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Origins of Pain in the Head

• Extra-cranial pain sensitive structures:– Sinuses– Eyes/orbits– Ears– Teeth– TMJ – Blood vessels– 5,7,9,10 cranial nerves carry pain from thes

strucure

• Intra-cranial pain sensitive structures:– Arteries of circle of willis and proximal dural

arteries,– Dural Venous sinuses,veins– Meninges– Dura

Classification of Headaches

• PRIMARY - NO structural or metabolic abnormality:

– Tension– Migraine– Cluster

• SECONDARY – structural or metabolic abnormality:– Extracranial: sinusitis, otitis media, glaucoma, TMJ ds– Inracranial: SAH, vasculitis, dissection, central vein thrombosis, tumor,

abscess, meningitis– Metabolic disorders: CO2 retention, CO poisoing

RED Flags

• New onset headache in a patient >50 y.o.• Sudden, worst headache of one’s life• Morning headache associated with N/V• Fever, weight loss• Worsens with valsalva maneuvers• Focal neurologic deficits, jaw claudication• Altered LOC• Hx of trauma, cancer or HIV

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

Part 1:The primary headaches

1. Migraine

2. Tension-type headache

3. Cluster headacheand other trigeminal autonomiccephalalgias

4. Other primary headaches

Primary Headache Types

Migraine Tension Cluster

Pain Description

Throbbing, moderate to

severe, worse w/exertion

Pressure, tightness, waxes and

wanes

Abrupt onset, deep,

continuous, excruciating,

explosive

Associated Symptoms

Photo/phono-phobia, n/v, aura

None Tearing, congestion, rhinorrhea,

pallor, sweating

Bajwa and Wootton. Up to Date 2007

Primary Headache Types

Migraine Tension Cluster

Location 60-70% unilateral

Bilateral Unilateral

Duration 4-72 hr Variable 0.5-3 hr, many per day

Patient Appearance

Resting in quiet dark

room; young female

Remains active or prefers to

rest

Remains active, prefers

hot shower, male, smoker

Bajwa and Wootton. Up to Date 2007

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1. Migraine

1.1 Migraine without aura1.2 Migraine with aura1.3 Childhood periodic syndromes that are

commonly precursors of migraine1.4 Retinal migraine1.5 Complications of migraine1.6 Probable migraine

Pathophysiology

• Brainstem neuronal hyperexcitability

• Cortical spreading depression w/aura

• Abnormalities of 5-HT, CGRP, NE, DA, GABA, glutamate, NO, and endorphins

• Trigeminal Activation

Marcus, DA. Headache Simplified 2008.

Presymptomatic hyperexcitabilty increases brain stem response to triggers

Release of Neurotransmitters

(5-HT, NE, DA, GABA, Glutamate, NO, CGRP, Substance P, Estrogen)

Neurotransmitters activate the Trigeminal Nucleus

Dilation of Meningeal blood

vessels (Throbbing)

Activation of Area Postrema

(N/V)

Activation of Hypothalamus

(Hypersensitivity)

Activation of cervical trigeminal system (Muscle

spasm)Activation of Cortex and

Thalamus (Head pain)

Marcus, DA. Headache Simplified 2008.

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.1 Migraine without auraA.At least 5 attacks fulfilling criteria B-DB.Headache attacks lasting 4-72 h (untreated or

unsuccessfully treated)C. Headache has 2 of the following characteristics:

1. unilateral location2.pulsating quality3.moderate or severe pain intensity4.aggravation by or causing avoidance of routine

physical activity (eg, walking, climbing stairs)D.During headache 1of the following:

1. nausea and/or vomiting2.photophobia and phonophobia

E.Not attributed to another disorder

1.1 Migraine without auraNotes

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.1 Migraine without auraNotes

• If <5 attacks but criteria B-E otherwise met, code as1.6.1 Probable migraine without aura

• When attacks occur on 15 d/mo for >3 mo, code as1.1 Migraine without aura + 1.5.1 Chronic migraine

• Pulsating means varying with the heartbeat• In children:

– attacks may last 1-72 h– occipital headache requires caution

• In young children:– photophobia and/or phonophobiamay be inferred

from their behaviour

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

‘Not attributed to another disorder’

Note

For all primary headaches, this criterion means:

• History and physical/neurological examinations do not suggest any of the disorders listed in groups 5-12, or history and/or physical/ neurological examinations do suggest such disorder but it is ruled out by appropriate investigations,or such disorder is present but headache does not occur for the first time in close temporal relation to the disorder

1.2 Migraine with aura

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2 Migraine with aura

1.2.1 Typical aura with migraine headache1.2.2 Typical aura with non-migraine headache1.2.3 Typical aura without headache1.2.4 Familial hemiplegicmigraine (FHM)1.2.5 Sporadic hemiplegicmigraine1.2.6 Basilar-type migraine

1.2 Migraine with aura

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2 Migraine with aura

A.At least 2 attacks fulfilling criterion B

B.Migraine aura fulfilling criteria B and C for one of the subforms 1.2.1-1.2.6

C. Not attributed to another disorder

1.2 Migraine with auraSubtypes new to classification

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2 Migraine with auraSubtypes new to classification

1.2.1 Typical aura with migraine headache

• most migraine auras are associated with headache fulfilling criteria for 1.1 Migraine without aura

1.2.2 Typical aura with non-migraine headache

1.2.3 Typical aura without headache

• migraine aura is sometimes associated with a headache that does not fulfil these criteria

• or occurs without headache

1.2.1 Typical aurawith migraine headache

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2.1 Typical aurawith migraine headache

A.At least 2 attacks fulfilling criteria B–DB.Aura consisting of 1of the following, but no motor

weakness:1. fully reversible visual symptoms including positive

and/or negative features2.fully reversible sensory symptoms including

positive and/or negative features3.fully reversible dysphasic speech disturbance

1.2.1 Typical aurawith migraine headache

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2.1 Typical aurawith migraine headache

C. At least two of the following:1. homonymous visual symptoms and/or unilateral

sensory symptoms2. at least one aura symptom develops gradually over

5 min and/or different aura symptoms occur in succession over 5 min

3. each symptom lasts 5 and 60 minD.Headache fulfilling criteria B-D for 1.1 Migraine

without aura begins during the aura or follows aura within 60 min

E.Not attributed to another disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2.2 Typical aurawith non-migraine headache

As 1.2.1 except:D.Headache that does not fulfil criteria B-D for

1.1 Migraine without aura begins during the aura or follows aura within 60 min

1.2.3 Typical aurawithout headache

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2.3 Typical aurawithout headache

As 1.2.1 except:D.Headache does not occur during aura nor follow aura

within 60 min

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2.4 Familial hemiplegicmigraine (FHM)

A.At least 2 attacks fulfilling criteria B and CB.Aura consisting of fully reversible motor weakness

and 1of:1. fully reversible visual symptoms including positive

and/or negative features2.fully reversible sensory symptoms including

positive and/or negative features3.fully reversible dysphasic speech disturbance

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.2.6 Basilar-type migraine

As 1.2.1 except:B.Aura consisting of 2 of the following fully reversible

symptoms, but no motor weakness:1. dysarthria; 2. vertigo; 3. tinnitus; 4. hypacusia;5. diplopia; 6. visual symptoms simultaneously in bothtemporal and nasal fields of both eyes; 7. ataxia;8.decreased level of consciousness;9.simultaneously bilateral paraesthesias

C. At least one of the following:1. at least one one aura symptom develops gradually over

5 min and/or different aura symptoms occur in succession over 5 min

2.each aura symptom lasts 5 and 60 min

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.3 Childhood periodic syndromes that are commonly

precursors of migraine

1.3.1 Cyclical vomiting1.3.2 Abdominal migraine1.3.3 Benign paroxysmal vertigo of childhood

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

1.3.2 Abdominal migraine

A.At least 5 attacks fulfilling criteria B-DB.Attacks of abdominal pain lasting 1-72 h C. Abdominal pain has all of the following

characteristics:1. midline location, periumbilical or poorly localised2.dull or “just sore” quality3. moderate or severe intensity

D.During abdominal pain 2 of the following:1. anorexia; 2. nausea; 3. vomiting; 4. pallor

E.Not attributed to another disorder

Types of Migraine Treatment

• Acute– Taken during an attack– Reduces pain, associated symptoms and disability

and stops progression• Preventive

– Taken daily for months to years– Reduces frequency, severity, and duration– Used in addition to acute treatments

.

Acute Treatment Principles

• Treat attacks rapidly and consistently

• Tailor treatment to the patient and the sx

• Minimize adverse events and cost

• Limit to 3 days per week or less

Antiemetics

• Prevent and treat nausea

• Improve GI motility

• Enhance absorption of other anti-migraine medications

• Limited RCT to support their use in migraine

Phenothiazines• Promethazine (Phenergan)

– Available PO, IM, PR– Dose = 25-50 mg Q6H PRN– Blocks dopamine and histamine receptors

• Prochlorperazine (Compazine)– Available PO, IM, IV, PR– Dose = 5-10 mg Q6H PRN– Blocks dopamine receptors

• SE = sedation, dizziness, dystonic rxn

Migraine Specific Medications

TriptansErgots

Acute Treatment - Triptans

Fast onset/short duration• Sumatriptan• Rizatriptan• Zomitriptan • Almotriptan• Eletriptan• Treximet (Suma + Naproxen)

Slow onset/long duration• Naratriptan• Frovatriptan

Acute Treatment - Triptans

• Reasonable first choice for patients with moderate to severe disability from migraines

• Limit use to 2-3 days per week

• Patients who fail one triptan often respond to another

• Do not use one triptan within 24 hours of another

Acute Treatment - Triptans

Mechanism of action• 5HT-1B/1D agonists• Inhibit release of CGRP & substance P• Inhibit activation of the trigeminal nerve• Inhibit vasodilation in the meninges

Precautions• Ischemic heart dz or stroke• High risk for CAD• Pregnancy• Hemiplegic or basilar migraine• Ergots• Use w/ SSRIs?

Johnston et al Drugs 2010Loder NEJM 2010

Triptan Side Effects

• Flushing, feeling or warmth• Chest pressure or heaviness• Throat tightness• Paresthesias• Dizziness, fatigue, drowsiness• Nausea• Intolerable taste with nasal formulations

Johnston et al Drugs 2010Loder NEJM 2010

Drug Tmax (h) T1/2 (h) Metabolism

Sumatriptan 50 &100 mg tablets

2.5 2 MAO-A

Sumatriptan 20 mg nasal 1 2

Sumatriptan 6 mg subQ 0.16-0.2 2

Zolmitriptan 2.5 mg tab 2 3 2D6 and MAO-A

Zolmitriptan 2.5 mg ODT 3.3 2.5-3

Zolmitriptan 5 mg nasal 4 2.82

Rizatriptan 10 mg tab 1.2 2 MAO-A

Rizatriptan 10 mg ODT 1.6-2.5 2

Naratriptan 2-3 5-6 P450, 50% unchanged

Almotriptan 1.4-3.8 3.2-3.7 MAO-A, 3A4, and 2D6

Frovatriptan 2-4 26 Mostly unchanged

Eletriptan 1-2 3.6-5.5 3A4

Triptan Comparison

Acute Treatment – Ergots

• Mechanism of Action– Constrict peripheral and cranial blood vessels– Bind to 5HT, NE, DA, alpha and beta receptors

• Contraindications and precautions– CAD or CVD (or high risk), uncontrolled HTN– Hemiplegic or basilar migraine– Pregnancy (category X) and breast feeding– Drugs metabolized by CYP3A4, triptans

Ergot Side Effects

• Nausea and vomiting (pre-treat with antiemetic)

• Coronary artery spasm, angina, MI

• Tingling, numbness, Dizziness

• Increased BP and HR

• “Ergotism”

Choosing Acute Rx

Early N/V• Nasal triptans• Sumatriptan SubQ• ODT triptans?

Sensitive to SE• Naratriptan• Frovatriptan• Almotriptan

Recurrence• Nara, Frova, Almotriptan• Ergots• Triptan + NSAID

Rapid Onset• Sumatriptan SubQ• Nasal Triptans• DHE nasal or IM

Indications for a Preventive Agent• Migraine-related disability > 3d/month

• Migraines last over 48 hours

• Acute treatments are contraindicated, ineffective, or overused

• Migraines cause profound disability or prolonged aura

• Patient preference

Beta Blockers

• FDA approved for migraine prevention– Propranolol (Inderal) 60-240 mg PO once daily for ER or divided

BID or TID for IR– Timolol (Blocadren) 10-30 mg PO daily in 2 divided doses

• Limited evidence for migraine prevention– Nadolol (Corgard) 20-240 mg PO once daily– Atenolol (Tenormin) 50-150 mg PO daily or divided BID– Metoprolol (Lotensin, Toprol XL) 100-200 mg daily or divided

BID for IR formulation

Beta Blockers

Advantages• Thoroughly studied and widely used• Timolol (Blocadren) and propranolol (Inderal) are FDA approved• Good choice for patients with HTN, CAD, tremor, or anxiety

Disadvantages• Side effects = fatigue, dizziness, depression, exercise intolerance,

may worsen aura• Avoid in patients with severe asthma, depression, bradycardia,

Raynaud's, overt CHF

Calcium Channel Blockers

. Although the mechanism by which calcium channel antagonists affect migraine is not known,

. vasoconstriction , prevention of platelet aggregation and alterations in release and reuptake of serotonin.

. Several trials have indicated some benefit for verapamil and flunarizine In recurrent migraine.

. Verapamil in doses of 80 to 160 mg 3 times a day reduces the incidence of migraine with aura, but it is not as useful in migraine without aura.

Tricyclic Antidepressants

• Amitriptyline (Elavil) 10-200 mg nightly

• Nortriptyline (Pamelor) 10-150 mg nightly

• Desipramine (Norpramin) 25-200 mg nightly

• Imipramine (Tofranil) 10-200 mg nightly

• Doxepin (Sinequan) 10-200 mg nightly

Lower end of dosage range is usually effective for migraine prevention

Tricyclic Antidepressants

Advantages• Inexpensive• Once daily dosing• Good choice for patients with insomnia, neuropathy,

mood disorders, fibromyalgia

Disadvantages• None are FDA-approved• Side effects = sedation, weight gain, dry mouth, urinary

retention• Avoid in sz disorder, cardiac conduction abnormalities,

BPH

Other Antidepressants

• Efficacy not established in clinical trials– Best for fluoxetine (Prozac) 20 mg daily– Anectodal evidence for other SSRIs, trazodone,

mirtazapine, bupropion, venalfaxine, and duloxetine

• Migraines are more likely to be poorly controlled if mood disorders are untreated

NSAIDs• Long-acting agents taken on a scheduled basis have low risk

of causing MOH

• Consider for patients who:– Have other chronic pain conditions – Frequently use short-acting NSAID for acute treatment – Are at low risk for developing complications from daily NSAID

• Caution patients about exceeding maximum daily dose

• Limited evidence to support efficacy

NSAIDs

• Diclofenac 75 mg PO BID

• Naproxen 500 mg PO BID

• Meloxicam 7.5-15 mg PO daily

• Celecoxib 200 mg PO daily

• Aspirin 81-325 mg PO daily– May be especially helpful for reducing aura

Antiepileptic Drugs (AEDs)

• FDA approved for migraine prevention– Divalporex Sodium (Depakote)– Topiramate (Topamax)

• Limited evidence for migraine prevention– Gabapentin (Neurontin)– Lamotrigine (Lamictal)– Levetiracetam (Keppra)– Zonisamide (Zonegran)

Divalproex Sodium (Depakote)

• Increases GABA and stabilizes nerve membrane activation thresholds

• Dose = 500 - 1500 mg daily divided BID or TID– ER formulation allows for once daily dosing

• NNT = 2.8 to 4.2 (to ↓ migraine frequency 50%)

• Therapeutic plasma concentration = 50-100 mg/L

Divalproex Side Effects

Common/dose related• Tremor• Drowsiness• Nausea/vomiting• Easy bruising• Weight gain• Nystagmus

Rare/idiosyncratic• Hepatotoxicity• Pancreatitis• Alopecia• Thrombocytopenia• Agranulocytosis• Rash (SJS)• Suicidal behavior

Topiramate (Topamax) - MOA

• Not completely understood

• Blocks NMDA receptors

• Blocks voltage dependant sodium channels

• Enhances GABA

• Weakly inhibits carbonic anhydrase

.

Topiramate Dosing• Dose titration in clinical trials:

– Initial dose = 25 mg daily– Titrate by 25 mg every week– No consistent additional benefit seen in doses >100 mg

• Dose titration in U of U Headache Clinic– Initial dose = 12.5 mg at bedtime– Titrate by 12.5 mg every week– Goal of 50 mg BID– May eventually increase up to 100 mg BID in certain patients

Topiramate Side Effects

Common• Paresthesias• Cognitive problems• Fatigue• Weight loss• Dizziness• Nausea• Taste perversion

Rare or Serious• Metabolic acidosis• Depression• Nephrolithiasis• Glaucoma• Oligohydrosis• Suicidal behavior

Gabapentin (Neurontin)• Mechanism of action

– Enhances GABA activity– Binds to alpha-2-delta subunit of voltage gated calcium channels– Inhibits high-voltage-activated calcium currents– Result is decreased synaptic transmission

• Limited evidence from clinical trials for migraine prevention– NNT = 3 (50% reduction in migraine frequency)– Only 2 RCT, did not use typical migraine outcomes

Vikelis and Rapoport. CNS Drugs 2010.

Botulinum Toxin

• Recently FDA approved for chronic migraine• Dose = 155-195 units injected into muscles of

face, neck and head• Mecahnism of Action (purposed)

– Blocks release of Substance P and CGRPInhibits peripheral signals to CNS and blocks central sensitization

Dodick DW. Headache 2010.

Botulinum Toxin

• Efficacy– Botulinum Toxin superior to placebo in 2 large,

double blind, randomized, controlled trials– Botulinum Toxin similar to topiramate and

amitriptyline in small, shorter duration studies– Botulinum toxin = placebo for episodic migraine

• Side effects = muscle weakness, injection site pain, and “spread of toxin effect”

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

2. Tension-type headache

2.1 Infrequent episodic tension-type headache2.2 Frequent episodic tension-type headache2.3 Chronic tension-type headache2.4 Probable tension-type headache

Tension Type Headache• Occurs in up to 80% of the population

• Most patients treat with OTCs and do not seek medical attention

• Pathophysiology unclear– Theory of increased muscle tension is unproven

• Pain characteristics– Bandlike, bilateral– Extends form forehead to sides of temples– Involves posterior neck muscles in cape-like distribution

Acute Treatment (Episodic TTH)

• First line: OTC analgesics (APAP, NSAIDs)

• Second line: ASA+APAP+caffeine, butalbital containing products

• High risk of rebound headaches

• Limit acute treatment to 2-3 days per week

Preventive Treatment (Chronic TTH)

Non-Pharmacologic• Proper sleep hygiene• Stress management• Acupuncture• Biofeedback• Physical therapy

Pharmacologic• TCAs (best efficacy)• SSRIs (better tolerated)

**Consider for patients with >15 headaches per month**

3. Cluster headacheand other trigeminal autonomic

cephalalgias

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

3. Cluster headacheand other trigeminal autonomic

cephalalgias

3.1Cluster headache3.2Paroxysmal hemicrania3.3Short-lasting unilateral neuralgiform

headache attacks with conjunctival injection and tearing (SUNCT)

3.4Probabletrigeminal autonomic cephalalgia

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

3.1 Cluster headacheA.At least 5 attacks fulfilling criteria B-DB.Severe or very severe unilateral orbital, supraorbital

and/or temporal pain lasting 15-180 min if untreatedC. Headache is accompanied by 1of the following:

1. ipsilateral conjunctival injection and/or lacrimation2.ipsilateral nasal congestion and/or rhinorrhoea3.ipsilateral eyelid oedema4.ipsilateral forehead and facial sweating5.ipsilateral miosis and/or ptosis6.a sense of restlessness or agitation

D. Attacks have a frequency from 1/2 d to 8/dE. Not attributed to another disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

3.1 Cluster headache

3.1.1 Episodic cluster headacheA. Attacks fulfilling criteria A-E for 3.1 Cluster

headacheB. At least two cluster periods lasting 7-365 d and

separated by pain-free remission periods of 1 mo

3.1.2 Chronic cluster headacheA. Attacks fulfilling criteria A-E for 3.1 Cluster

headacheB. Attacks recur over >1 y without remission periods

or with remission periods lasting <1 mo

Cluster Headache Abortive Treatment

• Inhalation of 100% oxygen up to 15 L/min

• Sumatriptan (Imitrex) 4-6mg subQ or 20 mg nasally

• Zolmitriptan (Zomig) 5-10 mg nasally or PO

• Dihdroergotamine (Migranal) 1 mg nasally up to 3 mg in 24 hours

• Prednisone 40-100 mg burst and taper

Bajwa and Wootton. Up to Date 2007

Cluster Headache Prevention• Verapamil 120-360 mg PO daily

• Lithium 300 mg PO BID to TID

• Divalproex 500-1500 mg PO daily to BID

• Topiramate 50-200 mg PO divided BID

• Prednisone 40-100 mg burst and taper

• Melatonin 3 mg PO QPM

Bajwa and Wootton. Up to Date 2007

The Headache Diary

• Pain score

• Characteristics of the pain

• Associated symptoms

• Acute treatments used and response

• Triggers

The Headache Diary• Makes the patient responsible for their disease

• Aids in diagnosis and differentiating between headache types

• Assesses efficacy of acute and preventive treatment

• Identifies triggers

• Minimizes recall bias

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

3.2 Paroxysmal hemicraniaA.At least 20 attacks fulfilling criteria B-DB.Attacks of severe unilateral orbital, supraorbital or

temporal pain lasting 2-30 minC. Headache is accompanied by 1of the following:

1. ipsilateral conjunctival injection and/or lacrimation2.ipsilateral nasal congestion and/or rhinorrhoea3.ipsilateral eyelid oedema4.ipsilateral forehead and facial sweating5.ipsilateral miosis and/or ptosis

D.Attacks have a frequency >5/d for > half of the time, although periods with lower frequency may occur

E.Attacks are prevented completely by therapeutic doses of indomethacin

F. Not attributed to another disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

4. Other primary headaches

4.1 Primary stabbing headache4.2 Primary cough headache4.3 Primary exertional headache4.4 Primary headache associated with sexual

activity4.5 Hypnicheadache4.6 Primary thunderclap headache4.7 Hemicraniacontinua4.8 New daily-persistent headache (NDPH)

4.5 Hypnic headache New entrant to classification

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

4.5 Hypnic headacheNew entrant to classification

A.Dull headache fulfilling criteria B-DB.Develops only during sleep, and awakens patientC. At least two of the following characteristics:

1. occurs >15 times/mo2. lasts 15 min after waking3.first occurs after age of 50 y

D.No autonomic symptoms and no more than one of nausea, photophobia or phonophobia

E.Not attributed to another disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

4.6 Primary thunderclap headache

A.Severe head pain fulfilling criteria B and CB.Both of the following characteristics:

1. sudden onset, reaching maximum intensity in <1 min2.lasting from 1 h to 10 d

C. Does not recur regularly over subsequent weeks or months

D.Not attributed to another disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

4.7 Hemicrania continuaNew entrant to classification

A.Headache for >3 mo fulfilling criteria B-DB.All of the following characteristics:

1. unilateral pain without side-shift2.daily and continuous, without pain-free periods3.moderate intensity, with exacerbations of severe pain

C. At least one of the following autonomic features occurs during exacerbations, ipsilateral to the pain:1. conjunctival injection and/or lacrimation2.nasal congestion and/or rhinorrhoea3.ptosis and/or miosis

D.Complete response to therapeutic doses of indomethacinE.Not attributed to another disorder

4.8 New daily-persistent headache New entrant to classification

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

4.8 New daily-persistent headache New entrant to classification

A.Headache for >3 mo fulfilling criteria B-DB.Headache is daily and unremitting from onset or from

<3 d from onsetC. At least two of the following pain characteristics:

1. bilateral location2.pressing/ tightening (non-pulsating) quality3.mild or moderate intensity4.not aggravated by routine physical activity

D.Both of thefollowing:1. not >1 of photophobia, phonophobiaor mild nausea2.neither moderate or severe nausea nor vomiting

E.Not attributed to another disorder

Disorder Demographic Clinical Features Recommended Treatments

Chronic migraineFemale/male, 3 : 1 Prevalence 2%

Headache ≥15 days per month for >3 mo, of which ≥8 days meet ICHD-II criteria for migraine without aura or relief with triptan or ergot

Topiramate, divalproex sodium, amitriptyline

Chronic tension-type headacheEqual sex ratio Prevalence 2%Mild-moderate severity; no migrainous symptoms; bilateral, nonthrobbing

Amitriptyline

New daily persistent headache Female > male

Bilateral, persistent, moderately severe; may be preceded by viral infection; may resemble migraine or tension-type headache

Amitriptyline

Hemicrania continua Female > male

Rare; unilateral, constant, exacerbations of severe headache, cranial autonomic symptoms, and ice-pick pain; responsive to indomethacin by definition

Indomethacin

Primary Chronic Daily Headache Disorders of Long-Duration (>4 h)

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

Diagnostic criteriafor secondary headaches

A.Headache with one (or more) of the following [listed] characteristics and fulfilling criteria C and D

B.Another disorder known to be able to cause headache has been demonstrated

C. Headache occurs in close temporal relation to the other disorder and/or there is other evidence of a causal relationship

D.Headache is greatly reduced or resolves within 3 mo (shorter for some disorders) after successful treatment or spontaneous remission of the causative disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

6. Headache attributed to cranial or cervical vascular disorder

6.1 Headache attributed to ischaemicstroke or transient ischaemic attack

6.2 Headache attributed to non-traumatic intracranialhaemorrhage

6.3 Headache attributed to unruptured vascular malformation

6.4 Headache attributed to arteritis6.5 Carotid or vertebral artery pain6.6 Headache attributed to cerebral venous thrombosis 6.7 Headache attributed to other intracranial vascular

disorder

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

6.4.1 Headache attributed to giant cell arteritis

A. Any new persisting headache fulfilling criteria C and D

B.At least one of the following:1. swollen tender scalp artery with elevated

erythrocyte sedimentation rate (ESR) and/orC reactive protein (CRP)

2.temporal artery biopsy demonstrating giant cellarteritis

C. Headache develops in close temporal relation to other symptoms and signs of giant cell arteritis

D.Headache resolves or greatly improves within 3 d of high-dose steroid treatment

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

6.7.1 CADASI L

A.Attacks of migraine with aura, with or without other neurological signs

B.Typical white matter changes on MRI T2WIC. Diagnostic confirmation from skin biopsy evidence or

genetic testing (Notch 3 mutations)

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

7.4.2 Headache attributed directly to neoplasm

A.Headache with 1of the following characteristics and fulfilling criteria C and D:1. progressive2.localised3.worse in the morning4.aggravated by coughing or bending forward

B. Intracranial neoplasm shown by imagingC. Headache develops in temporal (and usually spatial)

relation to the neoplasmD.Headache resolves within 7 d after surgical removal

or volume-reduction of neoplasm or treatment with corticosteroids

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

9.1 Headache attributed tointracranial infection

9.1.1 Headache attributed to bacterial meningitis

9.1.2 Headache attributed to lymphocyticmeningitis

9.1.3 Headache attributed to encephalitis

9.1.4 Headache attributed to brain abscess

9.1.5 Headache attributed to subdural empyema

9.1.1 Headache attributed to bacterial meningitis

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

9.1.1 Headache attributed tobacterial meningitis

A.Headache with 1of the following characteristics and fulfilling criteria C and D:1. diffuse pain2.intensity increasing to severe3.associated with nausea, photophobia and/or

phonophobiaB.Evidence of bacterial meningitis from examination of CSFC. Headache develops during the meningitisD.One or other of the following:

1. headache resolves within 3 mo after relief from meningitis

2.headache persists but 3 mo have not yet passed since relief from meningitis

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

9.1.1 Headache attributed tobacterial meningitis

Notes

• Criterion D does not relate to evidence of causation• Causation is established by onset during diagnosed

bacterial meningitis, whilst it is well recognised that this headache often persists

• When this occurs, 9.4.1 Chronic post-bacterial meningitis headache is diagnosed

• Criterion D2 allows a default diagnosis within 3 mo, before it is known whether headache will resolve or persist

©International Headache Society 2003/4ICHD-I I . Cephalalgia 2004; 24 (Suppl 1)

9.2 Headache attributed to systemic infection

A.Headache with 1of the following characteristics and fulfilling criteria C and D:1. diffusepain2.intensity increasingto moderate or severe3.associated with fever, general malaise or other

symptoms of systemic infectionB.Evidence of systemic infectionC. Headache develops during the systemic infectionD.Headache resolves within 72 h after effective

treatment of the infection

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