头痛的国际分类(第三版)中文

2023偏头痛的诊断和治疗偏头痛是位列第二的常见神经系统失能性疾病，目前国内仍存在诊断正确率低、治疗不规范等问题。

近年来，随着偏头痛机制的深入研究，其分类、诊断及治疗方法均有所更新。

本文依据《中国偏头痛诊治指南（2023版）》，整理了偏头痛相关的分类及诊断以及治疗方面内容，以供学习。

偏头痛的分类和诊断在第三版国际头痛疾病分类（ICHD-3）中，6种亚型偏头痛，包括无先兆偏头痛、有先兆偏头痛、慢性偏头痛、偏头痛并发症、很可能的偏头痛、可能与偏头痛相关的周期综合征以及其他类型，常见类型的详细诊断标准如下：1.无先兆偏头痛诊断标准：A.符合B~D标准的头痛至少发作5次；B.头痛发作持续4~72小时（未治疗或治疗效果不佳）；C.至少符合4项中的2项（单侧、搏动性、中重度疼痛、日常体力活动加重头痛或因头痛而避免日常活动如行走或上楼梯）；D.至少符合2项中的1项（恶心或呕吐、畏光和畏声）；E.不能用ICHD-3中的其他诊断更好地解释。

诊断标准：A.至少2次发作符合B和C;B.至少有1个可完全恢复的先兆症状（视觉、感觉、言语或语言、运动、脑干、视网膜）；C.至少符合6项中的3项（至少有1个先兆持续超过5分钟、2个或更多的症状连续发生、每个独立先兆症状持续5~60分钟、至少有一个先兆是单侧的、至少有一个先兆是阳性的、与先兆伴发或在先兆出现60分钟内出现头痛）；D.不能用ICHD-3中的其他诊断更好地解释。

先兆症状通常发生在头痛前，较少情况下也可以和头痛伴随出现或出现于头痛发作后。

部分患者可既出现有先兆偏头痛发作，也有无先兆偏头痛发作，此时两种头痛应同时诊断。

具体可分为以下4种亚型：诊断标准：A.符合B和C的头痛（偏头痛样头痛或紧张型样头痛）每月发作至少15天，至少持续3个月；B.符合无先兆偏头痛诊断B~D标准或有先兆偏头痛B和C标准的头痛至少发生5次；C.头痛符合下列①〜③任意1项，且每月发作大于8天，持续时间大于3个月：①无先兆偏头痛的C和D；②有先兆偏头痛的B和C;③患者所认为的偏头痛发作可通过服用曲普坦类或麦角类药物缓解；D.不能用ICHD-3中的其他诊断更好地解释。

The international Classification of Headache disorders, 3rd Editon4.6.3.2 Probable external-traction headache 4.7.1 Probable primary stabbing headache6.2 Headache attributed to non-traumatic intracranial haemorrhage6.2.1 Headache attributed to non-traumatic intracerebral haemorrhage非外伤性脑出血6.2.2 Headache attributed to non-traumatic subarachnoid haemorrhage (SAH)6.2.3 Headache attributed to non-traumatic acute subdural haemorrhage (ASDH)急性硬膜下血肿6.3 Headache attributed to unruptured vascular malformation未破裂的血管畸形6.3.1 Headache attributed to unruptured saccular aneurysm囊性动脉瘤6.3.2 Headache attributed to arteriovenous malformation (AVM) 动静脉畸形6.3.3 Headache attributed to dural arteriovenous fistula (DAVF) 硬膜动静脉瘘6.3.4 Headache attributed to cavernous angioma 海绵窦血管瘤6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome)脑三叉神经或软脑膜血管瘤病6.4 Headache attributed to arteritis6.4.1 Headache attributed to giant cell arteritis (GCA) 颞动脉炎6.4.2 Headache attributed to primary angiitis of the central nervous system (PACNS)6.4.3 Headache attributed to secondary angiitis of the central nervous system (SACNS)6.8.2 Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS)6.8.3 Headache attributed to another genetic vasculopathy6.9 Headache attributed to pituitary apoplexy垂体卒中7.缘于非血管性颅内疾病的头痛（Headache attributed to non-vascular intracranial disorder ）7.1 Headache attributed to increased cerebrospinal fluid pressure颅内压增高7.1.1 Headache attributed to idiopathic intracranial hypertension (IIH)7.1.2 Headache attributed to intracranial hypertension secondary to metabolic, toxic or hormonal causes7.1.3 Headache attributed to intracranial hypertension secondary to hydrocephalus7.2 Headache attributed to low cerebrospinal fluid pressure颅内压低下7.2.1 Post-dural puncture headache腰穿后7.2.2 CSF fistula headache脑脊液漏7.2.3 Headache attributed to spontaneous intracranial hypotension自发性低颅压7.3 Headache attributed to non-infectious inflammatory disease非感染性炎性疾病7.3.1 Headache attributed to neurosarcoidosis7.3.2 Headache attributed to aseptic (non-infectious) meningitis7.3.3 Headache attributed to other non-infectious inflammatory disease7.3.4 Headache attributed to lymphocytic hypophysitis7.3.5 Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL)7.4 Headache attributed to intracranial neoplasia颅内新生物7.4.1 Headache attributed to intracranial neoplasm7.4.1.1 Headache attributed to colloid cyst of the third ventricle7.4.2 Headache attributed to carcinomatous meningitis癌性脑膜炎7.4.3 Headache attributed to hypothalamic or pituitary hyper- or hyposecretion7.5 Headache attributed to intrathecal injection鞘内注射7.6 Headache attributed to epileptic seizure7.6.1 Hemicrania epileptica癫痫半颅痛7.6.2 Post-ictal headache癫痫发作后头痛7.7 Headache attributed to Chiari malformation type I (CM1)7.8 Headache attributed to other non-vascular intracranial disorder8.缘于物质或物质戒断的头痛（Headache attributed to a substance or its withdrawal）8.1 Headache attributed to use of or exposure to a substance or its withdrawal缘于某种物质的应用或戒断8.1.1 Nitric oxide (NO) donor-induced headache一氧化氮诱发8.1.1.1 Immediate NO donor-induced headache8.1.1.2 Delayed NO donor-induced headache8.1.2 Phosphodiesterase (PDE) inhibitor-induced headache磷酸二酯酶抑制剂8.1.3 Carbon monoxide (CO)-induced headache一氧化碳诱发8.1.4 Alcohol-induced headache酒精诱发8.3.4 Headache attributed to withdrawal from chronic use of other substance9.缘于感染的头痛（Headache attributed to infection）9.1 Headache attributed to intracranial infection颅内感染9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis细菌性脑膜炎/脑膜脑炎9.1.1.1 Acute headache attributed to bacterial meningitis or meningoencephalitis9.1.1.2 Chronic headache attributed to bacterial meningitis or meningoencephalitis9.1.1.3 Persistent headache attributed to past bacterial meningitis or meningoencephalitis9.1.2 Headache attributed to viral meningitis or encephalitis病毒性脑膜炎或脑炎9.1.2.1 Headache attributed to viral meningitis9.1.2.2 Headache attributed to viral encephalitis9.1.3 Headache attributed to intracranial fungal or other parasitic infection真菌或寄生虫感染9.1.3.1 Acute headache attributed to intracranial fungal or other parasitic infection9.1.3.2 Chronic headache attributed to intracranial fungal or other parasitic infection9.1.4 Headache attributed to brain abscess脑脓肿9.1.5 Headache attributed to subdural empyema硬膜下积脓9.2 Headache attributed to systemic infection系统感染9.2.1 Headache attributed to systemic bacterial infection系统细菌感染9.2.1.1 Acute headache attributed to systemic bacterial infection9.2.1.2 Chronic headache attributed to systemic bacterial infection9.2.2 Headache attributed to systemic viral infection系统病毒感染9.2.2.1 Acute headache attributed to systemic viral infection9.2.2.2 Chronic headache attributed to systemic viral infection9.2.3 Headache attributed to other systemic infection其他系统感染9.2.3.1 Acute headache attributed to other systemic infection9.2.3.2 Chronic headache attributed to other systemic infection10.缘于内环境紊乱的头痛（Headache attributed to disorder of homoeostasis）10.1 Headache attributed to hypoxia and/or hypercapnia缺氧和/或高碳酸血症10.1.1 High-altitude headache高海拔头痛10.1.2 Headache attributed to aeroplane travel飞行头痛10.1.3 Diving headache潜水头痛10.1.4 Sleep apnoea headache睡眠呼吸暂停性头痛10.2 Dialysis headache10.3 Headache attributed to arterial hypertension高血压性头痛10.3.1 Headache attributed to phaeochromocytoma嗜络细胞瘤10.3.2 Headache attributed to hypertensive crisis without hypertensive encephalopathy无高血压脑病的高血压危象10.3.3 Headache attributed to hypertensive encephalopathy高血压脑病10.3.4 Headache attributed to pre-eclampsia or eclampsia先兆子痫10.3.5 Headache attributed to autonomic dysreflexia自主反射障碍10.4 Headache attributed to hypothyroidism甲状腺低下10.5 Headache attributed to fasting禁食10.6 Cardiac cephalalgia心源性头痛10.7 Headache attributed to other disorder of homoeostasis其他内环境稳定失调疾患11.缘于头、颈、眼、耳、鼻、鼻窦、牙、口或其他头面部结构病变的头面痛（Headache or facial pain attributed to disorder of the cranium, neck,eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure ）11.1 Headache attributed to disorder of cranial bone颅骨11.2 Headache attributed to disorder of the neck 颈部11.2.1 Cervicogenic headache11.2.2 Headache attributed to retropharyngeal tendonitis11.2.3 Headache attributed to craniocervical dystonia11.3 Headache attributed to disorder of the eyes眼睛11.3.1 Headache attributed to acute glaucoma急性青光眼11.3.2 Headache attributed to refractive error屈光不正11.3.3 Headache attributed to heterophoria or heterotropia (latent or persistent squint)斜视11.3.4 Headache attributed to ocular inflammatory disorder11.3.5 Headache attributed to trochleitis11.4 Headache attributed to disorder of the ears耳朵11.5 Headache attributed to disorder of the nose or paranasal sinuses鼻子或鼻窦11.5.1 Headache attributed to acute rhinosinusitis急性鼻窦炎11.5.2 Headache attributed to chronic or recurring rhinosinusitis11.6 Headache attributed to disorder of the teeth or jaw11.7 Headache attributed to temporomandibular disorder (TMD)11.8 Head or facial pain attributed to inflammation of the stylohyoid ligament11.9 Headache or facial pain attributed to other disorder of cranium, neck, eyes, ears, nose,sinuses, teeth, mouth or other facial or cervical structure12.缘于精神疾病的头痛（Headache attributed to psychiatric disorder）12.1 Headache attributed to somatization disorder躯体化障碍12.2 Headache attributed to psychotic disorder精神疾病三、脑神经痛、中枢和原发性颜面痛及其他头痛（painful cranial neuropathies, other facial pains and other headaches）13.脑神经痛和中枢性颜面痛（Painful cranial neuropathies and other facial pains）13.1 Trigeminal neuralgia三叉神经痛13.1.1 Classical trigeminal neuralgia13.1.1.1 Classical trigeminal neuralgia, purely paroxysmal13.1.1.2 Classical trigeminal neuralgia with concomitant persistent facial pain。

The international Classification of Headache disorders, 3rd Editon 头痛分类的国际标准(ICHD-III)概述：头痛分为三大组：原发性头痛；继发性头痛；脑神经痛、中枢和原发性颜面痛及其他头痛。

每一种原发性头痛均视为一种独立的疾病；继发性头痛一般只是某种疾病的一种症状，该头痛称为“缘于”此种疾病的头痛。

三大组头痛共分为14类： 一、原发性头痛（the primary headaches ） 1.偏头痛（Migraine ） 1.1 Migraine without aura 无先兆偏头痛 1.2 Migraine with aura 有先兆偏头痛 1.2.1 Migraine with typical aura1.2.1.1 Typical aura with headache 1.2.1.2 Typical aura without headache 1.2.2 Migraine with brainstem aura 1.2.3 Hemiplegic migraine 偏瘫型偏头痛1.2.3.1 Familial hemiplegic migraine (FHM)—家族性 1.2.3.1.1 Familial hemiplegic migraine type 1 (FHM1)1.2.3.1.2 Familial hemiplegic migraine type 2 (FHM2)1.2.3.1.3 Familial hemiplegic migraine type 3 (FHM3) 1.2.3.1.4 Familial hemiplegic migraine, other loci 1.2.3.2 Sporadic hemiplegic migraine —散发性 1.2.4 Retinal migraine 1.3 Chronic migraine 慢性偏头痛1.4 Complications of migraine 偏头痛并发症1.4.1 Status migrainosus 偏头痛持续状态 1.4.2 Persistent aura without infarction 1.4.3 Migrainous infarction 偏头痛脑梗死 1.4.4 Migraine aura-triggered seizure 1.5 Probable migraine 很可能的偏头痛 1.5.1 Probable migraine without aura1.5.2 Probable migraine with aura 1.6 Episodic syndromes that may be associated with migraine 可能与偏头痛有关的发作性综合征1.6.1 Recurrent gastrointestinal disturbance1.6.1.1Cyclical vomiting syndrome 周期性呕吐 1.6.1.2 Abdominal migraine 腹型偏头痛1.6.2 Benign paroxysmal vertigo 良性发作性眩晕1.6.3 Benign paroxysmal torticollis 良性发作性斜颈2.紧张型头痛（Tension-type headache ）每次发作持续30min-7d ，慢性者可持续不缓解2.1 Infrequent episodic tension-type headache 偶发性 <1d/月，至少10次以上（<12d/年）2.1.1 Infrequent episodic tension-type headache associated with pericranial tenderness无先兆偏头痛的诊断标准：A ：符合B-D 特征，至少发作5次发作B ：头痛（未经治疗）发作持续4-72小时C ：至少有以下特征中的2项单侧性、搏动性、中重度痛、日常活动会加重D ：至少以下一项伴随症状：恶心和/或呕吐、畏光和畏声E ：不能归因于其他 先兆性偏头痛的诊断标准：A ：符合B-C 特征，至少发作2次发作B ：完全可逆以下症状的1项或多项✓ 视觉、感觉、语言障碍（典型先兆）✓ 肢体无力症状（偏瘫型偏头痛）✓ 脑干症状（基底型偏头痛） 视网膜症状（视网膜型偏头痛） C ：至少有以下特征中的2项 ✓ 至少1个先兆逐渐发展的过程>5min ，和/或 不同先兆接连发生过程>5min ✓ 每个先兆症状持续时间5-60min✓ 至少一个先兆症状为单侧性✓ 先兆发生同时或之后内60min 出现符合偏头痛 D ：不能归因于其他 肢体无力症状可以持续至72h ，部分患者肢体无力感可持续数周； 若患者先兆持续1周以上，称为无梗死的持续先兆一般先兆症状多为双侧，可持续数月或数年若先兆持续60min 至1周以内，则称为propablemigraine with aura若出现影像学梗死灶，则称为偏头痛性脑梗死 （年轻女性，后循环比较常见） 慢性偏头痛：偏头痛发作每月>15天，连续3个月以上偏头痛持续状态：单次发作持续72小时以上4.3.1 Probable primary headache associated with sexual activity 4.4 Primary thunderclap headache 原发性霹雳头痛 4.5 Cold-stimulus headache 冷刺激性头痛 4.5.1 Headache attributed to external application of a cold stimulus4.5.2 Headache attributed to ingestion or inhalation of a cold stimulus4.5.3 Probable cold-stimulus headache 4.5.3.1 Headache probably attributed to external application of a cold stimulus4.5.3.2 Headache probably attributed to ingestion or inhalation of a cold stimulus4.6 External-pressure headache 4.6.1 External-compression headache 4.6.2 External-traction headache 4.6.3 Probable external-pressure headache4.6.3.1 Probable external-compression headache4.6.3.2 Probable external-traction headache 4.7 Primary stabbing headache 4.7.1 Probable primary stabbing headache 4.8 Nummular headache 4.8.1 Probable nummular headache4.9 Hypnic headache4.9.1 Probable hypnic headache4.10 New daily persistent headache (NDPH) 新症每日持续性头痛4.10.1 Probable new daily persistent headach 二、继发性头痛（the secondary headaches ）5.缘于头、颈部外伤的头痛（Headache attributed to trauma or injury to the head and/or neck ）5.1 Acute headache attributed to traumatic injury to the head5.1.1 Acute headache attributed to moderate or severe traumatic injury to the head5.1.2 Acute headache attributed to mild traumatic injury to the head5.2 Persistent headache attributed to traumatic injury to the head5.2.1 Persistent headache attributed to moderate or severe traumatic injury to the head5.2.2 Persistent headache attributed to mild traumatic injury to the head5.3 Acute headache attributed to whiplash5.4 Persistent headache attributed to whiplash5.5 Acute headache attributed to craniotomy5.6 Persistent headache attributed to craniotomy6.缘于头颈部血管疾病的头痛（Headache attributed to cranial or cervical vascular disorder ）6.1 Headache attributed to ischaemic stroke or transient ischaemic attack6.1.1 Headache attributed to ischaemic stroke (cerebral infarction)6.1.2 Headache attributed to transient ischaemic attack (TIA)6.2 Headache attributed to non-traumatic intracranial haemorrhage6.2.1 Headache attributed to non-traumatic intracerebral haemorrhage 非外伤性脑出血6.2.2 Headache attributed to non-traumatic subarachnoid haemorrhage (SAH)NDPH 诊断标准：A ：持续性头痛，符合B-C 的标准B ：Distinct and clearly remembered onset, with pain becomingcontinuous and unremitting within 24 hours C ：Present for>3 months D ：除外其他疾患慢性头痛，起病急，可清晰回忆发病时间为哪一天 疼痛缺乏特点，可以为偏头痛，可以为紧张性，亦可两者混合若患者符合慢性偏头痛或慢性紧张型头痛的标准，则不诊断NDPH 若患者为药物过度使用性头痛，且符合NDPH 的标准， 则可两者均可作为诊断6.2.3 Headache attributed to non-traumatic acute subdural haemorrhage (ASDH)急性硬膜下血肿6.3 Headache attributed to unruptured vascular malformation未破裂的血管畸形6.3.1 Headache attributed to unruptured saccular aneurysm囊性动脉瘤6.3.2 Headache attributed to arteriovenous malformation (AVM) 动静脉畸形6.3.3 Headache attributed to dural arteriovenous fistula (DAVF) 硬膜动静脉瘘6.3.4 Headache attributed to cavernous angioma 海绵窦血管瘤6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome)脑三叉神经或软脑膜血管瘤病6.4 Headache attributed to arteritis6.4.1 Headache attributed to giant cell arteritis (GCA) 颞动脉炎6.4.2 Headache attributed to primary angiitis of the central nervous system (PACNS)6.4.3 Headache attributed to secondary angiitis of the central nervous system (SACNS)颞动脉炎诊断标准：美国风湿学会1990年1. 50岁以上发病：50岁以上发现症状2．新发的头痛：出现新发的局限性头痛3．颞动脉异常：颞动脉压痛或者与颈动脉的动脉硬化无关的搏动减弱4．血沉值升高：50mm/h以上5．动脉活检异常：单核细胞为主的细胞浸润或者伴多核巨细胞的肉芽肿出现为特征的血管炎证据5项中如满足3项以上，敏感度93．5％。

头痛分类的国际标准按照头痛分类的国际标准（ ICHD-III）的分类标准，临床上头痛一共有14种类型，每种类型又包含若干种头痛。

偏头痛▎概述临床最常见的原发性头痛，人群中患病率高达5%-10%，也就是说在中国有至少有6500万的患者。

头痛发作位置大多如图所示，疼痛为中重度搏动样头痛，可持续4-72小时，非常影响患者生活。

▎诱因偏头痛的诱因非常复杂，据美国流行病调查统计，大约70%-80%的患者有家族史，同时外界因素（声、光刺激等）和生活规律（食物、睡眠、压力等）也可能引发偏头痛。

▎治疗因此对偏头痛的治疗方案也较多，如果考虑针对病因的治疗，可以让患者记录下每次发作的时间、地点和状况，以便分析其诱因。

针对中轻度头痛，一般治疗可单用非特异性止痛药，如非甾体类消炎药（对乙酰氨基酚、萘普生、布洛芬等）。

对中重度头痛，可选用偏头痛特异性治疗药物如麦角类制剂和曲谱坦类药物。

由于这两类药物有强烈的血管收缩作用，并且长期大量使用可引起严重不良后果，因此，对于以往服用非甾体类消炎药（NSAIDs）效果较好的中重度头痛患者，可以继续给予NSAIDs类药物。

由于偏头痛诱因复杂，部分患者可建议进行心理疏导，对于慢性长期性偏头痛，美国有研究者认为认知行为治疗也是可行的替代方案。

丛集性头痛▎概述此病之所以得这么个名字，是因为它的疼痛成群结队而来，表现为一连串的密集的非搏动性剧痛。

丛集性期长达2周至3个月，每次发作持续15-180分钟。

位置如图，在一侧眼眶或额颞部。

发作时患者坐立不安，部分患者甚至会用拳击打头部以减轻疼痛。

此病发病率比偏头痛较少，近6.8/10万。

▎诱因丛集性头痛一般无家族史，可由饮酒、吸烟、缺氧、热度或高海拔等因素诱发。

其病因尚不明确，一般认为是颅内、外血管扩张导致。

Horton认为与组胺释放有关。

▎治疗对于安定类药物效果不佳的丛集性头痛可给予吸氧（100%氧气8-10L/min，10-15分钟）。

曲普坦类药物可以迅速缓解头痛。