頭痛分類的國際標準(ICHD-III)

The international Classification of Headache disorders, 3rd Editon

顧建文，解放軍306醫院，神經外科

概述：頭痛分為三大組：原發性頭痛；繼發性頭痛；腦神經痛、中樞和原發性顏面痛及其他頭痛。每一種原發性頭痛均視為一種獨立的疾病；繼發性頭痛一般只是某種疾病的一種癥狀，該頭痛稱為「緣於」此種疾病的頭痛。

三大組頭痛共分為14類：

一、原發性頭痛（the primary headaches）

1.偏頭痛（Migraine）

1.1 Migraine without aura無先兆偏頭痛

1.2 Migraine with aura有先兆偏頭痛

1.2.1 Migraine with typical aura

先兆性偏頭痛的診斷標準：A：符合B-C特徵，至少發作2次發作B：完全可逆以下癥狀的1項或多項ü 視覺、感覺、語言障礙（典型先兆）ü 肢體無力癥狀（偏癱型偏頭痛）ü 腦幹癥狀（基底型偏頭痛） 視網膜癥狀（視網膜型偏頭痛）C：至少有以下特徵中的2項ü 至少1個先兆逐漸發展的過程>5min，和/或不同先兆接連發生過程>5minü 每個先兆癥狀持續時間5-60minü 至少一個先兆癥狀為單側性ü 先兆發生同時或之後內60min出現符合偏頭痛D：不能歸因於其他 肢體無力癥狀可以持續至72h，部分患者肢體無力感可持續數周； 若患者先兆持續1周以上，稱為無梗死的持續先兆一般先兆癥狀多為雙側，可持續數月或數年 若先兆持續60min至1周以內，則稱為propable migraine with aura 若出現影像學梗死灶，則稱為偏頭痛性腦梗死 （年輕女性，後循環比較常見）

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偏癱型偏頭痛

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—散發性

1.2.4 Retinal migraine

1.3 Chronic migraine慢性偏頭痛

1.4 Complications of migraine偏頭痛併發症

1.4.1 Status migrainosus偏頭痛持續狀態

1.4.2 Persistent aura without infarction

1.4.3 Migrainous infarction偏頭痛腦梗死

1.4.4 Migraine aura-triggered seizure

1.5 Probable migraine很可能的偏頭痛

1.5.1 Probable migraine without aura

1.5.2 Probable migraine with aura

1.6 Episodic syndromes that may be associated with migraine可能與偏頭痛有關的發作性綜合征

慢性偏頭痛：偏頭痛發作每月>15天，連續3個月以上偏頭痛持續狀態：單次發作持續72小時以上

1.6.1 Recurrent gastrointestinal disturbance

1.6.1.1Cyclical vomiting syndrome周期性嘔吐

1.6.1.2 Abdominal migraine腹型偏頭痛

1.6.2 Benign paroxysmal vertigo良性發作性眩暈

1.6.3 Benign paroxysmal torticollis良性發作性斜頸

2.緊張型頭痛（Tension-type headache）每次發作持續30min-7d，慢性者可持續不緩解

2.1 Infrequent episodic tension-type headache偶發性 <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頻發性≥1d/月，但<15d，至少發作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慢性緊張型頭痛每月發作≥15d，3個月以上

2.3.1 Chronic tension-type headache associated with pericranial tenderness

2.3.2 Chronic tension-type headache not associated with pericranial tenderness

叢集性頭痛的診斷標準：A：至少符合B-D的5次以上的發作B：單側、眼眶、上眼眶及/或顳部重度或極重度疼痛，如不治療持續15min-180min；C：伴隨至少以上一項癥狀同側結膜充血/流淚同側鼻腔充血/流鼻水同側眼皮水腫同側前額及臉部出汗同側瞳孔縮小/眼瞼下垂不安的癥狀或躁動D：發作頻率隔日1至8次/日E：除外其他疾患

2.4 Probable tension-type headache很可能的緊張型頭痛

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

3.三叉自主神經性頭痛（Trigeminal autonomic cephalalgias）

3.1 Cluster headache叢集性頭痛

3.1.1 Episodic cluster headache發作性叢集性頭痛

3.1.2 Chronic cluster headache慢性叢集性頭痛

（複發作大於1年無緩解期，或緩解期<1個月）

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.其它的原發性頭痛 Other primary headache disorders ）

4.1 Primary cough headache原發性咳嗽頭痛

4.1.1 Probable primary cough headache

4.2 Primary exercise headache原發性運動性頭痛

4.2.1 Probable primary exercise headache

4.3 Primary headache associated with sexual activity原發性性活動伴隨的頭痛

4.3.1 Probable primary headache associated with sexual activity

4.4 Primary thunderclap headache原發性霹靂頭痛

4.5 Cold-stimulus headache冷刺激性頭痛

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診斷標準：A：持續性頭痛，符合B-C的標準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 慢性頭痛，起病急，可清晰回憶發病時間為哪一天 疼痛缺乏特點，可以為偏頭痛，可以為緊張性，亦可兩者混合 若患者符合慢性偏頭痛或慢性緊張型頭痛的標準，則不診斷NDPH 若患者為藥物過度使用性頭痛，且符合NDPH的標準，則可兩者均可作為診斷

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)新症每日持續性頭痛

4.10.1 Probable new daily persistent headach

二、繼發性頭痛（the secondary headaches）

5.緣於頭、頸部外傷的頭痛（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.緣於頭頸部血管疾病的頭痛（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非外傷性腦出血

6.2.2 Headache attributed to non-traumatic subarachnoid haemorrhage (SAH)

6.2.3 Headache attributed to non-traumatic acute subdural haemorrhage (ASDH)急性硬膜下血腫

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囊性動脈瘤

6.3.2 Headache attributed to arteriovenous malformation (AVM) 動靜脈畸形

6.3.3 Headache attributed to dural arteriovenous fistula (DAVF) 硬膜動靜脈瘺

6.3.4 Headache attributed to cavernous angioma 海綿竇血管瘤

6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome)

腦三叉神經或軟腦膜血管瘤病

6.4 Headache attributed to arteritis

6.4.1 Headache attributed to giant cell arteritis (GCA)顳動脈炎

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)

6.5 Headache attributed to cervical carotid or vertebral artery disorder頸動脈或錐動脈疼痛

6.5.1 Headache or facial or neck pain attributed to cervical carotid or vertebral artery dissection動脈夾層

6.5.2 Post-endarterectomy headache內膜剝脫術後頭痛

6.5.3 Headache attributed to carotid or vertebral angioplasty頸動脈血管成型術頭痛

6.6 Headache attributed to cerebral venous thrombosis (CVT) 腦靜脈血栓形成

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顱內動脈夾層

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垂體卒中

7.緣於非血管性顱內疾病的頭痛（Headache attributed to non-vascular intracranial disorder ）

7.1 Headache attributed to increased cerebrospinal fluid pressure顱內壓增高

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顱內壓低下

7.2.1 Post-dural puncture headache腰穿後

7.2.2 CSF fistula headache腦脊液漏

7.2.3 Headache attributed to spontaneous intracranial hypotension自發性低顱壓

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顱內新生物

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癌性腦膜炎

7.4.3 Headache attributed to hypothalamic or pituitary hyper- or hyposecretion

7.5 Headache attributed to intrathecal injection鞘內注射

7.6 Headache attributed to epileptic seizure

7.6.1 Hemicrania epileptica癲癇半顱痛

7.6.2 Post-ictal headache癲癇發作後頭痛

7.7 Headache attributed to Chiari malformation type I (CM1)

7.8 Headache attributed to other non-vascular intracranial disorder

8.緣於物質或物質戒斷的頭痛（Headache attributed to a substance or its withdrawal）

8.1 Headache attributed to use of or exposure to a substance or its withdrawal緣於某種物質的應用或戒斷

8.1.1 Nitric oxide (NO) donor-induced headache一氧化氮誘發

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磷酸二酯酶抑制劑

8.1.3 Carbon monoxide (CO)-induced headache一氧化碳誘發

8.1.4 Alcohol-induced headache酒精誘發

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由於食物成分/添加劑誘發

8.1.5.1 Monosodium glutamate (MSG)-induced headache谷氨酸單鈉鹽（味精）

8.1.6 Cocaine-induced headache可卡因誘發

8.1.7 Histamine-induced headache組織胺誘發

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降鈣素基因相關多肽誘發

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)藥物過度使用性頭痛

8.2.1 Ergotamine-overuse headache麥角胺類

8.2.2 Triptan-overuse headache曲普坦類

8.2.3 Simple analgesic-overuse headache單純止痛藥類

8.2.3.1 Paracetamol (acetaminophen)-overuse headache對乙醯氨基酚

8.2.3.2 Acetylsalicylic acid-overuse headache乙醯水楊酸/阿司匹林

8.2.3.3 Other non-steroidal anti-inflammatory drug (NSAID)-overuse headache

8.2.4 Opioid-overuse headache鴉片類

8.2.5 Combination-analgesic-overuse headache複合製劑過度應用

8.2.6 Medication-overuse headache attributed to multiple drug classes not individually over used聯合用藥所致

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物質戒斷

8.3.1 Caffeine-withdrawal headache咖啡因

8.3.2 Opioid-withdrawal headache鴉片類

8.3.3 Oestrogen-withdrawal headache雌激素

8.3.4 Headache attributed to withdrawal from chronic use of other substance

9.緣於感染的頭痛（Headache attributed to infection）

9.1 Headache attributed to intracranial infection顱內感染

9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis細菌性腦膜炎/腦膜腦炎

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病毒性腦膜炎或腦炎

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真菌或寄生蟲感染

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腦膿腫

9.1.5 Headache attributed to subdural empyema硬膜下積膿

9.2 Headache attributed to systemic infection系統感染

9.2.1 Headache attributed to systemic bacterial infection系統細菌感染

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系統病毒感染

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其他系統感染

9.2.3.1 Acute headache attributed to other systemic infection

9.2.3.2 Chronic headache attributed to other systemic infection

10.緣於內環境紊亂的頭痛（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高海拔頭痛

10.1.2 Headache attributed to aeroplane travel飛行頭痛

10.1.3 Diving headache潛水頭痛

10.1.4 Sleep apnoea headache睡眠呼吸暫停性頭痛

10.2 Dialysis headache

10.3 Headache attributed to arterial hypertension高血壓性頭痛

10.3.1 Headache attributed to phaeochromocytoma嗜絡細胞瘤

10.3.2 Headache attributed to hypertensive crisis without hypertensive encephalopathy無高血壓腦病的高血壓危象

10.3.3 Headache attributed to hypertensive encephalopathy高血壓腦病

10.3.4 Headache attributed to pre-eclampsia or eclampsia先兆子癇

10.3.5 Headache attributed to autonomic dysreflexia自主反射障礙

10.4 Headache attributed to hypothyroidism甲狀腺低下

10.5 Headache attributed to fasting禁食

10.6 Cardiac cephalalgia心源性頭痛

10.7 Headache attributed to other disorder of homoeostasis其他內環境穩定失調疾患

11.緣於頭、頸、眼、耳、鼻、鼻竇、牙、口或其他頭面部結構病變的頭面痛（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顱骨

11.2 Headache attributed to disorder of the neck 頸部

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)斜視

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鼻子或鼻竇

11.5.1 Headache attributed to acute rhinosinusitis急性鼻竇炎

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.緣於精神疾病的頭痛（Headache attributed to psychiatric disorder）

12.1 Headache attributed to somatization disorder軀體化障礙

12.2 Headache attributed to psychotic disorder精神疾病

三、腦神經痛、中樞和原發性顏面痛及其他頭痛（painful cranial neuropathies, other facial pains and other headaches）

13.腦神經痛和中樞性顏面痛（Painful cranial neuropathies and other facial pains）

13.1 Trigeminal neuralgia三叉神經痛

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舌咽神經痛

13.3 Nervus intermedius (facial nerve) neuralgia中間神經痛（面神經痛）

13.3.1 Classical nervus intermedius neuralgia

13.3.2 Nervus intermedius neuropathy attributed to Herpes zoster Hunt』s綜合症？

THS診斷標準：A：側頭痛，符合C特徵B：海綿竇、眶上裂或眼眶內肉芽腫，被MRI或活檢證實； 單側的動眼、滑車、展神經麻痹（單個或多個組合）C：頭痛先於眼肌麻痹2周內出現，或與眼神經麻痹同時出現 頭痛部位在單側的眉、眼處D：排除其他

13.4 Occipital neuralgia枕神經痛

13.5 Optic neuritis視神經炎

13.6 Headache attributed to ischaemic ocular motor nerve palsy缺血性眼神經麻痹

13.7 Tolosa-Hunt syndrome

13.8 Paratrigeminal oculosympathetic (Raeder』s) syndrome

13.9 Recurrent painful ophthalmoplegic neuropathy複發性痛性眼肌麻痹

13.10 Burning mouth syndrome (BMS)

13.11 Persistent idiopathic facial pain (PIFP)

13.12 Central neuropathic pain中樞神經痛

13.12.1 Central neuropathic pain attributed to multiple sclerosis (MS)

13.12.2 Central post-stroke pain (CPSP)

14. 其他頭痛（Other headache disorders）