INTERNATIONAL CLASSIFICATION

of
HEADACHE DISORDERS
2nd edition

(ICHD-II)

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

Headache:
Headache
a clinical tour
for residents

April 2017
Nur Amaliah Verbty MD
Headache is the Most Common Symptom that
Humans Experience
 Primary headaches (No underlying cause)
Migraine
Tension-type
Cluster
Other
Secondary headaches (Underlying cause)
Medication overuse
Head/neck injury
Space-occupying lesion (i.e. brain tumour)
Vascular cause (i.e. Subarachnoid hemorrhage, intracranial
bleed)
Infectious cause (i.e. meningitis or upper respiratory tract
infection)
>65% in
+ many others
patients
older
than 50
Headache Classification Committee of the International Headache Society,1988
 Any secondary
Headache disorder
can mimic a primary
headache disorder

Wolff HG, et al., 2001

  1876 consec pts (38yo); 2/3 non acute:1.2%
◦ 99% had normal neuro exam
◦ Pituitary adenoma, arachnoid cyct, meningioma,
hydrocephalus, chiari I, stroke, cavernoma, glioma..
◦ Half treated surgically
◦ Only one lesion not appreciated on CT

 US headache consortium meta-analysis of

patient with migraine and normal exam:
0.18% rate of significant pathology therefore
imaging not indicated.

Cephalalgia 2005;25:30-35. Neurology 1994;44:1353-54

 Chronic Migraine
New Daily
+/- medication Persistent Headache
overuse (NDPH)

Chronic
Daily Headache
(CDH)

Chronic Hemicrania
Tension Continua
Type
Headache
Other

Headache Classification Subcommittee of the International Headache Society, 2004

What are the Secondary Causes of Chronic
Headaches?
 Cervicogenic
Medication Overuse
headache
Headache (MOH)
Post-traumatic
headache
Chronic
Daily Headache
Sleep apnea Decreased ICP
(CDH)
(spontaneous intracranial
hypotension)

Increased ICP
Other (tumour/mass,
pseudotumour cerebri,
hydrocephalus)
What are the Headache “Red Flags”?
 Systemic symptoms (fever, weight loss)
 Secondary risk factors (cancer,
HIV/immunocompromised)
 Neurologic symptoms or abnormal signs
 Onset (i.e. new-onset chronic headache)
 Older patient (i.e. new headaches at age >50 yrs)
 Previous headache different (i.e. significant
change in headache frequency or clinical
features)
 Positional component (i.e. increases when
upright)
 Provocative factors (precipitated by coughing,
exercise, sex)
  Vitals (particularly BP)
 Pupil symmetry, reactivity and fundoscopy
 Visual fields
 Eye movements
 Motor – look for asymmetrical weakness R vs L
 Reflexes – look for asymmetry (increased
reflexes) R vs L
 Sensation – extinction to double simultaneous
tactile stimuli
 Coordination – finger-nose-finger, gain and
tandem gait
 Examine/touch the head and neck

Olesen J , et al., 2006

Pryse –Phyllips WEM, et al., 1997
 Part 2:
The secondary headaches
5. Headache attributed to head and/or neck trauma
6. Headache attributed to cranial or cervical vascular
disorder Part 2:
7. Headache attributed to non-vascular intracranial
The secondary headaches
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 structures
12. Headache attributed to psychiatric disorder
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
 Primary or secondary
headache?
Primary:
• no other causative or
Primary disorder
secondary
Secondary headache?
(ie, caused by another disorder):
• new headache occurring in close temporal
relation to another disorder that is a known
cause of headache
• coded as attributed to that disorder

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

Diagnosis Primary Primary and
Headache only Secondary
Temporal relation of Loose Close
the other disorder to
headache worsening

Degree of worsening Slight Marked

Evidence disorder Weak Strong

causes secondary
headaches
Other disorder Headache Headache
eliminated
unchanged improves
The Headaches, 3rd edition. Lippincott Williams and Wilkins 2006
 5. Headache attributed to
head and/or neck trauma
5.1 Acute post-traumatic headache
5. Headache
5.2 Chronic post-traumaticattributed
headache to
5.3 Acute headache attributed to whiplash injury
head and/or neck trauma
5.4 Chronic headache attributed to whiplash injury
5.5 Headache attributed to traumatic intracranial
haematoma
5.6 Headache attributed to other head and/or neck
trauma
5.7 Post-craniotomy headache

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

 Secondary headache after injury
 Should begin within 7 days of head
injury (to meet IHS criteria)
 Headaches may resemble primary
headache disorders (i.e. migraine,
tension)
 Often assoc with other “post-
concussive” symptoms: vertigo,
tinnitus, cognitive changes, sleep
problems, depression, medication
overuse
 There is no evidence-based
approach and no guidelines
6. Headache attributed to cranial
or cervical vascular disorder
6.1 Headache attributed to ischaemic stroke or
6. Headache attributed to
transient ischaemic attack Up to 1/3 pts with stroke
6.2 Headache attributed to non-traumatic intracranial
cranial or cervical vascular
haemorrhage
6.3 Headache attributed to unruptured vascular
malformation
disorder
6.4 Headache attributed to arteritis
6.5 Carotid or vertebral artery pain
6.6 Headache attributed to cerebral venous thrombosis
6.7 Headache attributed to other intracranial vascular
disorder
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
-anterior communicating artery
(30-35%),
-the bifurcation of the internal
carotid and posterior
communicating artery (30-35%) -
-the bifurcation of the middle
cerebral artery (20%)
-bifurcation of the basilar artery,
and the remaining posterior
circulation arteries (5%)
 Utility of CT and LP in SAH
• CT: 90-95% sensitivity within 24h
• 80% at 3 days, 50% at 1 week.

• MRI FLAIR (3-14 d more sensitive than CT)

• LP may be negative less than 2 hours after the bleed;

– most sensitive at 12 hours after symptom onset.
– Xanthochromia (yellow-to-pink CSF supernatant)
usually is seen by 12 hours
 Symptoms
• Headache 85-95%
• Neck stiffness 74-84%
• n/v, photophobia 48%
• Mental status 43%

• Less common:
– Focal deficit, seizures, coma, CN palsy, papilledema, ocular
hemorrhage

*sentinel bleed
 First primary sexual or exertional
headache

• SAH has to be excluded as 1/3 of SAHs

occur during activities such as bending,
lifting, defecation or sexual intercourse.
 6.6 Cerebral Venous Sinus thrombosis
5 patterns of presentation
1. Isolated Intracranial hypertension
1. Progressive onset over days to weeks
2. Papilledema
3. CN6 palsy, tinnitus, visual obscurations
2. Venous infarction with focal signs
1. Mimics arterial stroke
2. Chronic and progressive like a tumor or abscess
3. Subacute encephalopathy
1. Diffuse HA with decr LOC
2. Focal signs
3. Seizures
4. +/-papilledema
4. Cavernous sinus thrombosis
1. Frontal headache
2. Chemosis, proptosis, opthalmoparesis
5. Thunderclap headache mimics SAH
Secondary causes of CVST
• Hypercoagable state
– Factor V Leiden, Protein C & S,ATIII
deficiency, prothrombin gene
mutation,etc
• Cancer
• Sepsis, dehydration, infections
• Behcet’s
• SLE
• Estrogen – pregnancy, puerperium
 6.4 Headache attributed to
arteritis

6.4.1
6.4Headache attributedattributed
Headache to giant cell arteritis
to
(GCA)
arteritis
6.4.2 Headache attributed to primary central
nervous system (CNS) angiitis
6.4.3 Headache attributed to secondary central
nervous system (CNS) angiitis

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

 Arteritis Headache
• Cerebral vasculitis
– Primary angiitis of the CNS
– AIDS, zoster
– Fungal, viral, parasitic, treponemal meningitis
– Drug related - amphetamines, cocaine,
– Lymphoma (angioendotheliosis)
• Systemic vasculitis
– Behcet’s - genital ulcers, arthritis, eye
– PAN - fever, arthralgias, myalgias, mononeuropathies
– Churg-Strauss - asthma, eosinophilia, neuropathy
– Wegener’s – lower/upper resp, neuropathy
– SLE - fever, rash, arthritis, pleuritis, encephalopathy
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:
6.4.1 Headache attributed to
1. swollen tender scalp artery with elevated
erythrocyte sedimentation rate (ESR) and/or
giant cell arteritis
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

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

6.7 Headache attributed to other
intracranial vascular disorder

6.7
6.7.1 Headache
Cerebral attributed to
Autosomal Dominant
Arteriopathy with Subcortical Infarcts and
other intracranial
Leukoencephalopathy vascular
(CADASIL)
disorder
6.7.2 Mitochondrial Encephalopathy, Lactic
Acidosis and Stroke-like episodes (MELAS)
6.7.3 Headache attributed to benign angiopathy
of the central nervous system
6.7.4 Headache attributed to pituitary apoplexy

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

 7.
7. Headache
Headache attributed
attributed to
to
non-vascular
non-vascular intracranial
intracranial disorder
disorder
7.1
7.1 Headache
Headache attributed
attributed to
to high
high cerebrospinal
cerebrospinal fluid
fluid pressure
pressure
7.2 7. Headache attributed to
7.2 Headache
Headache attributed
attributed to
to low
low cerebrospinal
cerebrospinal fluid
fluid pressure
pressure
7.3
7.3 Headache
Headache attributed
attributed to
to non-infectious
non-infectious inflammatory
inflammatory
non-vascular intracranial
disease
disease
7.4
7.4 Headache
Headache attributed
attributed to
to intracranial
intracranial neoplasm
neoplasm
7.5
7.5 Headache
disorder
Headache attributed
attributed to
to intrathecal
intrathecal injection
injection
7.6
7.6 Headache
Headache attributed
attributed to
to epileptic
epileptic seizure
seizure
7.7 Headache attributed to Chiari malformation type I
7.8 Syndrome of transient Headache and Neurological
Deficits with cerebrospinal fluid Lymphocytosis (HaNDL)
7.9 Headache attributed to other non-vascular intracranial
disorder
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
7.1.1 Headache attributed to IIH

A. Progressive headache with 1 of the following

characteristics and fulfilling criteria C and D:
7.1.1 Headache attributed to
1. daily occurrence
2. diffuse and/or constant (non-pulsating) pain
IIH
3. aggravated by coughing or straining
B. Intracranial hypertension (criteria on next slide)
C. Headache develops in close temporal relation to
increased intracranial pressure
D. Headache improves after withdrawal of CSF to reduce
pressure to 120-170 mm H2O and resolves within 72 h
of persistent normalisation of intracranial pressure

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

7.1.1 Headache attributed to IIH
A. Diffuse constant headache worsened by coughing or straining
B. Intracranial hypertension fulfilling the following criteria:
1. alert patient with neurological examination that either is normal or
7.1.1 Headache attributed to
demonstrates any of the following abnormalities:
a) papilloedema
IIH
b) enlarged blind spot
c) visual field defect (progressive if untreated)
d) sixth nerve palsy
2. increased CSF pressure (>200 mm H2O [non-obese], >250 mm
H2O [obese]) measured by lumbar puncture in the recumbent
position or by epidural or intraventricular pressure monitoring
3. normal CSF chemistry (low CSF protein acceptable) and cellularity
4. intracranial diseases (including venous sinus thrombosis) ruled out
by appropriate investigations
5. no metabolic, toxic or hormonal cause of intracranial hypertension

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

 Pathophysiology
• Disorder of homeostasis where there may
be:
– Increased brain water content
– Incr. cerebral blood volume
– Incr CSF production
– Venous outflow obstruction (increased
resistance to CSF outflow)
 Secondary causes
Disruption of outflow Hormonal
-venous thrombosis, dural fistula -hypoparathyroidism
-radical neck dissection
-hyper/hypothyroidism
-right heart failure
-Cushing's ?Addison's
-COPD
-PCO
-(obesity)

Toxins/meds
-vitamin A
-Nalidixic acid,tetracycline,nitrofurantoin,indocid,steroids/withdrawal,others

Medical conditions
CRF, SLE, Anemia/polycythemia

Infectious-
meningitis,encephalitis,Lyme,HIV

Trauma
 7.2 Headache attributed to
low cerebrospinal fluid pressure

7.2 Headache attributed to

7.2.1 Post-dural puncture headache
low cerebrospinal fluid
7.2.2 CSF fistula headache
pressure
7.2.3 Headache attributed to spontaneous (or
idiopathic) low CSF pressure

(CSF below 5-9 cm H2O)

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

7.2.1 Post-dural (post-lumbar)
puncture headache
A. Headache that worsens within 15 min after sitting or
standing and improves within 15 min after lying, with
7.2.1 Post-dural (post-lumbar)
1 of the following and fulfilling criteria C and D:
1. neck stiffness; 2. tinnitus; 3. hypacusia;
puncture headache
4. photophobia; 5. nausea
B. Dural puncture has been performed
C. Headache develops within 5 d after dural puncture
D. Headache resolves either:
1. spontaneously within 1 wk
2. within 48 h after effective treatment of the spinal
fluid leak
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
 8.1.1 – NO donor: NTG, nitrates, nitrites
 8.1.2 – Phosphodiesterase inhibitor: Viagra,
etc – migraineurs most at risk; lower doses
better.
 10.1 Headache attributed to
hypoxia and/or hypercapnia

10.1 Headache attributed to

10.1.1 High-altitude headache
hypoxia and/or
10.1.2 Diving headache
hypercapnia
10.1.3 Sleep apnoea headache

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

 10.3 Headache attributed to
arterial hypertension
10.3.1 Headache attributed to phaeochromocytoma
10.3.2 Headache
10.3 attributed to
Headache hypertensive crisis
attributed to
without hypertensive encephalopathy
arterial
10.3.3 Headache hypertension
attributed to hypertensive
encephalopathy
10.3.4 Headache attributed to pre-eclampsia
10.3.5 Headache attributed to eclampsia
10.3.6 Headache attributed to acute pressor
response to an exogenous agent
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
 11. Headache or facial pain attributed to
disorder of cranium, neck, eyes, ears, nose,
sinuses, teeth, mouth or other facial or
cranial structures
11. Headache or facial pain
attributed
11.1 Headache to disorder
attributed of cranium,
to disorder of cranial bone
11.2 Headache attributed
neck, eyes, to disorder
ears, nose, of neck
sinuses,
11.3 Headache attributed to disorder of eyes
11.4teeth, mouth
Headache or other
attributed facialofor
to disorder cranial
ears
structures
11.5 Headache attributed to rhinosinusitis
11.6 Headache attributed to disorder of teeth, jaws or
related structures
11.7 Headache or facial pain attributed to
temporomandibular joint (TMJ) disorder
11.8 Headache attributed to other disorder of the above
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
 Part 3:
Cranial neuralgias, central
and primary facial
Part 3: pain and
other headaches
Cranial neuralgias, central and
primary facial pain and other
13. Cranial neuralgias and central causes of
facial pain headaches
14. Other headache, cranial neuralgia, central or
primary facial pain

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

 13. Cranial neuralgias and
central causes of facial pain
13.1 Trigeminal neuralgia
13.2 Glossopharyngeal neuralgia
13.313. Cranial
Nervus neuralgias
intermedius neuralgia and
13.4 Superior laryngeal neuralgia
central
13.5 causes
Nasociliary neuralgiaof facial pain
13.6 Supraorbital neuralgia
13.7 Other terminal branch neuralgias
13.8 Occipital neuralgia
13.9 Neck-tongue syndrome
13.10 External compression headache
13.11 Cold-stimulus headache
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
 13. Cranial neuralgias and
central causes of facial pain
13.12 Constant pain caused by compression,
irritation or distortion of cranial nerves or upper
13.roots
cervical Cranial neuralgias
by structural lesions and
13.13 Optic neuritis
13.14central causes
Ocular diabetic of facial pain
neuropathy
13.15 Head or facial pain attributed to herpes zoster
13.16 Tolosa-Hunt syndrome
13.17 Ophthalmoplegic ‘migraine’
13.18 Central causes of facial pain
13.19 Other cranial neuralgia or other centrally
mediated facial pain
ICHD-II. Cephalalgia 2004; 24 (Suppl 1) ©International Headache Society 2003/4
 14.2 Headache unspecified

A. Headache is or has been present

14.2 Headache unspecified
B. Not enough information is available to classify the
headache at any level of this classification
WE don’t need to use this code most of the time!

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