国际偏头痛分类最新版（头痛分类的国际标准）
国际偏头痛分类最新版（头痛分类的国际标准）1.2.1 Migraine with typical aura1.2 Migraine with aura有先兆偏头痛一、原发性头痛（the primary headaches）1.偏头痛（Migraine）1.1 Migraine without aura无先兆偏头痛
The international Classification of Headache disorders 3rd Editon
顾建文，解放军306医院，神经外科
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 aura
1.2.1.1 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 aura
1.5.2 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
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
1.6.1 Recurrent gastrointestinal disturbance
1.6.1.1Cyclical 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
2.1.2 Infrequent episodic tension-type headache not associated with pericranial tenderness
2.2Frequent episodic tension-type headache频发性≥1d/月，但<15d，至少发作10次以上（≥12d/年但<180d）
2.2.1 Frequent episodic tension-type headache associated with pericranial tenderness
2.2.2 Frequent episodic tension-type headache not associated with pericranial tenderness
2.3 Chronic tension-type headache慢性紧张型头痛每月发作≥15d，3个月以上
2.3.1 Chronic tension-type headache associated with pericranial tenderness
2.3.2 Chronic tension-type headache not associated with pericranial tenderness
丛集性头痛的诊断标准：A：至少符合B-D的5次以上的发作B：单侧、眼眶、上眼眶及/或颞部重度或极重度疼痛，如不治疗持续15min-180min；C：伴随至少以上一项症状同侧结膜充血/流泪同侧鼻腔充血/流鼻水同侧眼皮水肿同侧前额及脸部出汗同侧瞳孔缩小/眼睑下垂不安的症状或躁动D：发作频率隔日1至8次/日E：除外其他疾患
2.4 Probable tension-type headache很可能的紧张型头痛
2.4.1 Probable infrequent episodic tension-type headache
2.4.2 Probable frequent episodic tension-type headache
2.4.3 Probable chronic tension-type headache
3.三叉自主神经性头痛（Trigeminal autonomic cephalalgias）
3.1 Cluster headache丛集性头痛
3.1.1 Episodic cluster headache发作性丛集性头痛
3.1.2 Chronic cluster headache慢性丛集性头痛
（复发作大于1年无缓解期，或缓解期<1个月）
3.2 Paroxysmal hemicrania
3.2.1 Episodic paroxysmal hemicrania
3.2.2 Chronic paroxysmal hemicrania
3.3 Short-lasting unilateral neuralgiform headache attacks
3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)
3.3.1.1 Episodic SUNCT
3.3.1.2 Chronic SUNCT
3.3.2 Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)
3.3.2.1 Episodic SUNA
3.3.2.2 Chronic SUNA
3.4 Hemicrania continua
3.5 Probable trigeminal autonomic cephalalgia
3.5.1 Probable cluster headache
3.5.2 Probable paroxysmal hemicrania
3.5.3 Probable short-lasting unilateral neuralgiform headache attacks
3.5.4 Probable hemicrania continua
4.其它的原发性头痛 Other primary headache disorders ）
4.1 Primary cough headache原发性咳嗽头痛
4.1.1 Probable primary cough headache
4.2 Primary exercise headache原发性运动性头痛
4.2.1 Probable primary exercise headache
4.3 Primary headache associated with sexual activity原发性性活动伴随的头痛
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 stimulus
4.5.2 Headache attributed to ingestion or inhalation of a cold stimulus
4.5.3 Probable cold-stimulus headache
4.5.3.1 Headache probably attributed to external application of a cold stimulus
4.5.3.2 Headache probably attributed to ingestion or inhalation of a cold stimulus
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 becoming continuous and unremitting within 24 hoursC：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
4.6 External-pressure headache
4.6.1 External-compression headache
4.6.2 External-traction headache
4.6.3 Probable external-pressure headache
4.6.3.1 Probable external-compression headache
4.6.3.2 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 headache
4.9 Hypnic headache
4.9.1 Probable hypnic headache
4.10 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 head
5.1.1 Acute headache attributed to moderate or severe traumatic injury to the head
5.1.2 Acute headache attributed to mild traumatic injury to the head
5.2 Persistent headache attributed to traumatic injury to the head
5.2.1 Persistent headache attributed to moderate or severe traumatic injury to the head
5.2.2 Persistent headache attributed to mild traumatic injury to the head
5.3 Acute headache attributed to whiplash
5.4 Persistent headache attributed to whiplash
5.5 Acute headache attributed to craniotomy
5.6 Persistent headache attributed to craniotomy
6.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 attack
6.1.1 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 haemorrhage
6.2.1 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 arteritis
6.4.1 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％。特异性91．2％可能分类为颞动脉炎。 |
6.5 Headache attributed to cervical carotid or vertebral artery disorder颈动脉或锥动脉疼痛
6.5.1 Headache or facial or neck pain attributed to cervical carotid or vertebral artery dissection动脉夹层
6.5.2 Post-endarterectomy headache内膜剥脱术后头痛
6.5.3 Headache attributed to carotid or vertebral angioplasty颈动脉血管成型术头痛
6.6 Headache attributed to cerebral venous thrombosis (CVT) 脑静脉血栓形成
6.7 Headache attributed tootheracute intracranial arterial disorder
6.7.1 Headache attributed to an intracranial endovascular procedure
6.7.2 Angiography headache
6.7.3 Headache attributed to reversible cerebral vasoconstriction syndrome (RCVS)
6.7.3.1 Headache probably attributed to reversible cerebral vasoconstrictionsyndrome (RCVS)
6.7.4 Headache attributed to intracranial arterial dissection颅内动脉夹层
6.8 Headache attributed to genetic vasculopathy
6.8.1 Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)
6.8.2 Mitochondrial Encephalopathy Lactic Acidosis and Stroke-like episodes (MELAS)
6.8.3 Headache attributed to another genetic vasculopathy
6.9 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 causes
7.1.3 Headache attributed to intracranial hypertension secondary to hydrocephalus
7.2 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 neurosarcoidosis
7.3.2 Headache attributed to aseptic (non-infectious) meningitis
7.3.3 Headache attributed to other non-infectious inflammatory disease
7.3.4 Headache attributed to lymphocytic hypophysitis
7.3.5 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 neoplasm
7.4.1.1 Headache attributed to colloid cyst of the third ventricle
7.4.2 Headache attributed to carcinomatous meningitis癌性脑膜炎
7.4.3 Headache attributed to hypothalamic or pituitary hyper- or hyposecretion
7.5 Headache attributed to intrathecal injection鞘内注射
7.6 Headache attributed to epileptic seizure
7.6.1 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 disorder
8.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 headache
8.1.1.2 Delayed NO donor-induced headache
8.1.2 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
8.1.4.1 Immediate alcohol-induced headache
8.1.4.2 Delayed alcohol-induced headache
8.1.5 Headache induced by food and/or additive由于食物成分/添加剂诱发
8.1.5.1 Monosodium glutamate (MSG)-induced headache谷氨酸单钠盐（味精）
8.1.6 Cocaine-induced headache可卡因诱发
8.1.7 Histamine-induced headache组织胺诱发
8.1.7.1 Immediate histamine-induced headache
8.1.7.2 Delayed histamine-induced headache
8.1.8 Calcitonin gene-related peptide (CGRP)-induced headache降钙素基因相关多肽诱发
8.1.8.1 Immediate CGRP-induced headache
8.1.8.2 Delayed CGRP-induced headache
8.1.9 Headache attributed to exogenous acute pressor agent
8.1.10 Headache attributed to occasional use of non-headache medication
8.1.11 Headache attributed to long-term use of non-headache medication
8.1.12 Headache attributed to exogenous hormone
8.1.13 Headache attributed to use of or exposure to other substance
8.2 Medication-overuse headache (MOH)药物过度使用性头痛
| MOH诊断标准1． 符合3-4项的头痛表现≥15天2． 规律使用1种或多种用于头痛急性期的止痛药超过3个月3． 在药物过度使用期间，头痛进展或明显加重4． 停用所用过度使用药物之后的2个月内，头痛必需改善（缓解或重归于之前的头痛模式 MOH头痛表现多样，常有特征转换的独特模式 过度是指规律且频繁，即每周2天或以上 最常见的药物：对乙酰氨基酚—曲坦类—巴比妥类—阿片类，此外阿司匹林、麦角类、镇痛药及复方制剂均可引起MOH |
8.2.1 Ergotamine-overuse headache麦角胺类
8.2.2 Triptan-overuse headache曲普坦类
8.2.3 Simple analgesic-overuse headache单纯止痛药类
8.2.3.1 Paracetamol (acetaminophen)-overuse headache对乙酰氨基酚
8.2.3.2 Acetylsalicylic acid-overuse headache乙酰水杨酸/阿司匹林
8.2.3.3 Other non-steroidal anti-inflammatory drug (NSAID)-overuse headache
8.2.4 Opioid-overuse headache鸦片类
8.2.5 Combination-analgesic-overuse headache复合制剂过度应用
8.2.6 Medication-overuse headache attributed to multiple drug classes not individually over used联合用药所致
8.2.7 Medication-overuse headache attributed to unverified overuse of multiple drug classes
8.2.8 Medication-overuse headache attributed to other medication
8.3 Headache attributed to substance withdrawal物质戒断
8.3.1 Caffeine-withdrawal headache咖啡因
8.3.2 Opioid-withdrawal headache鸦片类
8.3.3 Oestrogen-withdrawal headache雌激素
8.3.4 Headache attributed to withdrawal from chronic use of other substance
9.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 meningoencephalitis
9.1.1.2 Chronic headache attributed to bacterial meningitis or meningoencephalitis
9.1.1.3 Persistent headache attributed to past bacterial meningitis or meningoencephalitis
9.1.2 Headache attributed to viral meningitis or encephalitis病毒性脑膜炎或脑炎
9.1.2.1 Headache attributed to viral meningitis
9.1.2.2 Headache attributed to viral encephalitis
9.1.3 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 infection
9.1.3.2 Chronic headache attributed to intracranial fungal or other parasitic infection
9.1.4 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 infection
9.2.1.2 Chronic headache attributed to systemic bacterial infection
9.2.2 Headache attributed to systemic viral infection系统病毒感染
9.2.2.1 Acute headache attributed to systemic viral infection
9.2.2.2 Chronic headache attributed to systemic viral infection
9.2.3 Headache attributed to other systemic infection其他系统感染
9.2.3.1 Acute headache attributed to other systemic infection
9.2.3.2 Chronic headache attributed to other systemic infection
10.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 headache
10.3 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 headache
11.2.2 Headache attributed to retropharyngeal tendonitis
11.2.3 Headache attributed to craniocervical dystonia
11.3 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 disorder
11.3.5 Headache attributed to trochleitis
11.4 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 rhinosinusitis
11.6 Headache attributed to disorder of the teeth or jaw
11.7 Headache attributed to temporomandibular disorder (TMD)
11.8 Head or facial pain attributed to inflammation of the stylohyoid ligament
11.9 Headache or facial pain attributed to other disorder of cranium neck eyes ears nose sinuses teeth mouth or other facial or cervical structure
12.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 neuralgia
13.1.1.1 Classical trigeminal neuralgia purely paroxysmal
13.1.1.2 Classical trigeminal neuralgia with concomitant persistent facial pain
13.1.2 Painful trigeminal neuropathy
13.1.2.1 Painful trigeminal neuropathy attributed to acute Herpes zoster
13.1.2.2 Post-herpetic trigeminal neuropathy
13.1.2.3 Painful post-traumatic trigeminal neuropathy
13.1.2.4 Painful trigeminal neuropathy attributed to multiple sclerosis (MS) plaque
13.1.2.5 Painful trigeminal neuropathy attributed to space-occupying lesion
13.1.2.6 Painful trigeminal neuropathy attributed to other disorder
13.2 Glossopharyngeal neuralgia舌咽神经痛
13.3 Nervus intermedius (facial nerve) neuralgia中间神经痛（面神经痛）
13.3.1 Classical nervus intermedius neuralgia
13.3.2 Nervus intermedius neuropathy attributed to Herpes zoster Hunt’s综合症？
THS诊断标准：A：侧头痛，符合C特征B：海绵窦、眶上裂或眼眶内肉芽肿，被MRI或活检证实； 单侧的动眼、滑车、展神经麻痹（单个或多个组合）C：头痛先于眼肌麻痹2周内出现，或与眼神经麻痹同时出现 头痛部位在单侧的眉、眼处D：排除其他
13.4 Occipital neuralgia枕神经痛
13.5 Optic neuritis视神经炎
13.6 Headache attributed to ischaemic ocular motor nerve palsy缺血性眼神经麻痹
13.7 Tolosa-Hunt syndrome
13.8 Paratrigeminal oculosympathetic (Raeder’s) syndrome
13.9 Recurrent painful ophthalmoplegic neuropathy复发性痛性眼肌麻痹
13.10 Burning mouth syndrome (BMS)
13.11 Persistent idiopathic facial pain (PIFP)
13.12 Central neuropathic pain中枢神经痛
13.12.1 Central neuropathic pain attributed to multiple sclerosis (MS)
13.12.2 Central post-stroke pain (CPSP)
14. 其他头痛（Other headache disorders）
