DEPARTMENT OF FAMILY

MEDICINE FPE
HEADACHE SYNDROME
-VANAVEERA PANDIAN SWETHA
GROUP 415 B
SUBGROUP”15”
 OUTLINE OF THE
PRESENTATION
 1. Name of the syndrome.
 2. Key facts and milestones regarding the syndrome (frequency of
occurrence, clinical features).
 3. The most likely causes of the syndrome (presumptive diagnosis).
 4. The most dangerous diseases that are manifested by this syndrome.
 5. “Red flags” of danger (clinical signs of a threat to life).
 6. Diagnostic approach to the syndrome and diseases in which this
syndrome occurs (history, physical examination data, laboratory and
instrumental examination).
 7. Tactics of a general practitioner.
 8. The used literature.
 HEADACHE SYNDROMES

 Primary headache syndromes are divided into 4 groups:

migraine, tension-type, trigeminal autonomic cephalalgias
and other. Other is defined as headaches not of the other 3
groups that do not have a secondary cause. All headaches can be
infrequent (episodic) or may become chronic. Chronic headache
refers to a headache that occurs on 15 days or more a month. In
the case of cluster headache, the most common of the trigeminal
autonomic cephalalgias, chronic is defined as the absence of
headache for less than 1 week a month for more than 6 months.
 Primary headaches disorders are not associated with any
demonstrable structural abnormality of the brain. The diagnosis
of the headache type is based on patient history, headache
characteristics, and a normal neurological exam. Laboratory and
imaging test results are normal, so in general, expensive studies
like imaging are not obtained. During the headache attack,
however, patients with cluster and migraine headache may have
some abnormal clinical findings. Primary headache disorders
typically occur early in life with a decreased incidence of new
primary headache disorders after the age of 40 to 50.
 Secondary headaches are usually of recent onset and associated
with abnormalities found on clinical examination. Laboratory
testing, imaging studies, or both confirm the diagnosis.
Recognizing headaches related to an underlying condition or
disease is critical not only because treatment of the underlying
problem usually eliminates the headache, but because the
condition causing the headache may be life-threatening. 
 Origins of Pain in the Head
• Extra-cranial pain • Intra-cranial pain
sensitive structures: sensitive structures:
– Sinuses – Arteries of circle of willis
– Eyes/orbits and proximal dural
– Ears arteries,
– Teeth – Dural Venous
– TMJ sinuses,veins
– Blood vessels – Meninges
– 5,7,9,10 cranial nerves – Dura
carry pain from
different structures
 Classification of Headaches
• PRIMARY - NO structural • SECONDARY – structural
or metabolic abnormality: or metabolic abnormality:
– Extracranial: sinusitis, otitis
– Tension media, glaucoma, TMJ ds
– Migraine – Inracranial:
SAH, vasculitis, dissection, ce
– Cluster ntral 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
 Part 1:
The primary headaches

1. Migraine
2. Tension-type headache
3. Cluster headache
and other trigeminal autonomic cephalalgias
4. Other primary headaches
 Primary Headache Types
Migraine Tension Cluster
Pain Throbbing, mod Pressure, t Abrupt onset,
Description erate to ightness, deep,
severe, worse waxes and continuous,
w/exertion wanes excruciating,
explosive
Associated Photo/phono- None Tearing,
phobia, n/v, aura congestion,
Symptoms rhinorrhea,
pallor, sweating
 Primary Headache Types
Migraine Tension Cluster
Location 60-70% Bilateral Unilateral
unilateral
Duration 4-72 hr Variable 0.5-3 hr,
many per day
Patient Resting in Remains Remains
Appearance quiet dark active or active, prefers
room; young prefers to hot shower,
female rest male, smoker
 1. Migraine

1. Migraine without aura

2. Migraine with aura
3. Childhood periodic syndromes that are
commonly precursors of migraine
4. Retinal migraine
5. Complications of migraine
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
 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 Activation of Activation of Activation of

Meningeal blood Area Postrema Hypothalamus cervical trigeminal
vessels (N/V) (Hypersensitivity) system (Muscle
(Throbbing) spasm)
Activation of
Cortex and
Thalamus (Head
pain)
 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 attributed to another disorder
©International eadache Society 2003/4
 1.1 Migraine without aura
Notes
• If <5 attacks but criteria B-E otherwise met, code as
1.6.1 Probable migraine without aura
.1 Migraine without aura
• When attacks occur on 15 d/mo for >3 mo, code
Notes as
1.1 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 phonophobia may be inferred
from their behaviour
 ‘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

1. Typical aura with migraine headache

2. 1.2 Migraine
Typical with aura
aura with non-migraine headache
3. Typical aura without headache
4. Familial hemiplegic migraine (FHM)
5. Sporadic hemiplegic migraine
6. Basilar-type migraine
 1.2 Migraine with aura

A. At least 2 attacks fulfilling criterion B

1.2 Migraine with aura
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 aura
Subtypes new to classification

1.2.1 Typical aura with migraine headache

•
1.2 Migraine with aura
most migraine auras are associated with headache
Subtypes
fulfilling criteria for new to classification
1.1 Migraine without aura
2. Typical aura with non-migraine
headache
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 aura with
migraine headache

A. At least 2 attacks fulfilling criteria B–D

1.2.1 Typical aura
B. Aura consisting of 1 of the following, but no
motor weakness:
with migraine headache
1. fully reversible visual symptoms including positive
and/or negative features
2. fully reversible sensory symptoms including
positive and/or negative features
3. fully reversible dysphasic speech
disturbance
 1.2.1 Typical aura with
migraine headache
C. At least two of the following:
1. homonymous visual symptoms and/or unilateral
1.2.1 Typical aura
sensory symptoms
2. at least one aura symptom develops gradually
with migraine headache
over 5 min and/or different aura symptoms occur
in succession over 5 min
3. each symptom lasts 5 and 60 min
D. 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
 1.2.2 Typical aura
with 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 aura without
headache

1.2.3 Typical aura

As 1.2.1 except:
without headache
D. Headache does not occur during aura nor follow
aura within 60 min
 1.2.4 Familial hemiplegic
migraine (FHM)
A. At least 2 attacks fulfilling criteria B and C
B. Aura consisting of fully reversible motor weakness
and 1 of:
1. fully reversible visual symptoms including positive
and/or negative features
2. fully reversible sensory symptoms including
positive and/or negative features
3. fully reversible dysphasic speech
disturbance
 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 both
temporal and nasal fields of both eyes; 7. ataxia;
8. decreased level of consciousness;
9. simultaneously bilateral paraesthesias
C. At least one of the following:
10.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
1.3 Childhood periodic syndromes that are
commonly
precursors of migraine

1. Cyclical vomiting
2. Abdominal migraine
3. Benign paroxysmal vertigo of childhood
 1.3.2 Abdominal migraine

A. At least 5 attacks fulfilling criteria B-D

B. Attacks of abdominal pain lasting 1-72 h
C. Abdominal pain has all of the following
characteristics:
1. midline location, periumbilical or
poorly localised
2. dull or “just sore” quality
3. moderate or severe intensity
D. During abdominal pain 2 of the
following:
1. anorexia; 2. nausea; 3. vomiting; 4.
pallor
The SNOOP mnemonic is useful to identify secondary headache:

S: Systemic signs and symptoms

Fever, chills, weight loss, history of HIV or malignancy

N: Neurological signs and symptoms

Primary headache disorders have a normal neurological exam.

O: Onset
First and the worst headache of life. Headache that reaches pick intensity within seconds
to minutes.

O: Older age
New onset of headache in someone after the age of 40. In general, primary headache
disorders begin in young people.

P: Progression of an existing headache disorder

Change in location, quality, or frequency of the headache. The most common cause of
this is medication overuse.
 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

Triptans Ergots
 Acute Treatment - Triptans
Fast onset/short duration Slow onset/long duration
• Sumatriptan • Naratriptan
• Rizatriptan • Frovatriptan
• Zomitriptan
• Almotriptan
• Eletriptan
• Treximet (Suma +
Naproxen)
 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 Precautions
• 5HT-1B/1D agonists • Ischemic heart dz or
• Inhibit release of CGRP & stroke
substance P • High risk for CAD
• Inhibit activation of the
• Pregnancy
trigeminal nerve
• Inhibit vasodilation in • Hemiplegic or basilar
the meninges migraine
• Ergots
• Use w/ SSRIs?
 Triptan Side Effects
• Flushing, feeling or warmth
• Chest pressure or heaviness
• Throat tightness
• Paresthesias
• Dizziness, fatigue, drowsiness
• Nausea
• Intolerable taste with nasal formulations
 Triptan Comparison
Drug Tmax (h) T1/2 (h) Metabolism
Sumatriptan 50 &100 mg 2.5 2 MAO-A
tablets
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
 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 Recurrence
• Nasal triptans • Nara, Frova, Almotriptan
• Sumatriptan SubQ • Ergots
• ODT triptans? • Triptan + NSAID
Sensitive to SE Rapid Onset
• Naratriptan • Sumatriptan SubQ
• Frovatriptan • Nasal Triptans
• Almotriptan • 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 Disadvantages
• Thoroughly studied and • Side effects =
widely used fatigue, dizziness, depression
• Timolol (Blocadren) and , exercise intolerance, may
propranolol (Inderal) are FDA worsen aura
approved • Avoid in patients with severe
• Good choice for patients asthma, depression, bradycar
with HTN, CAD, tremor, or dia, Raynaud's, overt CHF
anxiety
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 Disadvantages
• Inexpensive • None are FDA-approved
• Once daily dosing • Side effects = sedation,
• Good choice for patients weight gain, dry mouth,
with urinary retention
insomnia, neuropathy, m • Avoid in sz disorder,
ood cardiac conduction
disorders, fibromyalgia 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 Rare/idiosyncratic
• Tremor • Hepatotoxicity
• Drowsiness • Pancreatitis
• Nausea/vomiting • Alopecia
• Easy bruising • Thrombocytopenia
• Weight gain • Agranulocytosis
• Nystagmus • 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 Rare or Serious

• Paresthesias • Metabolic acidosis
• Cognitive problems • Depression
• Fatigue • Nephrolithiasis
• Weight loss • Glaucoma
• Dizziness • Oligohydrosis
• Nausea • Suicidal behavior
• Taste perversion
 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
 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 CGRP
Inhibits peripheral signals to CNS and blocks
central sensitization
 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”
 2. Tension-type headache

1. Infrequent episodic tension-type headache

2. Frequent episodic tension-type headache
3. Chronic tension-type headache
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
Pharmacologic
• Proper sleep hygiene
• TCAs (best efficacy)
• Stress management
• SSRIs (better tolerated)
• Acupuncture
• Biofeedback
• Physical therapy

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

 3. Cluster headache
and other trigeminal autonomic
cephalalgias

1. Cluster headache
2. Paroxysmal hemicrania
3. Short-lasting unilateral neuralgiform
headache attacks with conjunctival injection
and tearing (SUNCT)
3.4 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 if untreated
C. Headache is accompanied by 1 of the following:
1. ipsilateral conjunctival injection and/or
lacrimation
2. ipsilateral nasal congestion and/or rhinorrhoea
3. ipsilateral eyelid oedema
4. ipsilateral forehead and facial sweating
5. ipsilateral miosis and/or ptosis
6. a sense of restlessness or agitation
D. Attacks have a frequency from 1/2 d to 8/d
E. Not attributed to another disorder
 3.1 Cluster headache
1. Episodic cluster headache
A.Attacks fulfilling criteria A-E for 3.1 Cluster
headache
B.At least two cluster periods lasting 7-365 d and
separated by pain-free remission periods of
1 mo

2. Chronic cluster headache

A.Attacks fulfilling criteria A-E for 3.1 Cluster
headache
B.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
 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
 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
 3.2 Paroxysmal hemicrania
A. At least 20 attacks fulfilling criteria B-D
B. Attacks of severe unilateral orbital, supraorbital or
temporal pain lasting 2-30 min
C. Headache is accompanied by 1 of the
following:
1. ipsilateral conjunctival injection and/or
lacrimation
2. ipsilateral nasal congestion and/or rhinorrhoea
3. ipsilateral eyelid oedema
4. ipsilateral forehead and facial sweating
5. 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
 4. Other primary headaches

1. Primary stabbing headache

2. Primary cough headache
3. Primary exertional headache
4. Primary headache associated with sexual
activity
5. Hypnic headache
6. Primary thunderclap headache
7. Hemicrania continua
8. New daily-persistent headache (NDPH)
 4.5 Hypnic headache
New entrant to classification

A. Dull headache fulfilling criteria B-D

4.5 Hypnic headache
B. Develops only during sleep, and awakens patient
C. At least two of
Newtheentrant
following characteristics:
to classification
1. occurs >15 times/mo
2. lasts 15 min after waking
3. 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
 4.6 Primary thunderclap headache

A. Severe head pain fulfilling criteria B and C

B. Both of the following characteristics:
1. sudden onset, reaching maximum intensity in <1 min
2. lasting from 1 h to 10 d
C. Does not recur regularly over subsequent weeks or
months
D. Not attributed to another disorder
 4.7 Hemicrania continua
New entrant to classification
A. Headache for >3 mo fulfilling criteria B-D
B. All of the following characteristics:
1. unilateral pain without side-shift
2. daily and continuous, without pain-free periods
3. 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 lacrimation
2. nasal congestion and/or rhinorrhoea
3. ptosis and/or miosis
D. Complete response to therapeutic doses of
indomethacin
E. Not attributed to another disorder
4.8 New daily-persistent headache
New entrant to classification
A. Headache for >3 mo fulfilling criteria B-D
B. Headache is daily and unremitting from onset or from
<3d from onset
ew daily-persistent heada
C. At least two of the following pain characteristics:
New entrant to classification
1. bilateral location
2. pressing/tightening (non-pulsating) quality
3. mild or moderate intensity
4. not aggravated by routine physical activity
D. Both of the following:
1. not >1 of photophobia, phonophobia or mild nausea
2. neither moderate or severe nausea nor vomiting
E. Not attributed to another disorder
Primary Chronic Daily Headache Disorders of Long-Duration (>4 h)

Disorder Demographic Clinical Features Recommended Treatments

Headache ≥15 days per month

for >3 mo, of which ≥8 days
Female/male, 3 : 1 Prevalence Topiramate, divalproex sodium,
Chronic migraine meet ICHD-II criteria for
2% amitriptyline
migraine without aura or relief
with triptan or ergot

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

Bilateral, persistent, moderately

severe; may be preceded by
New daily persistent headache Female > male viral infection; may resemble Amitriptyline
migraine or tension-type
headache

Rare; unilateral, constant,

exacerbations of severe
headache, cranial autonomic
Hemicrania continua Female > male symptoms, and ice-pick pain; Indomethacin
responsive to indomethacin by
definition
 Diagnostic criteria for
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
6. Headache attributed to cranial or cervical
vascular disorder
1. Headache attributed to ischaemic stroke or
transient ischaemic attack
2. Headache attributed to non-traumatic intracranial
haemorrhage
3. Headache attributed to unruptured vascular
malformation
4. Headache attributed to arteritis
5. Carotid or vertebral artery pain
6. Headache attributed to cerebral venous
thrombosis
7. Headache attributed to other intracranial vascular
disorder
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/or
C reactive protein (CRP)
2. temporal artery biopsy demonstrating giant cell
arteritis
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
 6.7.1 CADASIL

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

neurological signs
B. Typical white matter changes on MRI T2WI
C. Diagnostic confirmation from skin biopsy evidence or
genetic testing (Notch 3 mutations)
 7.4.2 Headache attributed directly to
neoplasm
A. Headache with 1 of the following characteristics
and fulfilling criteria C and D:
1. progressive
2. localised
3. worse in the morning
4. aggravated by coughing or bending forward
B. Intracranial neoplasm shown by imaging
C. Headache develops in temporal (and usually spatial)
relation to the neoplasm
D. Headache resolves within 7 d after surgical removal
or volume-reduction of neoplasm or treatment with
corticosteroids
 9.1 Headache attributed to
intracranial infection

1. Headache attributed to bacterial meningitis

2. Headache attributed to lymphocytic
meningitis
3. Headache attributed to encephalitis
4. Headache attributed to brain abscess
5. Headache attributed to subdural empyema
 9.1.1 Headache attributed to bacterial
meningitis
A. Headache with 1 of the following characteristics and
fulfilling criteria C and D:
.1.1 Headache attributed t
1. Diffuse pain
2. intensity increasing to severe
bacterial meningitis
3. associated with nausea, photophobia and/or
phonophobia
B. Evidence of bacterial meningitis from examination of CSF
C. Headache develops during the meningitis
D. 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
 9.1.1 Headache attributed to
bacterial 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
 9.2 Headache attributed to systemic
infection
A. Headache with 1 of the following characteristics
and fulfilling criteria C and D:
1. diffuse pain
2. intensity increasing to moderate or severe
3. associated with fever, general malaise or other
symptoms of systemic infection
B. Evidence of systemic infection
C. Headache develops during the systemic infection
D. Headache resolves within 72 h after effective
treatment of the infection

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

 USED LITERATURES:

• ICHD-II. Cephalalgia 2004; 24 (Suppl 1)

• ©International Headache Society 2003/4
• Bajwa and Wootton. Up to Date 2007
• Marcus, DA. Headache Simplified 2008.