九版神经病学头痛

1、头痛疾病的国际分类 International Classification of Headache Disorders - second edition ICHD-II 背景 n偏头痛的患病率在欧美国家为1500 2000/10万人，发病率为1015；在中国， 患病率为732.1/10万人，发病率为0.06 n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

2、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 n偏头痛 n紧张性头痛 n丛集性头痛及其它三叉自主神经性头痛 n其它原发性头痛 n继发性头痛继发性头痛 n归因于头和（或）颈部外伤的头痛 n归因于颅或颈部血

3、管疾病的头痛 n归因于非血管性的颅疾病的头痛 n归因于某些物质或它的戒断的头痛 n归因于感染的头痛 n归因于代谢疾病的头痛 n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 n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 n颅神经痛和中枢性疾病有关的面部痛 1.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.

4、1.2一氧化氮前体的迟发性头痛 一、原发性头痛 Part 1: The primary headaches International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) Part 1: The primary headaches 1. Migraine 2. Tension-type headache 3. Cluster headache and other trigeminal autonomic cephalalgias 4. Other primary headaches Migraine Pr

5、evalence Lipton, 2007 One-year period prevalence of migraine by age and gender American Migraine Prevalence and Prevention Study 1、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 n至少有满足标准B-D的5次发作 n每次持续472小时（未治疗或治疗无效） n头痛至少有下列特征中的两项 n单侧痛 n搏动性痛 n中或重度

6、疼痛 n因日常体力活动加重或避免此类活动（如走路或爬 楼梯） n头痛过程中至少伴随下列一项 n恶心和/或呕吐 n畏光和畏声 n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20 60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

7、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 n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） n至少一个症状

8、渐渐发展5分钟和/或不同症状接连发生。 n每个症状持续560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 n 至少次发作符合标准和。 n 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。 n 发作期间呕吐至少次小时，或至少 小时。 n 次发作间期症状完全缓解。 n 不能归因于其它疾病。 1.3.2 腹型偏头痛 n 至少次发作符合标准 n 腹部疼痛发作持续小时（未治疗或治疗 不成功）

9、。 n 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 n 腹痛期间至少有以下项 食欲减退 恶心 呕吐 苍白。 n 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

10、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： n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 n中

11、风发生在典型的偏头痛发作过程中 1.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 n 至少次发作满足 n 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 A. 适当的检查排除眼窝和后颅窝组织损伤。 THE TREATMENT APPROACH TO MIGRAINE Migraine, Depression and Anxiety nPatients with migraine 3 times more likely to develop dep

12、ression nPatients 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

13、 panic disorder reported taking pain medications daily (Kuch 1991) n50% of Migraineurs experience anxiety (Devlen 1994) Pathophysiology of Migraine As described by Goadsby, migraine involves a dysfunction of brain stem pathways that normally modulate sensory input. More precisely, migraine involves

14、abnormal sensory modulation. Boyd, 2005 Trigeminal Nerve Ending Neurogenic inflammation: Following stimulation of the trigeminal nerve, neuroinflammatory peptides, such as substance P, CGRP, and neurokinin A, are released from perivascular nerve fibers, triggering neurogenic inflammation, which even

15、tually leads to the pain of migraine. Silberstein, 1998 Rates of Migraine Symptoms Symptom Approximate % Throbbing* 80 Unilaterality* 61 Moderate to Severe* 97 Worse with Activity* 95 Nausea* 87 Vomiting* 56 Photophobia* 85 Phonophobia* 80 Allodynia 65 Nasal Congestion 70 Neck Pain 65 Stabbing Pain

16、40 Cluster-like eye symptoms 5 * International Headache Society Criteria Young and Silberstein, 2006 Aura Visual Scotoma: scintillating; flashes, mosaic vision Illusion: fortification, shimmering, rotation, oscillation, metamorphopsia, macropsia SensoryParesthesias: often migrating, lasting for minu

17、tes, can become bilateral LanguageDysarthria or dysphasia MotorWeakness Disturbed sensorium Dj vu, jamais-vu Scintillating Scotoma Clinical Phases of a Migraine Attack Graph courtesy of the Migraine Association of Ireland Acute Treatment of Migraine nGoals of Acute Treatment nRapid treatment nMinimi

18、ze recurrence nRestore ability to function nMinimize the use of backup and rescue medications nOptimize self-care nReduce use of resources nCost effectiveness nMinimal or no adverse events Triptans nSelective agonist of 5-HT1D and 5-HT1B receptors nBlocks plasma extravasation from cranial vessels nB

19、locks effects of Calcitonin Gene-Related Peptide (CGRP) Nonspecific Treatments Medication Comments ASA APAP ASA/APAP/caffeine (AAC) 1000 mg APAP during pregnancy Effective for moderate pain migraine (AAC more effective than either alone) NSAIDS Superior to APAP Effective for moderate pain migraine C

20、an lead to Medication Overuse Headache Ketorolac plus Sumatriptan for allodynia Baclofen GABA agonist Opioids Most studies poorly designed or negative Avoid for acute treatment Nasal Butorphanol as rescue agents Metoclopramide Antiemetic/ prokinetic Used in addition to pain relievers Acute Treatment

21、 in Clinic or Emergency Department Preventive Treatment nDecrease 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 therapy n25 % of Migraineurs have 3 attacks/month nPhysicians should offer

22、therapy when 2 attacks/month -Adrenergic Blockers nMost widely used preventive medication class n50% effective in producing 50% reduction in attack frequency nAnalysis of 74 controlled trials confirm effectiveness of propranolol (atenolol, metoprolol, timolol, nadolol also effective) nBlock central

23、-receptors that interfere with vigilance- enhancing adrenergic pathway nInhibit nitric oxide production (propranolol) nNot Effective - -blockers with intrinsic sympathomimetic activity (acebutolol, alprenolol, oxprenolol, pindolol) Calcium Channel Antagonists nMechanism of Action nBlock 5-HT release

24、 nBlock calcium dependent enzymes involved in prostaglandin formation nInterfere with propagation of spreading depression n45 controlled trials - high drop out rates due to Side Effects nVerapamil most useful nNicardipine and nifedipine not recommended Anticonvulsants nValproic Acid nIncreases brain

25、 levels of -aminobutyric acid n5 studies with strong evidence for efficacy nweight gain, GI symptoms, thrombocytopenia, hepatitis/pancreatitis nTopiramate nNo interference with birth control pills at 48 hours nHeadache severity is extreme nMigraine attacks are accompanied by prolonged aura nUnaccept

26、able adverse effects occur with acute migraine treatment nContraindication to acute treatment nMigraine substantially interferes with the patients daily routine, despite acute treatment nSpecial circumstances such as hemiplegic migraine or attacks with a risk of permanent neurologic injury nPatient

27、preference DrugsDose (mg/d) |Betablockers nPropranolol 40-320 |Calcium Channel Blockers nFlunarizine nVerapamil 10-20 120-480 |TCAs nAmitriptyline 10-20 |SSRIs nFluoxetine 20-60 PREVENTIVE THERAPY FOR MIGRAINE DrugsDose (mg/d) |Anti-convulsant nSodium valproate 600-1200 |Anti-histaminic nCyproheptad

28、ine 4-8 PREVENTIVE THERAPY FOR MIGRAINE (CONTD.) ROLE OF BETA BLOCKERS IN MIGRAINE PROPHYLAXIS nGold standard in migraine prophylaxis nEstablished efficacy and safety in migraine prophylaxis nEspecially preferred if hypertension or anxiety co-exist ROLE OF PROPRANOLOL IN MIGRAINE PROPHYLAXIS LIMITAT

29、IONS OF IMMEDIATE-RELEASE PROPRANOLOL nShort t of 3-5 hrs nMultiple daily dosing required to maintain adequate degree of beta- receptor blockade throughout 24 hr nPoor patient compliance may compromise efficacy ADVANTAGES OF EXTENDED-RELEASE PREPARATION OF PROPRANOLOL nMigraine patients are asymptom

30、atic between attacks nImportant to minimize number of daily doses during prophylactic treatment nOnce-daily administration improves compliance nStable drug concentration for 24 hrs DOSAGE OF PROPRANOLOL nStarting dose: 40-80 mg once daily nMax. dose/day: 240 mg nIf satisfactory response is not obtai

31、ned within 4-6 weeks, after reaching the maximal dose, therapy should be discontinued nTaper slowly to avoid rebound headache and adrenergic side effects nMax. duration: 9 to 12 months 2. Tension-type headache International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) 2. Tension-ty

32、pe headache 2.1 Infrequent episodic tension-type headache 2.2 Frequent episodic tension-type headache 2.3 Chronic tension-type headache 2.4 Probable tension-type headache Diagnostic criteria nAt least 10 episodes fulfilling following criteria nHeadache lasting 30 mins to 7 days nHas 2 at least 2 of

33、the following nBilateral location nPressing/tightening (non-pulsating) quality nMild or moderate intensity nNot aggravated by physical activity such as walking or climbing stairs nNo nausea or vomiting n 2 episodes of photophobia or phonophobia nNot attributable to another disorder Categories nInfre

34、quent episodic tension type headache nOccurs 1 day per month ( 1 and 12 and 15 days/month ( 180 or more days/year) Causes nUncertain n? Activation of hyper excitable peripheral afferent neurons from head and neck muscles nAssociated with and aggravated by muscle tenderness and psychological tension

35、but do not cause it nAbnormalities in central pain processing and generalised increased pain sensitivity are found in some individuals nGenetic factors People at risk nPrevalence peaks at age 40-49 in both sexes nMean life time prevalence is 46% nChronic tension type headache affects 3% of general p

36、opulation nFemale to male ratio is 4:5 nPrevalence increases with educational level nCan occur in children Presentation nMild to moderate bilateral pain nSensation of muscle tightness or pressure nLasts hours to days nNot associated with constitutional or neurological symptoms nPeople with chronic t

37、ension headache more likely to seek help often have a history of episodic headache but delayed until frequency and disability are high Differential diagnosis nMigraine in chronic form characteristic features disappear and pain is less severe nNeck problems muscle tenderness of tension type headache

38、may involve the neck nMedication overuse headache consider in patients taking opioid or combination analgesics for an average of 10 days/month Examination and investigation nExamination nNeurological examination nManual palpation of pericranial muscles ( frontal, temporal, masseter, pterygoid, stern

39、omastoid, splenius and trapezius. nFundoscopy for papilloedema nInvestigations nIf neuro examination normal none needed Investigation nNeuroimaging should be arranged if nAtypical pattern of headache nHistory of seizures nNeurological signs or symptoms nSymptomatic illness acquired immunodeficiency

40、syndrome, tumours or neurofibromatosis Treatment nInfrequent headache nGood results from non prescription medication nMay need reassurance nIf require drugs on more than 2-3 days/week then medical treatment is indicated to prevent medication misuse headache Treatment nAcute therapy for individual at

41、tacks nSimple analgesia nAspirin 500 1000mg nNSAIDS nParacetamol more effective than placebo less effective than NSAIDS nCombination drugs containing simple analgesics and caffeine are helpful nOpioids or sedatives should not be used as impair alertness and can cause overuse and dependence Treatment

42、 nPreventive treatment nConsider when headaches are frequent or acute attacks dont respond to abortive treatment nBest evidence is for Amitriptyline 75- 150mg/day. It helps both pain and muscle tenderness. Works best when started at low dose and increased weekly nMirtazipine 15-30mg/day nUnhelpful n

43、SSRIs nBotulinium toxin Treatment nPreventive treatment nShould 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 pain nBenefit or preventive treatment is diminished when patients are simultaneously overu

44、sing abortive treatments. Withdrawal of medication is advised before starting preventative therapy Treatment nEducation, lifestyle and non- pharmacological treatment nLittle evidence exists to support or refute most dietary or lifestyle recommendations for tension type headache. Treatment nReferral

45、nDiagnosis is unclear nDoes not respond to treatment nComplicated by medication overuse nRequire neuroimaging Prognosis n45% of adults with frequent or chronic tension type headache will go into remission n39% will carry on with frequent headaches n16% will carry on with chronic headache Poor progno

46、sis nAssociated with nPresence of chronic headache at baseline nCo-existing migraine nNot being married nSleep problems Good prognosis nAssociated with nOlder age nAbsence of chronic tension type headache at baseline nImportant message intervene early before headaches become chronic 3. Cluster heada

47、che and other trigeminal autonomic cephalalgias International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) 3. Cluster headache and other trigeminal autonomic cephalalgias 3.1 Cluster headache 3.2 Paroxysmal hemicrania 3.3 Short-lasting unilateral neuralgiform headache attacks with

48、conjunctival injection and tearing (SUNCT) 3.4 Probable trigeminal autonomic cephalalgia 3.1 Cluster headache International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) 3.1 Cluster headache A.At least 5 attacks fulfilling criteria B-D B.Severe or very severe unilateral orbital, sup

49、raorbital and/or temporal pain lasting 15-180 min if untreated C. Headache is accompanied by 1 of the following: 1. ipsilateral conjunctival injection and/or lacrimation 2.ipsilateral nasal congestion and/or rhinorrhoea 3.ipsilateral eyelid oedema 4.ipsilateral forehead and facial sweating 5.ipsilat

50、eral miosis and/or ptosis 6.a sense of restlessness or agitation D. Attacks have a frequency from 1/2 d to 8/d E. Not attributed to another disorder 3.1 Cluster headache International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) 3.1 Cluster headache 3.1.1 Episodic cluster headache

51、A. Attacks fulfilling criteria A-E for 3.1 Cluster headache B. At least two cluster periods lasting 7-365 d and separated by pain-free remission periods of 1 mo 3.1.2 Chronic cluster headache A. Attacks fulfilling criteria A-E for 3.1 Cluster headache B. Attacks recur over 1 y without remission peri

52、ods or with remission periods lasting 5/d for half of the time, although periods with lower frequency may occur E.Attacks are prevented completely by therapeutic doses of indomethacin F. Not attributed to another disorder Part 2: The secondary headaches International Headache Society 2003/4ICHD-II.

53、Cephalalgia 2004; 24 (Suppl 1) Part 2: The secondary headaches 5. Headache attributed to head and/or neck trauma 6.Headache attributed to cranial or cervical vascular disorder 7. Headache attributed to non-vascular intracranial disorder 8. Headache attributed to a substance or its withdrawal 9.Heada

54、che attributed to infection 10. Headache attributed to disorder of homoeostasis 11. Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures 12. Headache attributed to psychiatric disorder Primary or secondary head

55、ache? International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) Primary or secondary headache? Primary: no other causative disorder Secondary (ie, caused by another disorder): new headache occurring in close temporal relation to another disorder that is a known cause of headache c

56、oded as attributed to that disorder Primary or secondary headache? International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) Primary or secondary headache? A pre-existing primary headache made worse in close temporal relation to another disorder: judgement required to code either

57、as the primary headache only or as both the primary headache and a secondary headache (attributed to the other disorder) Primary or secondary headache? International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) Primary or secondary headache? Diagnosis: Primary headache only Primary

58、 + secondary Temporal relation of other disorder to headache exacerbation LooseClose Degree of exacerbationSlightMarked Other evidence that other disorder can cause secondary headache WeakStrong Other disorder eliminatedHeadache unchanged Headache returns to previous pattern Diagnostic criteria for

59、secondary headaches International Headache Society 2003/4ICHD-II. Cephalalgia 2004; 24 (Suppl 1) Diagnostic criteria for secondary headaches A.Headache with one (or more) of the following listed characteristics and fulfilling criteria C and D B.Another disorder known to be able to cause headache has

60、 been demonstrated C. Headache occurs in close temporal relation to the other disorder and/or there is other evidence of a causal relationship D.Headache is greatly reduced or resolves within 3 mo (shorter for some disorders) after successful treatment or spontaneous remission of the causative disor