腹部疼痛具备以下所有特点 位于中线、脐周或难以定位 性质为钝痛或“微痛” 程度为中度或重度D.腹痛期间至少有以下项 食欲减退 恶心 呕吐 苍白。E.不能归因于另一种疾病。1.3.3 儿童良性发作性眩晕 n“无先兆多次严重眩晕发作，数分钟到数小时后自行缓解”至少应在次以上。n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n偏头痛15天/月，持续3个月以上，无药物滥用。n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n尽管经过治疗，头痛仍持续72小时以上。n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n先兆持续2周以上n无梗塞的影像学证据1.5.4 偏头痛性梗塞n 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。n与其它原因引起的中风的鉴别诊断：1.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2.中风发生在典型的偏头痛发作过程中3.必须排除其它中风的原因1.5.5 偏头痛激发的癫痫n癫痫在偏头痛先兆发生的一小时内发生o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A.至少次发作满足B.偏头痛样头痛发作的同时或日内发生第3、和（或）对脑神经中一条或多条轻瘫C.适当的检查排除眼窝和后颅窝组织损伤。 THE TREATMENTAPPROACH TO MIGRAINEMigraine, Depression and AnxietynPatients with migraine 3 times more likely to develop depressionnPatients with depression 3 times more likely to develop migraine (Breslau, 1991; Breslau,Davis, 1993, Patel, 2007)nMale patients with Panic Disorder 7 times more likely to have migraine (Stewart, 1989, Sheftell, 2007) n40% patients with anxiety reported a pain disorder and 7% patients with panic disorder reported taking pain medications daily (Kuch 1991)n50% of Migraineurs experience anxiety (Devlen 1994)Pathophysiology of MigraineAsdescribedbyGoadsby,migraineinvolvesadysfunctionofbrainstempathwaysthatnormallymodulatesensoryinput.Moreprecisely,migraineinvolvesabnormal sensory modulation.Boyd,2005Trigeminal Nerve EndingNeurogenic inflammation:Followingstimulationofthetrigeminalnerve,neuroinflammatorypeptides,suchassubstanceP,CGRP,andneurokininA,arereleasedfromperivascularnervefibers,triggeringneurogenicinflammation,whicheventuallyleadstothepainofmigraine.Silberstein,1998Rates of Migraine SymptomsYoungandSilberstein,2006AuraVisual Scotoma: scintillating; flashes, mosaic visionIllusion: fortification, shimmering, rotation, oscillation, metamorphopsia, macropsiaSensoryParesthesias: often migrating, lasting for minutes, can become bilateralLanguageDysarthria or dysphasiaMotorWeaknessDisturbed sensoriumDj vu, jamais-vuScintillating ScotomaClinical Phases of a Migraine AttackGraphcourtesyoftheMigraineAssociationofIrelandAcute Treatment of MigrainenGoals of Acute TreatmentnRapid treatmentnMinimize recurrencenRestore ability to functionnMinimize the use of backup and rescue medicationsnOptimize self-carenReduce use of resourcesnCost effectivenessnMinimal or no adverse eventsTriptansnSelective agonist of 5-HT1D and 5-HT1B receptors nBlocks plasma extravasation from cranial vesselsnBlocks effects of Calcitonin Gene-Related Peptide (CGRP)Nonspecific TreatmentsAcute Treatment in Clinic or Emergency DepartmentPreventive TreatmentnDecrease Office visits 51%nDecrease ER visits 82%nDecrease CT and MRI scan with migraine diagnosis (75% and 88%)nAMS II showed only 5% of migraineurs use preventive therapyn25 % of Migraineurs have 3 attacks/monthnPhysicians should offer therapy when 2 attacks/month -Adrenergic BlockersnMost widely used preventive medication class n50% effective in producing 50% reduction in attack frequencynAnalysis of 74 controlled trials confirm effectiveness of propranolol (atenolol, metoprolol, timolol, nadolol also effective)nBlock central -receptors that interfere with vigilance-enhancing adrenergic pathwaynInhibit nitric oxide production (propranolol)nNot Effective - -blockers with intrinsic sympathomimetic activity (acebutolol, alprenolol, oxprenolol, pindolol)Calcium Channel AntagonistsnMechanism of ActionnBlock 5-HT releasenBlock calcium dependent enzymes involved in prostaglandin formationnInterfere with propagation of spreading depressionn45 controlled trials - high drop out rates due to Side Effects nVerapamil most usefulnNicardipine and nifedipine not recommendedAnticonvulsantsnValproic AcidnIncreases brain levels of -aminobutyric acidn5 studies with strong evidence for efficacynweight gain, GI symptoms, thrombocytopenia, hepatitis/pancreatitisnTopiramatenNo interference with birth control pills at 48 hoursnHeadache severity is extremenMigraine attacks are accompanied by prolonged auranUnacceptable adverse effects occur with acute migraine treatmentnContraindication to acute treatmentnMigraine substantially interferes with the patients daily routine, despite acute treatmentnSpecial circumstances such as hemiplegic migraine or attacks with a risk of permanent neurologic injurynPatient preferenceDrugsDose (mg/d)1.BetablockersnPropranolol40-3202.Calcium Channel BlockersnFlunarizinenVerapamil10-20120-4803.TCAsnAmitriptyline10-204.SSRIsnFluoxetine20-60 PREVENTIVE THERAPY FOR MIGRAINEDrugsDose (mg/d)5.Anti-convulsantnSodium valproate600-12006.Anti-histaminicnCyproheptadine4-8PREVENTIVE THERAPY FOR MIGRAINE (CONTD.)ROLE OF BETA BLOCKERS IN MIGRAINE PROPHYLAXISnGold standard in migraine prophylaxisnEstablished efficacy and safety in migraine prophylaxisnEspecially preferred if hypertension or anxiety co-existROLE OF PROPRANOLOL IN MIGRAINE PROPHYLAXISLIMITATIONS OF IMMEDIATE-RELEASE PROPRANOLOLnShort t of 3-5 hrsnMultiple daily dosing required to maintain adequate degree of beta-receptor blockade throughout 24 hrnPoor patient compliance may compromise efficacyADVANTAGES OF EXTENDED-RELEASE PREPARATION OF PROPRANOLOLnMigraine patients are asymptomatic between attacksnImportant to minimize number of daily doses during prophylactic treatmentnOnce-daily administration improves compliancenStable drug concentration for 24 hrsDOSAGE OF PROPRANOLOLnStarting dose: 40-80 mg once dailynMax. dose/day: 240 mgnIf satisfactory response is not obtained within 4-6 weeks, after reaching the maximal dose, therapy should be discontinuednTaper slowly to avoid rebound headache and adrenergic side effectsnMax. duration: 9 to 12 months2. Tension-type headacheDiagnostic criterianAt least 10 episodes fulfilling following criterianHeadache lasting 30 mins to 7 daysnHas 2 at least 2 of the followingnBilateral locationnPressing/tightening (non-pulsating) qualitynMild or moderate intensitynNot aggravated by physical activity such as walking or climbing stairsnNo nausea or vomitingn 2 episodes of photophobia or phonophobianNot attributable to another disorderCategoriesnInfrequent episodic tension type headachenOccurs 1 day per month ( 1 and 12 and 15 days/month ( 180 or more days/year)CausesnUncertainn? Activation of hyper excitable peripheral afferent neurons from head and neck musclesnAssociated with and aggravated by muscle tenderness and psychological tension but do not cause itnAbnormalities in central pain processing and generalised increased pain sensitivity are found in some individualsnGenetic factorsPeople at risknPrevalence peaks at age 40-49 in both sexesnMean life time prevalence is 46%nChronic tension type headache affects 3% of general populationnFemale to male ratio is 4:5nPrevalence increases with educational levelnCan occur in childrenPresentationnMild to moderate bilateral painnSensation of muscle tightness or pressurenLasts hours to daysnNot associated with constitutional or neurological symptomsnPeople with chronic tension headache more likely to seek help often have a history of episodic headache but delayed until frequency and disability are highDifferential diagnosisnMigraine in chronic form characteristic features disappear and pain is less severenNeck problems muscle tenderness of tension type headache may involve the necknMedication overuse headache consider in patients taking opioid or combination analgesics for an average of 10 days/monthExamination and investigationnExamination nNeurological examinationnManual palpation of pericranial muscles ( frontal, temporal, masseter, pterygoid, sternomastoid, splenius and trapezius.nFundoscopy for papilloedema nInvestigationsnIf neuro examination normal none neededInvestigationnNeuroimaging should be arranged ifnAtypical pattern of headachenHistory of seizuresnNeurological signs or symptomsnSymptomatic illness acquired immunodeficiency syndrome, tumours or neurofibromatosisTreatmentnInfrequent headachenGood results from non prescription medicationnMay need reassurancenIf require drugs on more than 2-3 days/week then medical treatment is indicated to prevent medication misuse headacheTreatmentnAcute therapy for individual attacksnSimple analgesianAspirin 500 1000mgnNSAIDSnParacetamol more effective than placebo less effective than NSAIDSnCombination drugs containing simple analgesics and caffeine are helpfulnOpioids or sedatives should not be used as impair alertness and can cause overuse and dependenceTreatmentnPreventive treatmentnConsider when headaches are frequent or acute attacks dont respond to abortive treatmentnBest evidence is for Amitriptyline 75- 150mg/day. It helps both pain and muscle tenderness. Works best when started at low dose and increased weeklynMirtazipine 15-30mg/day nUnhelpfulnSSRIsnBotulinium toxinTreatmentnPreventive treatmentnShould be considered when at least 2 headaches/month as risk of chronic headache goes up exponentially when frequency reaches 1/week as does severity of painnBenefit or preventive treatment is diminished when patients are simultaneously overusing abortive treatments. Withdrawal of medication is advised before starting preventative therapyTreatmentnEducation, lifestyle and non-pharmacological treatmentnLittle evidence exists to support or refute most dietary or lifestyle recommendations for tension type headache.TreatmentnReferralnDiagnosis is unclearnDoes not respond to treatmentnComplicated by medication overusenRequire neuroimaging Prognosisn45% of adults with frequent or chronic tension type headache will go into remissionn39% will carry on with frequent headachesn16% will carry on with chronic headachePoor prognosisnAssociated withnPresence of chronic headache at baselinenCo-existing migrainenNot being marriednSleep problemsGood prognosisnAssociated withnOlder agenAbsence of chronic tension type headache at baselinenImportant message intervene early before headaches become chronic3. Cluster headacheand other trigeminal autonomiccephalalgias3.1 Cluster headache3.1 Cluster headache3.2 Paroxysmal hemicraniaPart 2:The secondary headachesPrimary or secondary headache?Primary or secondary headache?Primary or secondary headache?Diagnostic criteriafor secondary headachesImportant general rules11. Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures11.2.1 Cervicogenic headache11.2.1 Cervicogenic headache11.2.1 Cervicogenic headacheNotes11.3 Headache attributed to disorder of eyes11.3.1 Headache attributed to acute glaucoma11.5 Headache attributed to rhinosinusitis11.5 Headache attributed to rhinosinusitisNotes11.7 Headache or facial pain attributed to temporomandibular joint disorder12. Headache attributed to psychiatric disorderNew section in classification12. Headache attributed to psychiatric disorderNotes12.1 Headache attributed to somatisation disorder12.2 Headache attributed to psychotic disorderPart 3:Cranial neuralgias, central and primary facial pain and other headaches3. Cranial neuralgias and central causes of facial pain13.1 Trigeminal neuralgia13.1.1 Classical trigeminal neuralgia13.1.2 Symptomatic trigeminal neuralgia13.18.4 Persistent idiopathicfacial pain Previously used term: Atypical facial painACUTE HEADACHE IN THEEMERGENCY DEPARTMENT