INTERNATIONAL CLASSIFICATION of HEADACHE DISORDERS 3rd edition (beta version) (ICHD-3 beta)

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Presentation transcript:

INTERNATIONAL CLASSIFICATION of HEADACHE DISORDERS 3rd edition (beta version) (ICHD-3 beta)

Third Headache Classification Committee Members Jes Olesen, Denmark (Chairman) Lars Bendtsen, Denmark David Dodick, USA Anne Ducros, France Stefan Evers, Germany Michael First, USA Peter J Goadsby, USA Andrew Hershey, USA Zaza Katsarava, Germany Morris Levin, USA Julio Pascual, Spain Michael B Russell, Norway Todd Schwedt, USA Timothy J Steiner, UK (Secretary) Cristina Tassorelli, Italy Gisela M Terwindt, The Netherlands Maurice Vincent, Brazil Shuu-Jiun Wang, Taiwan

Structure One chapter (1-13) per major group: introduction headache types, subtypes, subforms with: previously used terms disorders that are related but coded elsewhere short descriptions explicit diagnostic criteria notes and comments selected bibliography

Structure Final chapter (14) for: headache not elsewhere classified headache entities still to be described headache unspecified headaches known to be present but insufficiently described

Structure Appendix for: research criteria for novel entities that have not been sufficiently validated alternative diagnostic criteria that may be preferable but for which the evidence is insufficient a first step in eliminating disorders included in the 2nd edition for which sufficient evidence has still not been published

Classification Part 1: Primary headache disorders Part 2: Secondary headache disorders Part 3: Painful cranial neuropathies and other facial pains

Classification Part 1: The primary headaches 1. Migraine 2. Tension-type headache 3. Trigeminal autonomic cephalalgias 4. Other primary headache disorders

Classification Part 2: The secondary headaches 5. Headache attributed to trauma or injury to the head and/or neck 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 Headache attributed to infection 10. Headache attributed to disorder of homoeostasis

Classification Part 2: The secondary headaches 11. Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure 12. Headache attributed to psychiatric disorder

Classification Part 3: Painful cranial neuropathies, other facial pains and other headaches 13. Painful cranial neuropathies and other facial pains 14. Other headache disorders

Part 1: The primary headaches 1. Migraine 2. Tension-type headache 3. Trigeminal autonomic cephalalgias 4. Other primary headache disorders

1. Migraine 1.1 Migraine without aura 1.2 Migraine with aura 1.3 Chronic migraine 1.4 Complications of migraine 1.5 Probable migraine 1.6 Episodic syndromes that may be associated with migraine

1. Migraine Reclassification 2004-2013 1.1 Migraine without aura 1.2 Migraine with aura 1.4 Retinal migraine 1.5 Complications of migraine (including 1.5.1 Chronic migraine) 1.6 Probable migraine 1.3 Childhood periodic syndromes 2013 1.1 Migraine without aura 1.2 Migraine with aura (including 1.2.4 Retinal migraine) 1.3 Chronic migraine 1.4 Complications of migraine 1.5 Probable migraine 1.6 Episodic syndromes that may be associated with migraine

1.1 Migraine without aura A. At least 5 attacks fulfilling criteria B-D B. Headache attacks lasting 4-72 h (untreated or unsuccessfully treated) C. Headache has 2 of the following characteristics: 1. unilateral location 2. pulsating quality 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity (eg, walking, climbing stairs) D. During headache 1 of the following: 1. nausea and/or vomiting 2. photophobia and phonophobia E. Not better accounted for by another ICHD-3 diagnosis

1.1 Migraine without aura Notes When <5 attacks but criteria B-E are met, code as 1.5.1 Probable migraine without aura When attacks occur on 15 d/mo for >3 mo, code as 1.1 Migraine without aura + 1.3 Chronic migraine When patient falls asleep during migraine and wakes without it, duration is reckoned until time of awakening In children and adolescents (aged under 18 y), attacks may last 2-72 h

“Not better accounted for by another ICHD-3 diagnosis” Note This is the last criterion for every headache disorder Consideration of other possible diagnoses (the differential diagnosis) is a routine part of the clinical diagnostic process. When a headache appears to fulfil the criteria for a particular headache disorder, this last criterion is a reminder always to consider other diagnoses that might better explain the headache.

1.2 Migraine with aura 1.2.1 Migraine with typical aura 1.2.2 Migraine with brainstem aura 1.2.3 Hemiplegic migraine 1.2.4 Retinal migraine

1.2 Migraine with aura A. At least 2 attacks fulfilling criteria B and C B. 1 of the following fully reversible aura symptoms: 1. visual; 2. sensory; 3. speech and/or language; 4. motor ; 5. brainstem; 6. retinal C. 2 of the following 4 characteristics: 1. 1 aura symptom spreads gradually over ≥5 min, and/or 2 symptoms occur in succession 2. each individual aura symptom lasts 5-60 min 3. 1 aura symptom is unilateral 4. aura accompanied or followed in <60 min by headache D. Not better accounted for by another ICHD-3 diagnosis, and TIA excluded

1.2.1 Migraine with typical aura A. At least 2 attacks fulfilling criteria B and C B. Aura of visual, sensory and/or speech/language symptoms, each fully reversible, but no motor, brainstem or retinal symptoms C. 2 of the following 4 characteristics: 1. 1 aura symptom spreads gradually over ≥5 min, and/or 2 symptoms occur in succession 2. each individual aura symptom lasts 5-60 min 3. 1 aura symptom is unilateral 4. aura accompanied or followed in <60 min by headache D. Not better accounted for by another ICHD-3 diagnosis, and TIA excluded

1.2.1.1 Typical aura with headache A. Fulfils criteria for 1.2.1 Migraine with typical aura B. Headache, with or without migraine characteristics, accompanies or follows the aura within 60 min

1.2.1.2 Typical aura without headache A. Fulfils criteria for 1.2.1 Migraine with typical aura B. No headache accompanies or follows the aura within 60 min

1.2.2 Migraine with brainstem aura A. At least 2 attacks fulfilling criteria B and C below, and criteria C and D for 1.2.1 Migraine with typical aura B. Aura of fully reversible visual, sensory and/or speech/language symptoms, but not motor or retinal C. 2 of the following brainstem symptoms: 1. dysarthria 2. vertigo 3. tinnitus 4. hypacusis 5. diplopia 6. ataxia 7. decreased level of consciousness

1.2.2 Migraine with brainstem aura Terminology and coding changes 1988-2013 1.2.2 Migraine with brainstem aura was previously classified as 1.2.6 Basilar-type migraine in ICHD-II 1.2.4 Basilar migraine in ICHD-I There is little evidence that the basilar artery or, necessarily, basilar-artery territory is involved

1.2.3 Hemiplegic migraine A. At least 2 attacks fulfilling criteria B and C B. Aura consisting of both of the following: 1. fully reversible motor weakness; 2. fully reversible visual, sensory and/or speech/language symptoms C. 2 of the following 4 characteristics: 1. 1 aura symptom spreads gradually over ≥5 min, and/or 2 symptoms occur in succession 2. each individual non-motor aura symptom lasts 5-60 min, and motor symptoms last <72 h 3. 1 aura symptom is unilateral 4. aura accompanied or followed in <60 min by headache D. Not better accounted for by another ICHD-3 diagnosis, and TIA excluded

1.2.3.1 Familial hemiplegic migraine (FHM) A. Fulfils criteria for 1.2.3 Hemiplegic migraine B. At least one first- or second-degree relative has had attacks fulfilling criteria for 1.2.3 Hemiplegic migraine

1.2.3.1.1 Familial hemiplegic migraine type 1 (FHM1) A. Fulfils criteria for 1.2.3.1 Familial hemiplegic migraine B. A causative mutation on the CACNA1A gene has been demonstrated

1.2.3.1.2 Familial hemiplegic migraine type 2 (FHM2) A. Fulfils criteria for 1.2.3.1 Familial hemiplegic migraine B. A causative mutation on the ATP1A2 gene has been demonstrated

1.2.3.1.3 Familial hemiplegic migraine type 3 (FHM3) A. Fulfils criteria for 1.2.3.1 Familial hemiplegic migraine B. A causative mutation on the SCN1A gene has been demonstrated

1.2.3.2 Sporadic hemiplegic migraine A. Fulfils criteria for 1.2.3 Hemiplegic migraine B. No first- or second-degree relative has had attacks fulfilling criteria for 1.2.3 Hemiplegic migraine

1.2.4 Retinal migraine A. At least 2 attacks fulfilling criteria B and C B. Aura of fully reversible monocular positive and/or negative visual phenomena confirmed during an attack by either or both of the following: 1. clinical visual field examination 2. patient’s drawing of a monocular field defect C. ≥2 of the following 3 characteristics: 1. aura spreads gradually over ≥5 min 2. aura symptoms last 5-60 min 3. aura accompanied or followed in <60 min by headache D. Not better accounted for by another ICHD-3 diagnosis, and other causes of amaurosis fugax excluded

1.3 Chronic migraine A. Headache (TTH-like and/or migraine-like) on ≥15 d/mo for >3 mo and fulfilling criteria B and C B. In a patient who has had ≥5 attacks fulfilling criteria B-D for 1.1 Migraine without aura and/or criteria B and C for 1.2 Migraine with aura C. On ≥8 d/mo for >3 mo fulfilling any of the following: 1. criteria C and D for 1.1 Migraine without aura 2. criteria B and C for 1.2 Migraine with aura 3. believed by the patient to be migraine at onset and relieved by a triptan or ergot derivative D. Not better accounted for by another ICHD-3 diagnosis

1.3 Chronic migraine Note Patients meeting criteria for 1.3 Chronic migraine and for 8.2 Medication-overuse headache should be given both diagnoses After drug withdrawal, migraine will either revert to the episodic subtype or remain chronic, and be re-diagnosed accordingly; in the latter case, the diagnosis of 8.2 Medication-overuse headache may be rescinded

1.4 Complications of migraine 1.4.1 Status migrainosus 1.4.2 Persistent aura without infarction 1.4.3 Migrainous infarction 1.4.4 Migraine aura-triggered seizure

1.4.1 Status migrainosus A. A headache attack fulfilling criteria B and C B. In a patient with 1.1 Migraine without aura and/or 1.2 Migraine with aura, and typical of previous attacks except for its duration and severity C. Both of the following characteristics: 1. unremitting for >72 h 2. pain and/or associated symptoms are debilitating D. Not better accounted for by another ICHD-3 diagnosis

1.5 Probable migraine 1.5.1 Probable migraine without aura 1.5.2 Probable migraine with aura

1.5.1 Probable migraine without aura A. Attacks fulfilling all but one of criteria A-D for 1.1 Migraine without aura B. Not fulfilling ICHD-3 criteria for any other headache disorder C. Not better accounted for by another ICHD-3 diagnosis

1.5.2 Probable migraine with aura A. Attacks fulfilling all but one of criteria A-C for 1.2 Migraine with aura or any of its subforms B. Not fulfilling ICHD-3 criteria for any other headache disorder C. Not better accounted for by another ICHD-3 diagnosis

1.6 Episodic syndromes that may be associated with migraine 1.6.1 Recurrent gastrointestinal disturbance 1.6.1.1 Cyclic vomiting syndrome 1.6.1.2 Abdominal migraine 1.6.2 Benign paroxysmal vertigo 1.6.3 Benign paroxysmal torticollis

2. Tension-type headache (TTH) 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

2.1 Infrequent episodic TTH A. At least 10 episodes of headache occurring on <1 d/mo (<12 d/y) and fulfilling criteria B-D B. Lasting from 30 min to 7 d C. 2 of the following 4 characteristics: 1. bilateral location 2. pressing or tightening (non-pulsating) quality 3. mild or moderate intensity 4. not aggravated by routine physical activity D. Both of the following: 1. no nausea or vomiting 2. no more than one of photophobia or phonophobia E. Not better accounted for by another ICHD-3 diagnosis

2.1 Infrequent episodic TTH 2.1.1 Infrequent episodic tension-type headache associated with pericranial tenderness A. Episodes fulfilling criteria for 2.1 Infrequent episodic tension-type headache B. Increased pericranial tenderness on manual palpation 2.1.2 Infrequent episodic tension-type headache not associated with pericranial tenderness B. No increase in pericranial tenderness

2.2 Frequent episodic TTH A. At least 10 episodes occurring on 1-14 d/mo for >3 mo (12 and <180 d/y) and fulfilling criteria B-D B. Lasting from 30 min to 7 d C. 2 of the following 4 characteristics: 1. bilateral location 2. pressing or tightening (non-pulsating) quality 3. mild or moderate intensity 4. not aggravated by routine physical activity D. Both of the following: 1. no nausea or vomiting 2. no more than one of photophobia or phonophobia E. Not better accounted for by another ICHD-3 diagnosis

2.2 Frequent episodic TTH 2.2.1 Frequent episodic tension-type headache associated with pericranial tenderness A. Episodes fulfilling criteria for 2.2 Frequent episodic tension-type headache B. Increased pericranial tenderness on manual palpation 2.2.2 Frequent episodic tension-type headache not associated with pericranial tenderness B. No increase in pericranial tenderness

2.3 Chronic TTH A. Headache occurring on 15 d/mo on average for >3 mo (180 d/y), fulfilling criteria B-D B. Lasting hours to days, or unremitting C. 2 of the following 4 characteristics: 1. bilateral location 2. pressing/tightening (non-pulsating) quality 3. mild or moderate intensity not aggravated by routine physical activity D. Both of the following: 1. not >1 of photophobia, phonophobia, mild nausea 2. neither moderate or severe nausea nor vomiting E. Not better accounted for by another ICHD-3 diagnosis

2.3 Chronic TTH 2.3.1 Chronic tension-type headache associated with pericranial tenderness A. Headache fulfilling criteria for 2.3 Chronic tension-type headache B. Increased pericranial tenderness on manual palpation 2.3.2 Chronic tension-type headache not associated with pericranial tenderness A. Episodes fulfilling criteria for 2.3 Chronic tension-type headache B. No increase in pericranial tenderness

2.4 Probable TTH 2.4.1 Probable infrequent episodic TTH A. One or more episodes fulfilling all but one of criteria A-D for 2.1 Infrequent episodic tension-type headache B. Not fulfilling ICHD-3 criteria for any other headache disorder C. Not better accounted for by another ICHD-3 diagnosis 2.4.2 Probable frequent episodic TTH A. Episodes fulfilling all but one of criteria A-D for 2.2 Frequent episodic tension-type headache B. Not fulfilling ICHD-3 criteria for any other headache disorder C. Not better accounted for by another ICHD-3 diagnosis

2.4 Probable TTH 2.4.3 Probable chronic TTH A. Headache fulfilling all but one of criteria A-D for 2.3 Chronic tension-type headache B. Not fulfilling ICHD-3 criteria for any other headache disorder C. Not better accounted for by another ICHD-3 diagnosis

3. Trigeminal autonomic cephalalgias (TACs) 3.1 Cluster headache 3.2 Paroxysmal hemicrania 3.3 Short-lasting unilateral neuralgiform headache attacks 3.4 Hemicrania continua 3.5 Probable trigeminal autonomic cephalalgia

3.1 Cluster headache A. At least 5 attacks fulfilling criteria B-D B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 min (when untreated) C. Either or both of the following: 1. 1 of the following ipsilateral symptoms or signs: a) conjunctival injection and/or lacrimation; b) nasal congestion and/or rhinorrhoea; c) eyelid oedema; d) fore-head and facial sweating; e) forehead and facial flushing; f) sensation of fullness in the ear; g) miosis and/or ptosis 2. a sense of restlessness or agitation D. Frequency from 1/2 d to 8/d for > half the time when active E. Not better accounted for by another ICHD-3 diagnosis

3.1 Cluster headache 3.1.1 Episodic cluster headache A. Attacks fulfilling criteria for 3.1 Cluster headache and occurring in bouts (cluster periods) B. 2 cluster periods lasting 7 d to 1 y (when untreated) and separated by pain-free remission periods of 1 mo 3.1.2 Chronic cluster headache A. Attacks fulfilling criteria for 3.1 Cluster headache and criterion B below B. Occurring without a remission period, or with remissions lasting <1 mo, for 1 y

3.2 Paroxysmal hemicrania A. At least 20 attacks fulfilling criteria B-E B. Severe unilateral orbital, supraorbital and/or temporal pain lasting 2-30 min C. 1 of the following ipsilateral symptoms or signs: 1. conjunctival injection and/or lacrimation 2. nasal congestion and/or rhinorrhoea 3. eyelid oedema 4. forehead and facial sweating 5. forehead and facial flushing 6. sensation of fullness in the ear 7. miosis and/or ptosis D. Frequency >5/d for > half the time E. Prevented absolutely by therapeutic doses of indometacin F. Not better accounted for by another ICHD-3 diagnosis

3.2 Paroxysmal hemicrania 3.2.1 Episodic paroxysmal hemicrania A. Attacks fulfilling criteria for 3.2 Paroxysmal hemicrania and occurring in bouts B. 2 bouts lasting 7d to 1 y (when untreated) and separated by pain-free remission periods of 1 mo 3.2.2 Chronic paroxysmal hemicrania A. Attacks fulfilling criteria for 3.2 Paroxysmal hemicrania B. Occurring without a remission period, or with remission periods lasting <1 mo, for 1 y

3.3 Short-lasting unilateral neuralgiform headache attacks A. At least 20 attacks fulfilling criteria B-D B. Moderate or severe unilateral head pain, with orbital, supraorbital, temporal and/or other trigeminal distribution, lasting 1-600 s and occurring as single stabs, series of stabs or in a saw-tooth pattern C. 1 of the following ipsilateral cranial autonomic symptoms or signs: 1. conjunctival injection and/or lacrimation; 2. nasal congestion and/or rhinorrhoea; 3. eyelid oedema; 4. forehead and facial sweating; 5. forehead and facial flushing; 6. sensation of fullness in the ear; 7. miosis and/or ptosis D. Frequency 1/d for > half the time when active E. Not better accounted for by another ICHD-3 diagnosis

3.3 Short-lasting unilateral neuralgiform headache attacks New terminology and subdivision Two subtypes recognized: 3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) 3.3.2 Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) 3.3.1 SUNCT may be a subform of 3.3.2 SUNA, although this requires further study.

3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks B. Both of conjunctival injection and lacrimation (tearing)

3.3.1 SUNCT 3.3.1.1 Episodic SUNCT A. Attacks fulfilling criteria for 3.3.1 SUNCT and occurring in bouts B. 2 bouts lasting 7d to 1 y and separated by pain-free remission periods of 1 mo 3.3.1.2 Chronic SUNCT A. Attacks fulfilling criteria for 3.3.1 SUNCT, and criterion B below B. Occurring without a remission period, or with remissions lasting <1 mo, for 1 y

3.3.2 Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks B. Only one or neither of conjunctival injection and lacrimation (tearing)

3.3.2 SUNA 3.3.2.1 Episodic SUNA A. Attacks fulfilling criteria for 3.3.2 SUNA and occurring in bouts B. 2 bouts lasting 7d to 1 y and separated by pain-free remission periods of 1 mo 3.3.2.2 Chronic SUNA A. Attacks fulfilling criteria for 3.3.2 SUNA, and criterion B below B. Occurring without a remission period, or with remissions lasting <1 mo, for 1 y

3.4 Hemicrania continua Recoded from 4.7, and revised criteria A. Unilateral headache fulfilling criteria B-D B. Present >3 mo, with exacerbations of moderate or greater intensity C. Either or both of the following: 1. 1 of the following ipsilateral symptoms or signs: a) conjunctival injection and/or lacrimation; b) nasal congestion and/or rhinorrhoea; c) eyelid oedema; d) fore-head and facial sweating; e) forehead and facial flushing; f) sensation of fullness in the ear; g) miosis and/or ptosis 2. a sense of restlessness or agitation, or aggravation of pain by movement D. Responds absolutely to therapeutic doses of indometacin E. Not better accounted for by another ICHD-3 diagnosis

3.4 Hemicrania continua New subdivision 3.4.1 Hemicrania continua, remitting subtype A. Headache fulfilling criteria for 3.4 Hemicrania continua, and criterion B below B. Headache is not daily or continuous, but interrupted by remission periods of ≥1 d without treatment 3.4.2 Hemicrania continua, unremitting subtype B. Headache is daily and continuous for ≥1 y, without remission periods of ≥1 d

3.4 Probable TAC A. Headache attacks fulfilling all but one of criteria A-D for 3.1 Cluster headache, criteria A-E for 3.2 Paroxysmal hemicrania, criteria A-D for 3.3 Short-lasting unilateral neuralgiform headache attacks or criteria A-D for 3.4 Hemicrania continua B. Not fulfilling ICHD-3 criteria for any other headache disorder C. Not better accounted for by another ICHD-3 diagnosis

4. Other primary headache disorders 4.1 Primary cough headache 4.2 Primary exercise headache 4.3 Primary headache associated with sexual activity 4.4 Primary thunderclap headache 4.5 Cold-stimulus headache 4.6 External pressure headache 4.7 Primary stabbing headache 4.8 Nummular headache 4.9 Hypnic headache 4.10 New daily persistent headache (NDPH)

4.1 Primary cough headache A. At least 2 headache episodes fulfilling criteria B-D B. Brought on by and occurring only in association with coughing, straining and/or other Valsalva manœuvre C. Sudden onset D. Lasting between 1 s and 2 h E. Not better accounted for by another ICHD-3 diagnosis

4.2 Primary exercise headache A. At least 2 headache episodes fulfilling criteria B and C B. Brought on by and occurring only during or after strenuous physical exercise C. Lasting <48 h D. Not better accounted for by another ICHD-3 diagnosis

4.3 Primary headache associated with sexual activity A. At least 2 episodes of pain in the head and/or neck fulfilling criteria B-D B. Brought on by and occurring only during sexual activity C. Either or both of the following: 1. increasing in intensity with increasing sexual excitement 2. abrupt explosive intensity just before or with orgasm D. Lasting from 1 min to 24 h with severe intensity and/or up to 72 h with mild intensity E. Not better accounted for by another ICHD-3 diagnosis

4.3 Primary headache associated with sexual activity Subforms no longer recognised Two subforms (preorgasmic headache and orgasmic headache) were included in ICHD-I and ICHD-II, but clinical studies have since been unable to distinguish these; therefore, 4.3 Primary headache associated with sexual activity is now regarded as a single entity with variable presentation

4.4 Primary thunderclap headache A. Severe head pain fulfilling criteria B and C B. Abrupt onset, reaching maximum intensity in <1 min C. Lasting for ≥5 min D. Not better accounted for by another ICHD-3 diagnosis

4.5 Cold-stimulus headache Recoded from 13.11 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

4.5.1 Headache attributed to external application of a cold stimulus A. At least 2 acute headache episodes fulfilling criteria B and C B. Brought on by and occurring only during application of an external cold stimulus to the head C. Resolving within 30 min after removal of the cold stimulus D. Not better accounted for by another ICHD-3 diagnosis

4.5.2 Headache attributed to ingestion or inhalation of a cold stimulus A. At least 2 episodes of acute frontal or temporal headache fulfilling criteria B and C B. Brought on by and occurring immediately after a cold stimulus to the palate and/or posterior pharyngeal wall from ingestion of cold food or drink or inhalation of cold air C. Resolving within 10 min after removal of the cold stimulus D. Not better accounted for by another ICHD-3 diagnosis

4.6 External-pressure headache 4.6.1 External-compression headache 4.6.2 External-traction headache

4.6.1 External-compression headache Recoded from 13.10 A. At least 2 episodes of headache fulfilling criteria B-D B. Brought on by and occurring within 1 h during sustained external compression of the forehead or scalp C. Maximal at the site of external compression D. Resolving within 1 h after external compression is relieved E. Not better accounted for by another ICHD-3 diagnosis

4.6.2 External-traction headache A. At least 2 episodes of headache fulfilling criteria B-D B. Brought on by and occurring within 1 h during sustained external traction on the scalp C. Maximal at the traction site D. Resolving within 1 h after traction is relieved E. Not better accounted for by another ICHD-3 diagnosis

4.7 Primary stabbing headache A. Head pain occurring spontaneously as a single stab or series of stabs and fulfilling criteria B-D B. Each stab lasts for up to a few seconds C. Stabs recur with irregular frequency, from one to many per day D. No cranial autonomic symptoms E. Not better accounted for by another ICHD-3 diagnosis

4.8 Nummular headache New entrant to main classification, recoded from A13.7.1 A. Continuous or intermittent head pain fulfilling criterion B B. Felt exclusively in an area of the scalp, with all of the following 4 characteristics: 1. sharply-contoured 2. fixed in size and shape 3. round or elliptical 4. 1-6 cm in diameter C. Not better accounted for by another ICHD-3 diagnosis

4.9 Hypnic headache A. Recurrent headache attacks fulfilling criteria B-E B. Developing only during sleep, and causing wakening C. Occurring on ≥10 d/mo for >3 mo D. Lasting ≥15 min and for up to 4 h after waking E. No cranial autonomic symptoms or restlessness F. Not better accounted for by another ICHD-3 diagnosis

4.10 New daily persistent headache (NDPH) Revised criteria A. Persistent headache fulfilling criteria B and C B. Distinct and clearly-remembered onset, with pain becoming continuous and unremitting within 24 h C. Present for >3 mo D. Not better accounted for by another ICHD-3 diagnosis

Part 2: The secondary headaches 5. Headache attributed to trauma or injury to the head and/or neck 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. Headache 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 structure 12. Headache attributed to psychiatric disorder

General diagnostic criteria for secondary headaches A. Any headache fulfilling criterion C B. Another disorder scientifically documented to be able to cause headache has been diagnosed C. Evidence of causation demonstrated by ≥2 of the following: 1. headache has developed in temporal relation to the onset of the presumed causative disorder 2. one or both of the following: a) headache has significantly worsened in parallel with worsening of the presumed causative disorder b) headache has significantly improved in parallel with improvement of the presumed causative disorder 3. headache has characteristics typical for the causative disorder 4. other evidence exists of causation D. Not better accounted for by another ICHD-3 diagnosis

Terminology change for secondary headaches During the first three months from onset, secondary headaches are considered acute. When they continue beyond that period they are designated persistent. This time period is consistent with ICHD-II diagnostic criteria, but the term persistent has been adopted in place of chronic. The term chronic is used only when headache persists because of chronicity (beyond 3 months) of the underlying disorder (eg, 9.1.1.2 Chronic headache attributed to bacterial meningitis or meningoencephalitis)

5. Headache attributed to head and/or neck trauma 5.1 Acute headache attributed to traumatic injury to the head 5.2 Persistent headache attributed to 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

5.1 Acute headache attributed to traumatic injury to the head A. Any headache fulfilling criteria C and D B. Traumatic injury to the head has occurred C. Headache is reported to have developed within 7 d after one of the following: 1. the injury to the head 2. regaining of consciousness following the injury 3. discontinuation of medication(s) that impair ability to sense or report headache following the injury D. Either of the following: 1. headache has resolved within 3 mo after the injury 2. headache has not resolved but 3 mo have not yet passed E. Not better accounted for by another ICHD-3 diagnosis

5.1 Persistent headache attributed to traumatic injury to the head A. Any headache fulfilling criteria C and D B. Traumatic injury to the head has occurred C. Headache is reported to have developed within 7 d after one of the following: 1. the injury to the head 2. regaining of consciousness following the injury 3. discontinuation of medication(s) that impair ability to sense or report headache following the injury D. Headache persists for >3 mo after injury to the head E. Not better accounted for by another ICHD-3 diagnosis

5.3 Acute headache attributed to whiplash A. Any headache fulfilling criteria C and D B. Whiplash, associated at the time with neck pain and/or headache, has occurred C. Headache has developed within 7 d after whiplash D. Either of the following: 1. headache has resolved within 3 mo after whiplash 2. headache has not resolved but 3 mo have not yet passed E. Not better accounted for by another ICHD-3 diagnosis

5.3 Persistent headache attributed to whiplash A. Any headache fulfilling criteria C and D B. Whiplash, associated at the time with neck pain and/or headache, has occurred C. Headache has developed within 7 d after whiplash D. Headache persists for >3 mo after whiplash E. Not better accounted for by another ICHD-3 diagnosis

6. Headache attributed to cranial or cervical vascular disorder 6.1 Headache attributed to ischaemic stroke or TIA 6.2 Headache attributed to non-traumatic intracranial haemorrhage 6.3 Headache attributed to unruptured vascular malformation 6.4 Headache attributed to arteritis 6.5 Headache attributed to cervical carotid or vertebral artery disorder 6.6 Headache attributed to cerebral venous thrombosis 6.7 Headache attributed to other acute intracranial arterial disorder 6.8 Headache attributed to genetic vasculopathy 6.9 Headache attributed to pituitary apoplexy

6.2 Headache attributed to non-traumatic intracranial haemorrhage 6.2.1 Headache attributed to non-traumatic intracerebral haemorrhage 6.2.2 Headache attributed to non-traumatic subarachnoid haemorrhage (SAH)

6.2.2 Headache attributed to non-traumatic SAH A. Any new headache fulfilling criterion C B. SAH in the absence of head trauma diagnosed C. Evidence of causation demonstrated by ≥2 of the following: 1. headache has developed in close temporal relation to other symptoms and/or clinical signs of SAH, or led to diagnosis of SAH 2. headache has significantly improved in parallel with stabilization or improvement of other symptoms or clinical or radiological signs of SAH 3. headache has sudden or thunderclap onset D. Not better accounted for by another ICHD-3 diagnosis

6.3 Headache attributed to unruptured vascular malformation 6.3.1 Headache attributed to unruptured saccular aneurysm 6.3.2 Headache attributed to arteriovenous malformation (AVM) 6.3.3 Headache attributed to dural arteriovenous fistula 6.3.4 Headache attributed to cavernous angioma 6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome)

6.4 Headache attributed to arteritis 6.4.1 Headache attributed to giant cell arteritis (GCA) 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)

6.4.1 Headache attributed to GCA A. Any new headache fulfilling criterion C B. Giant cell arteritis (GCA) diagnosed C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in close temporal relation to other symptoms and/or clinical or biological signs of onset of GCA, or led to diagnosis of GCA 2. either or both of: a) headache has significantly worsened in parallel with worsening of GCA; b) headache has significantly improved or resolved within 3 d of high-dose steroid treatment 3. headache is associated with scalp tenderness and/or jaw claudication D. Not better accounted for by another ICHD-3 diagnosis

6.6 Headache attributed to CVT A. Any new headache fulfilling criterion C B. Cerebral venous thrombosis (CVT) diagnosed C. Evidence of causation demonstrated by both of the following: 1. headache has developed in close temporal relation to other symptoms and/or clinical signs of CVT, or led to discovery of CVT 2. either or both of: a) headache has significantly worsened in parallel with clinical or radiological signs of extension of CVT; b) headache has significantly improved or resolved after improvement of CVT D. Not better accounted for by another ICHD-3 diagnosis

6.7 Headache attributed to other acute 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) 6.7.4 Headache attributed to intracranial arterial dissection

6.7.3 Headache attributed to RCVS A. Any new headache fulfilling criterion C B. Reversible cerebral vasoconstriction syndrome (RCVS) diagnosed C. Evidence of causation demonstrated by ≥1 of: 1. headache, with or without focal deficits and/or seizures, has led to angiography and diagnosis of RCVS 2. headache has ≥1 of the following characteristics: a) recurrent during ≤1 mo, and with thunderclap onset b) triggered by sexual activity, exertion, Valsalva manœuvres, emotion, bathing and/or showering 3. no new significant headache occurs >1 mo after onset D. Not better accounted for by another ICHD-3 diagnosis, and aneurysmal SAH excluded

7. Headache attributed to non-vascular intracranial disorder 7.1 Headache attributed to increased cerebrospinal fluid pressure 7.2 Headache attributed to low cerebrospinal fluid pressure 7.3 Headache attributed to non-infectious inflammatory intracranial disease 7.4 Headache attributed to intracranial neoplasia 7.5 Headache attributed to intrathecal injection 7.6 Headache attributed to epileptic seizure 7.7 Headache attributed to Chiari malformation type I 7.8 Headache attributed to other non-vascular intracranial disorder

7.1 Headache attributed to increased CSF pressure 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

7.1.1 Headache attributed to IIH A. Any headache fulfilling criterion C B. Idiopathic intracranial hypertension (IIH) diagnosed, with CSF pressure >250 mm CSF C. Evidence of causation demonstrated by ≥2 of the following: 1. headache has developed in temporal relation to IIH, or led to its discovery 2. headache is relieved by reducing intracranial hypertension 3. headache is aggravated in temporal relation to increase in intracranial pressure D. Not better accounted for by another ICHD-3 diagnosis

7.2 Headache attributed to low cerebrospinal fluid pressure 7.2.1 Post-dural puncture headache 7.2.2 CSF fistula headache 7.2.3 Headache attributed to spontaneous intracranial hypotension

7.2.1 Post-dural puncture headache A. Any headache fulfilling criterion C B. Dural puncture has been performed C. Headache has developed within 5 d of dural puncture D. Not better accounted for by another ICHD-3 diagnosis

7.4 Headache attributed to intracranial neoplasia 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 7.4.3 Headache attributed to hypothalamic or pituitary hyper- or hyposecretion

7.4.1 Headache attributed to intracranial neoplasm A. Headache fulfilling criterion C B. Space-occupying intracranial neoplasm demonstrated C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in temporal relation to development of the neoplasm, or led to its discovery 2. either or both of: a) headache has significantly worsened in parallel with worsening of the neoplasm; b) headache has significantly improved in temporal relation to successful treatment of the neoplasm 3. headache has ≥1 of the following 3 characteristics: a) progressive; b) worse in the morning or after daytime napping; c) aggravated by Valsalva-like manœuvres D. Not better accounted for by another ICHD-3 diagnosis

7.4.1.1 Headache attributed to colloid cyst of the third ventricle A. Headache fulfilling criterion C B. A colloid cyst of third ventricle demonstrated C. Evidence of causation demonstrated by both of: 1. headache has developed in temporal relation to development of colloid cyst, or led to its discovery 2. either or both of the following: a) headache is recurrent, with thunderclap onset and accompanied by reduced level or loss of consciousness b) headache has significantly improved or resolved in temporal relation to successful treatment of colloid cyst D. Not better accounted for by another ICHD-3 diagnosis

7.6 Headache attributed to epileptic seizure 7.6.1 Hemicrania epileptica 7.6.2 Post-ictal headache

7.6.2 Post-seizure (post-ictal) headache A. Any headache fulfilling criterion C B. Patient has recently had a partial or generalized epileptic seizure C. Evidence of causation demonstrated by both of the following: 1. headache has developed within 3 h after epileptic seizure has terminated 2. headache has resolved within 72 h after epileptic seizure has terminated D. Not better accounted for by another ICHD-3 diagnosis

8. Headache attributed to a substance or its withdrawal 8.1 Headache attributed to use of or exposure to a substance 8.2 Medication-overuse headache (MOH) 8.3 Headache attributed to substance withdrawal

8.1 Headache attributed to use of or exposure to a substance 8.1.1 Nitric oxide (NO) donor-induced headache 8.1.2 Phosphodiesterase (PDE) inhibitor-induced headache 8.1.3 Carbon monoxide (CO)-induced headache 8.1.4 Alcohol-induced headache. 8.1.5 Headache induced by food and/or additive 8.1.6 Cocaine-induced headache 8.1.7 Histamine-induced headache 8.1.8 Calcitonin gene-related peptide (CGRP)-induced headache

8.1 Headache attributed to use of or exposure to a substance 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

8.1.3 Carbon monoxide (CO)-induced headache A. Bilateral headache fulfilling criterion C B. Exposure to carbon monoxide (CO) has occurred C. Evidence of causation demonstrated by all of the following: 1. headache has developed within 12 h of exposure to CO 2. headache intensity varies with the severity of CO intoxication 3. headache has resolved within 72 h of elimination of CO D. Not better accounted for by another ICHD-3 diagnosis

8.1.12 Headache attributed to exogenous hormone A. Any headache fulfilling criterion C B. Regular intake of one or more exogenous hormones C. Evidence of causation demonstrated by both of: 1. headache has developed in temporal relation to the commencement of hormone intake 2. ≥1 of the following: a) headache has significantly worsened after an increase in dosage of hormone b) headache has significantly improved or resolved after a reduction in dosage of hormone c) headache has resolved after cessation of hormone intake D. Not better accounted for by another ICHD-3 diagnosis

8.2 Medication-overuse headache (MOH) 8.2.1 Ergotamine-overuse headache 8.2.2 Triptan-overuse headache 8.2.3 Simple analgesic-overuse headache 8.2.4 Opioid-overuse headache 8.2.5 Combination -analgesic-overuse headache 8.2.6 MOH attributed to multiple drug classes not individually overused 8.2.7 MOH headache attributed to unverified overuse of multiple drug classes 8.2.8 MOH attributed to other medication

8.2 Medication-overuse headache (MOH) A. Headache occurring on ≥15 d/mo in a patient with a pre-existing headache disorder B. Regular overuse for >3 mo of one or more drugs that can be taken for acute and/or symptomatic treatment of headache C. Not better accounted for by another ICHD-3 diagnosis

8.2.1 Ergotamine-overuse headache A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Regular intake of ergotamine on ≥10 d/mo for >3 mo

8.2.2 Triptan-overuse headache A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Regular intake of one or more triptans, in any formulation, on ≥10 d/mo for >3 mo

8.2.3 Simple analgesic-overuse headache 8.2.3.1 Paracetamol (acetaminophen)-overuse headache A. Headache fulfilling criteria for 8.2 MOH B. Regular intake of paracetamol on ≥15 d/mo for >3 mo 8.2.3.2 Acetylsalicylic acid-overuse headache B. Regular intake of ASA on ≥15 d/mo for >3 mo 8.2.3.3 Other non-steroidal anti-inflammatory drug (NSAID)-overuse headache B. Regular intake of one or more NSAIDs other than ASA on ≥15 d/mo for >3 mo

8.2.4 Opioid-overuse headache A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Regular intake of one or more opioids on ≥10 d/mo for >3 mo

8.2.5 Combination-analgesic-overuse headache A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Regular intake of one or more combination-analgesic medications on ≥10 d/mo for >3 mo Note: “Combination-analgesic” is used specifically for formulations combining drugs of two or more classes, each with analgesic effect or acting as adjuvants

8.2.6 MOH attributed to multiple drug classes not individually overused A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Regular intake of any combination of ergotamine, triptans, simple analgesics, NSAIDs and/or opioids on a total of ≥10 d/mo for >3 mo without overuse of any single drug or drug class alone Note: “Without overuse of any single drug or drug class alone” means criterion B has not been fulfilled for any of the specific subforms 8.2.1-8.2.5

8.2.7 MOH attributed to unverified overuse of multiple drug classes A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Both of the following: 1. regular intake of any combination of ergotamine, triptans, simple analgesics, NSAIDs and/or opioids on ≥10 d/mo for >3 mo 2. identity, quantity and/or pattern of use or overuse of these classes of drug cannot be reliably established

8.2.8 MOH attributed to other medication A. Headache fulfilling criteria for 8.2 Medication-overuse headache B. Regular overuse, on ≥10 d/mo for >3 mo, of one or more medications other than those described above, taken for acute or symptomatic treatment of headache

8.3 Headache attributed to substance withdrawal 8.3.1 Caffeine-withdrawal headache 8.3.2 Opioid-withdrawal headache 8.3.3 Estrogen-withdrawal headache 8.3.4 Headache attributed to withdrawal from chronic use of other substance

8.4.1 Caffeine-withdrawal headache A. Headache fulfilling criterion C B. Caffeine consumption of >200 mg/d for >2 wk, which has been interrupted or delayed C. Evidence of causation demonstrated by both of: 1. headache has developed within 24 h after last caffeine intake 2. either or both of the following: a) headache is relieved within 1 h by intake of caffeine 100 mg b) headache has resolved within 7 d after total caffeine withdrawal D. Not better accounted for by another ICHD-3 diagnosis

8.4.3 Estrogen-withdrawal headache A. Headache or migraine fulfilling criterion C B. Daily use of exogenous estrogen for ≥3 wk, which has been interrupted C. Evidence of causation demonstrated by both of: 1. headache or migraine has developed within 5 d after last use of estrogen 2. headache or migraine has resolved within 3 d of its onset D. Not better accounted for by another ICHD-3 diagnosis

9. Headache attributed to infection 9.1 Headache attributed to intracranial infection 9.2 Headache attributed to systemic infection

9.1 Headache attributed to intracranial infection 9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis 9.1.2 Headache attributed to viral meningitis or encephalitis 9.1.3 Headache attributed to intracranial fungal or other parasitic infection 9.1.4 Headache attributed to brain abscess 9.1.5 Headache attributed to subdural empyema

9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis A. Headache of any duration fulfilling criterion C B. Bacterial meningitis or meningoencephalitis diagnosed C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in temporal relation to onset of bacterial meningitis or meningoencephalitis 2. headache has significantly worsened in parallel with worsening of bacterial meningitis or meningoencephalitis 3. headache has significantly improved in parallel with improvement in bacterial meningitis or meningoencephalitis 4. headache is either or both of: a) holocranial; b) located in nuchal area and associated with neck stiffness D. Not better accounted for by another ICHD-3 diagnosis

9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis Note Three subforms described because pathophysiology and treatment differ depending on whether infection has been completely eradicated or remains active: 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 bacterial meningitis or meningoencephalitis

9.1.1.1 Acute headache attributed to bacterial meningitis or meningoencephalitis A. Headache fulfilling criteria for 9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis, and criterion C below B. Bacterial meningitis or meningoencephalitis remains active or has recently resolved C. Headache has been present for <3 mo

9.1.1.2 Chronic headache attributed to bacterial meningitis or meningoencephalitis A. Headache fulfilling criteria for 9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis, and criterion C below B. Bacterial meningitis or meningoencephalitis remains active or has resolved within last 3 mo C. Headache has been present for >3 mo

9.1.1.3 Persistent headache attributed to bacterial meningitis or meningoencephalitis A. Headache fulfilling criteria for 9.1.1 Headache attributed to bacterial meningitis or meningoencephalitis, and criterion C below B. Bacterial meningitis or meningoencephalitis has resolved C. Headache has persisted for >3 mo after resolution of bacterial meningitis or meningoencephalitis D. Not better accounted for by another ICHD-3 diagnosis

9.2 Headache attributed to systemic infection 9.2.1 Headache attributed to systemic bacterial infection 9.2.2 Headache attributed to systemic viral infection 9.2.3 Headache attributed to other systemic infection

10. Headache attributed to disorder of homoeostasis 10.1 Headache attributed to hypoxia and/or hypercapnia 10.2 Dialysis headache 10.3 Headache attributed to arterial hypertension 10.4 Headache attributed to hypothyroidism 10.5 Headache attributed to fasting 10.6 Cardiac cephalalgia 10.7 Headache attributed to other disorder of homoeostasis

10.1 Headache attributed to hypoxia and/or hypercapnia 10.1.1 High-altitude headache 10.1.2 Headache attributed to aeroplane travel 10.1.3 Diving headache 10.1.4 Sleep apnoea headache

10.3 Headache attributed to arterial hypertension 10.3.1 Headache attributed to phaeochromocytoma 10.3.2 Headache attributed to hypertensive crisis without hypertensive encephalopathy 10.3.3 Headache attributed to hypertensive encephalopathy 10.3.4 Headache attributed to pre-eclampsia or eclampsia 10.3.5 Headache attributed to autonomic dysreflexia

11. 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 11.2 Headache attributed to disorder of neck 11.3 Headache attributed to disorder of eyes 11.4 Headache attributed to disorder of ears 11.5 Headache attributed to nose or paranasal sinuses 11.6 Headache attributed to disorder of teeth or jaws 11.7 Headache or facial pain attributed to temporomandibular disorder (TMD) 11.8 Head or facial pain attributed to inflammation of stylohyoid ligament 11.9 Headache or facial pain attributed to other disorder of these structures

11.2.1 Cervicogenic headache A. Any headache fulfilling criterion C B. Clinical, laboratory and/or imaging evidence of a disorder or lesion within cervical spine or soft tissues of neck, known to be able to cause headache C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in temporal relation to onset of cervical disorder or appearance of lesion 2. headache has significantly improved or resolved in parallel with improvement in or resolution of cervical disorder or lesion 3. cervical range of motion is reduced and headache is made significantly worse by provocative manœuvres 4. headache is abolished following diagnostic blockade of a cervical structure or its nerve supply D. Not better accounted for by another ICHD-3 diagnosis

11.3.1 Headache attributed to acute glaucoma A. Any headache fulfilling criterion C B. Acute narrow-angle glaucoma diagnosed C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in temporal relation to onset of glaucoma 2. headache has significantly worsened in parallel with progression of glaucoma 3. headache has significantly improved or resolved in parallel with improvement in or resolution of glaucoma 4. pain location includes affected eye D. Not better accounted for by another ICHD-3 diagnosis

11.7 Headache attributed to temporomandibular disorder (TMD) A. Any headache fulfilling criterion C B. Clinical and/or imaging evidence of TMD C. Evidence of causation demonstrated by ≥2 of: 1. headache has developed in temporal relation to onset of TMD 2. either or both of: a) headache has significantly worsened in parallel with progression of TMD; b) headache has significantly improved or resolved in parallel with improvement in or resolution of TMD 3. headache produced or exacerbated by active jaw movements, passive movements through range of motion of jaw and/or provocative manœuvres such as pressure on TMJ and surrounding muscles of mastication 4. headache, when unilateral, is ipsilateral to TMD D. Not better accounted for by another ICHD-3 diagnosis

12. Headache attributed to psychiatric disorder 12.1 Headache attributed to somatization disorder 12.2 Headache attributed to psychotic disorder

Part 3: Painful cranial neuropathies, other facial pains and other headaches 13. Painful cranial neuropathies and other facial pains 14. Other headache disorders

13. Painful cranial neuropathies and other facial pains 13.1 Trigeminal neuralgia 13.2 Glossopharyngeal neuralgia 13.3 Nervus intermedius (facial nerve) neuralgia 13.4 Occipital neuralgia 13.5 Optic neuritis 13.6 Headache attributed to ischaemic ocular motor nerve palsy 13.7 Tolosa-Hunt syndrome 13.8 Paratrigeminal oculosympathetic (Raeder’s) syndrome 13.9 Recurrent painful ophthalmoplegic neuropathy 13.10 Burning mouth syndrome (BMS) 13.11 Persistent idiopathic facial pain (PIFP) 13.12 Central neuropathic pain

13.1 Trigeminal neuralgia 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

13.1.1 Classical trigeminal neuralgia A. At least 3 attacks of unilateral facial pain fulfilling criteria B and C B. In ≥1 divisions of trigeminal nerve, with no radiation beyond trigeminal distribution C. Pain has ≥3 of the following 4 characteristics: 1. recurring in paroxysmal attacks lasting from a fraction of a second to 2 min 2. severe intensity 3. electric shock-like, shooting, stabbing or sharp in quality 4. precipitated by innocuous stimuli to affected side of face D. No clinically evident neurological deficit E. Not better accounted for by another ICHD-3 diagnosis

13.4 Occipital neuralgia A. Unilateral or bilateral pain fulfilling criteria B-E B. In distribution of greater, lesser and/or third occipital nerves C. ≥2 of the following 3 characteristics: 1. recurring in paroxysmal attacks lasting from a few seconds to minutes 2. severe intensity 3. shooting, stabbing or sharp in quality D. Associated with both of the following: 1. dysaesthesia and/or allodynia apparent during innocuous stimulation of scalp and/or hair 2. either or both of: a) tenderness over affected nerve branches; b) trigger points at emergence of greater occipital nerve or in area of distribution of C2 E. Eased temporarily by local anaesthetic block of affected nerve F. Not better accounted for by another ICHD-3 diagnosis

13.10 Burning mouth syndrome (BMS) A. Oral pain fulfilling criteria B and C B. Recurring daily for >2 h per day for >3 mo C. Pain has both of the following characteristics: 1. burning quality 2. felt superficially in the oral mucosa D. Oral mucosa is of normal appearance and clinical examination including sensory testing is normal E. Not better accounted for by another ICHD-3 diagnosis

13.18.4 Persistent idiopathic facial pain (PIFP) A. Facial and/or oral pain fulfilling criteria B and C B. Recurring daily for >2 h per day for >3 mo C. Pain has both of the following characteristics: 1. poorly localized, and not following distribution of a peripheral nerve 2. dull, aching or nagging quality D. Clinical neurological examination is normal E. Dental cause excluded by appropriate investigations F. Not better accounted for by another ICHD-3 diagnosis

14. Other headache disorders 14.1 Headache not elsewhere classified 14.2 Headache unspecified

14.1 Headache not elsewhere classified A. Headache with characteristic features suggesting that it is a unique diagnostic entity B. Does not fulfil criteria for any of the headache disorders described in chapters 1-13

14.2 Headache unspecified A. Headache is or has been present B. Not enough information is available to classify the headache at any level of this classification

Appendix Presents research criteria for a number of novel entities that have not been sufficiently validated Presents alternative diagnostic criteria that may be preferable but for which the evidence is insufficient Is a first step in eliminating disorders included in the 2nd edition for which sufficient evidence has still not been published

Copyright The International Classification of Headache Disorders, 3rd edition beta version, is published in Cephalalgia 2013; 33: 629-808 It may be reproduced freely for scientific or clinical uses by institutions, societies or individuals Otherwise, copyright belongs exclusively to International Headache Society