Views & Reviews

CME

Classification of primary headaches

R.B. Lipton, MD; M.E. Bigal, MD, PhD; T.J. Steiner, MB, PhD, LLM;
S.D. Silberstein, MD; and J. Olesen, MD, PhD

AbstractGiven the range of disorders that produce headache, a systematic approach to classification and diagnosis is
an essential prelude to clinical management. For the last 15 years, the diagnostic criteria of the International Headache
Society (IHS) have been the accepted standard. The second edition of The International Classification of Headache
Disorders (January 2004) reflects our improved understanding of some disorders and the identification of new disorders.
Neurologists who treat headache should become familiar with the revised criteria. Like its predecessor, the second edition
of the IHS classification separates headache into primary and secondary disorders. The four categories of primary
headaches include migraine, tension-type headache, cluster headache and other trigeminal autonomic cephalalgias, and
other primary headaches. There are eight categories of secondary headache. Important changes in the second edition
include a restructuring of these criteria for migraine, a new subclassification of tension-type headache, introduction of the
concept of trigeminal autonomic cephalalgias, and addition of previously unclassified primary headaches. Several disorders were eliminated or reclassified. In this article, the authors present an overview of the revised IHS classification,
highlighting the primary headache disorders and their diagnostic criteria. They conclude by presenting an approach to
headache diagnosis based upon these criteria.
NEUROLOGY 2004;63:427435

Headache is one of the most common reasons for

neurologic consultation.1,2 Given the range of disorders that present with headache, a systematic approach to headache classification and diagnosis is
essential both to good clinical management and to
useful research. For the last 15 years, the 1988 classification of the International Headache Society
(IHS)3 has been the accepted standard for headache
diagnosis, establishing both uniform terminology
and consistent operational diagnostic criteria for the
entire range of headache disorders. Translation into
22 languages has facilitated clinical practice, epidemiologic studies, and multinational clinical trials.
The criteria provide the basis for clinical trials
guidelines for several conditions.4 With the publication of the second edition of The International Classification of Headache Disorders (ICHD-2)5 in
January 2004, neurologists who treat headache
should become familiar with the revised criteria,
which will become the standard for treatment and
research. Although the basic structure and most of
the original categories are preserved, there were
many changes that will influence neurologic practice.
These changes include a restructuring of the criteria
for migraine, new subclassification of tension-type
headache (TTH), introduction of the concept of tri-

geminal autonomic cephalalgias (TACs), and addition of several previously unclassified types of
primary headache. The revised classification was developed by a panel of experts in the field chosen by
the IHS. Although we attempted to review the relevant literature, the classification represents a clinical consensus based on data, experience, judgment,
and compromise. Nonetheless, the classification provides a foundation for clinical practice and research,
and will eventually itself be revised.
In this article we present an overview of the
ICHD-2,5 highlighting the primary headache disorders and their diagnostic criteria. We then offer an
algorithmic approach to primary headache diagnosis
based on attack frequency and duration, using the
ICHD-2.
The ICHD-2an overview. Like the 1988 classification, The International Classification of Headache
Disorders, 2nd edition, groups headache disorders
into primary and secondary headaches. The four categories of primary headache include migraine, TTH,
cluster headache (CH) and other TACs, and other
primary headaches. There are also eight categories
of secondary headache (table 1), and a third group
that includes central and primary causes of facial
pain and other headaches. The criteria for the pri-

From the Departments of Neurology (Drs. Lipton and Bigal), Epidemiology, and Population Health (Dr. Lipton), Albert Einstein College of Medicine, Bronx,
NY; The New England Center for Headache (Dr. Bigal), Stamford, CT; Division of Neuroscience, Imperial College London (Dr. Steiner), London, UK;
Department of Neurology (Dr. Silberstein), Thomas Jefferson University School of Medicine, Philadelphia; Jefferson Headache Center (Dr. Silberstein),
Thomas Jefferson University Hospital, Philadelphia, PA; and Department of Neurology (Dr. Olesen), Glostrup University Hospital, Copenhagen, Denmark.
Received October 8, 2003. Accepted in final form March 8, 2004.
Address correspondence and reprint requests to Dr. Richard B. Lipton, AECOM, 1300 Morris Park Ave., Bronx, NY 10461-1926; e-mail:
rlipton@aecom.yu.edu
Copyright 2004 by AAN Enterprises, Inc.

427

Table 1 First level of The International Classification of

Headache Disorders, 2nd edition

Table 2 ICHD-2 diagnostic criteria for 1.1 Migraine without

aura

Part one: The primary headaches

A.

At least 5 attacks fulfilling criteria BD

B.

Headache attacks lasting 4 72 hours (untreated or

unsuccessfully treated)

C.

Headache has at least two of the following characteristics:

1. Migraine
2. Tension-type headache (TTH)
3. Cluster headache and other trigeminal autonomic
cephalalgias

1. unilateral location

4. Other primary headaches

2. pulsating quality

Part two: The secondary headaches

3. moderate or severe pain intensity

5. Headache attributed to head and/or neck trauma

6. Headache attributed to cranial or cervical vascular
disorder

4. aggravation by or causing avoidance of routine physical

activity (e.g., walking or climbing stairs)
D.

During headache at least one of the following:

7. Headache attributed to non-vascular intracranial disorder

1. nausea and/or vomiting

8. Headache attributed to a substance or its withdrawal

2. photophobia and phonophobia

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
Part three: Cranial neuralgias, central and primary facial pain,
and other headaches
13. Cranial neuralgias and central causes of facial pain
14. Other headache, cranial neuralgia, central or primary
facial pain

mary headaches are clinical and descriptive and,

with a few exceptions (i.e., familial hemiplegic migraine [FHM]), are based on headache features, not
etiology. In contrast, secondary headaches are attributed to another underlying disorder.
Key operational rules for the classification are
summarized below.5
1. The classification is hierarchical, allowing diagnoses with varying degrees of specificity, using up
to four digits for coding at subordinate levels. The
first digit specifies the major diagnostic type, e.g.,
migraine (1.). The second digit indicates a subtype
within the category, e.g., migraine with aura (1.2)
(see table 1). Subsequent digits permit more specific diagnosis for some subtypes of headache: according to circumstantial requirements, FHM, for
example, could be coded as migraine (1.), migraine with aura (1.2), or, most precisely, as FHM
(1.2.4).
2. In clinical practice, patients should receive a diagnosis for each headache type or subtype they currently have (that is, have experienced within the
last year). For example, the same patient may
have and should be coded for all of medication
overuse headache (8.2), migraine without aura
(1.1), and frequent episodic TTH (ETTH) (2.2).
Multiple diagnostic codes should be listed in their
order of importance to the patient.
3. For headaches that meet all but one of a set of
diagnostic criteria, without fulfilling those of an428

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E.

Not attributed to another disorder

ICHD-2 International Classification of Headache Disorders

Second Edition.

other headache disorder, there are probable subcategories: for example, probable migraine (1.6).
4. The diagnosis of any primary headache requires
the exclusion of any other disorder that might be
the cause of the headache (i.e., of a secondary
headache disorder). This means that the history
and physical and neurologic examinations do not
suggest any of the disorders classified as secondary headaches, or these suggest such a disorder
but it is ruled out by investigations, or such disorder is present, but the headache does not occur for
the first time in close temporal relation to it.
5. Secondary headache diagnoses are applied when a
patient develops a new type of headache for the
first time in close temporal relation to onset of
another disorder known to cause headache; the
headache is categorized as attributed to that
disorder.
6. Diagnosis of secondary headache in a patient with
a preexisting primary headache can be challenging.
When headache worsens in the presence of another
disorder known to cause headache, there are two
possibilities. First, worsening may represent exacerbation of the preexisting primary headache. Second,
it may represent onset of a new, secondary headache. The readers are referred to Use of the IHS
criteria in neurologic practice, later in this article,
where we discuss this issue in detail.
1. Migraine. Migraine is classified into five major categories, the two most important of which are
migraine without aura (1.1) and migraine with aura
(1.2). The diagnostic criteria for migraine without
aura are unchanged from 1988. However, there is a
restructuring of the criteria for migraine with aura.
Chronic migraine (1.5.1) has been added. Ophthalmoplegic migraine, now considered a cranial neuralgia,
has been moved to item 13 (cranial neuralgias and
central causes of facial pain). When a patient fulfills
criteria for more than one type of migraine, each type

Table 3 ICHD-2 criteria for 1.2.1 Typical aura with migraine

headache
A. At least 2 attacks fulfilling criteria BD
B. Aura consisting of at least one of the following, but no motor
weakness:
1. fully reversible visual symptoms including positive features
(e.g., flickering lights, spots, or lines) and/or negative
features (i.e., loss of vision)
2. fully reversible sensory symptoms including positive
features (i.e., pins and needles) and/or negative features
(i.e., numbness)
3. fully reversible dysphasic speech disturbance
C. At least two of the following:
1. homonymous visual symptoms and/or unilateral sensory
symptoms
2. at least one aura symptom develops gradually over 5
minutes and/or different aura symptoms occur in succession
over 5 minutes
3. each symptom lasts 5 and 60 minutes
D. Headache fulfilling criteria BD for 1.1 Migraine without
aura begins during the aura or follows aura within 60
minutes
E. Not attributed to another disorder
ICHD-2 International Classification of Headache Disorders
Second Edition.

should be diagnosed and coded. Since chronic migraine

usually develops from episodic migraine, a patient
coded 1.5.1 will usually have an additional code for the
antecedent disorder (usually 1.1).
1.1 Migraine without aura. Relative to those of
1988, the diagnostic criteria for migraine without
aura (1.1) are little modified (table 2). They require
at least five lifetime attacks, lasting 4 to 72 hours,
with at least two of four pain features and at least
one of two sets of associated symptoms. In children,
attacks may be shorter, 1 to 72 hours, and in young
children photophobia and phonophobia may be inferred from behavior rather than reported. In addition, when attacks occur on 15 days/month, the
diagnoses are chronic migraine (1.5.1) plus migraine
without aura (1.1).
As before, criteria for migraine without aura are
met by various combinations of features. With regard to pain, unilateral, pulsating headache meets
the criteria but so does bilateral, pressing headache
if it is moderate or severe in intensity and aggravated by routine physical activity. With regard to
associated features, a patient without nausea or
vomiting but with both photophobia and phonophobia may fulfill the criteria (see table 2).
1.2 Migraine with aura. The criteria for migraine with aura (1.2) have been revised substantially. The typical aura of migraine is characterized
by focal neurologic features that usually precede migrainous headache but may accompany it or occur in
the absence of the headache (table 3).6 Typical aura
symptoms develop over 5 minutes and last no more
than 60 minutes, and visual aura is overwhelmingly

the most common.7 Typical visual aura is homonymous, often having a hemianopic distribution and
expanding in the shape of a crescent with a bright,
ragged edge, which scintillates. Scotoma, photopsia,
or phosphenes and other visual manifestations may
occur. Visual distortions such as metamorphopsia,
micropsia, and macropsia are more common in
children.8-10
Sensory symptoms occur in about one-third of patients who have migraine with aura.11 Typical sensory aura consists of numbness (negative symptom)
and tingling or paresthesia (positive symptoms). The
distribution is often cheiro-oral (face and hand). Dysphasia may be part of typical aura but motor weakness, symptoms of brainstem dysfunction, and
changes in level of consciousness, all of which may
occur,12 signal particular subtypes of migraine with
aura (hemiplegic, and basilar-type).
Recently, typical migraine aura has been noted to
occur with non-migrainous headache (i.e., headache
not fulfilling the criteria of 1.1). Such cases are coded
typical aura with non-migraine headache (1.2.2). Reports have associated apparently typical aura with
CH, chronic paroxysmal hemicrania, and hemicrania
continua (HC).13-15 These cases are classified according to both disorders [e.g., CH (3.1) plus typical aura
with non-migraine headache (1.2.2)].
Typical aura occurring in the absence of any
headache16-18 is coded typical aura without headache
(1.2.3), a disorder most often reported by middleaged men.24 Differentiating this benign disorder from
TIA, a medical emergency, may require investigation, especially when it first occurs after age 40,
when negative features (i.e., hemianopia) are predominant, or when the aura is of atypical duration.19
FHM (1.2.4) is the first migraine syndrome to be
linked to a specific set of genetic polymorphisms.20-23
The criteria for this disorder9 include those of migraine with aura (1.2) except that aura includes
some degree of motor weakness (hemiparesis) and
may be more prolonged than 60 minutes (up to 24
hours); additionally, at least one first-degree relative
has had similar attacks (also meeting these criteria).
Cerebellar ataxia may occur in 20% of FHM sufferers. The onset of weakness may be abrupt, but usually lasts less than 1 hour.24 A person with FHM may
develop migraine with aura on adulthood and migraine without aura later in life.24
The two known loci for FHM are on chromosomes
1 and 19, but some families do not link to either of
these, indicating that there is at least one additional
locus.25,26
Patients otherwise meeting these criteria but who
have no family history of this disorder are classified
as sporadic hemiplegic migraine (1.2.5), a disorder
new to the revised classification.27
Basilar-type migraine (1.2.6) is a new term, replacing basilar migraine. The change is intended to remove the implication that the basilar artery (or,
necessarily, its territory) is involved. The distinguishing feature of basilar-type migraine is a symptom proAugust (1 of 2) 2004

NEUROLOGY 63

429

file that suggests posterior fossa involvement.28

Diagnosis requires at least two of the following aura
symptoms, all fully reversible: dysarthria, vertigo, tinnitus, decreased hearing, double vision, visual symptoms simultaneously in both temporal and nasal fields
of both eyes, ataxia, decreased level of consciousness,
and simultaneously bilateral paresthesias. Because
60% of patients with FHM have basilar-type symptoms, basilar-type migraine should be diagnosed only
when weakness is absent. The headache meets criteria
for 1.1 migraine without aura (see table 2).
1.3 Childhood periodic syndromes that are commonly precursors of migraine. A number of more or
less well-described disorders are classified under this
heading.29
Cyclical vomiting (1.3.1) occurs in up to 2.5% of
schoolchildren.30-32 The hallmark of this disorder is recurrent and stereotyped episodes of intense but otherwise unexplained nausea and vomiting which last 1
hour to 5 days in children free of symptoms interictally.
Vomiting occurs at least four times in an hour, and no
signs of gastrointestinal disease can be found.
Abdominal migraine (1.3.2) afflicts up to 12% of
schoolchildren with recurrent attacks of abdominal
pain associated with anorexia, nausea, and sometimes vomiting.33,34 The abdominal pain has all of the
following characteristics: midline location, periumbilical or poorly localized; dull or just sore quality;
moderate or severe intensity. During it, there are at
least two of anorexia, nausea, vomiting, and/or pallor. Physical examination and investigations exclude
other causes of these symptoms.
Benign paroxysmal vertigo (1.3.3) is a disorder
characterized by recurrent (at least five) attacks,
each of multiple episodes of severe vertigo resolving
spontaneously after minutes to hours.35 Neurologic
examination and audiometric and vestibular functions are all normal between attacks, and the EEG is
also normal.
1.4 Retinal migraine. This disorder is rare. Recurrent attacks (at least two) of fully reversible scintillations, scotomata, or blindness, affecting one eye
only, are accompanied or followed within 1 hour by
migrainous headache (fulfilling criteria for 1.1).
Other causes of monocular visual loss, including TIA,
optic neuropathy, and retinal detachment, must be
ruled out by appropriate investigation.36 A recent review suggests that many patients with monocular
aura experience retinal infarction of migrainous origin.37 These patients should be coded as migrainous
infarction (1.5.4).
1.5 Complications of migraine. The ICHD-2 classifies chronic migraine (1.5.1) (see Controversies in
the classification of chronic daily headache, below)
when headache is both present and meets criteria for
migraine on 15 days per month for 3 months, in
the absence of medication overuse. Most cases of
chronic migraine, following this approach, evolve
from episodic migraine, and most from migraine
without aura,38 hence its classification as a complication of migraine. When medication overuse is
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present39 (acute anti-migraine drugs and/or opioids

or combination analgesics taken on 10 days/month,
or simple analgesics on 15 days/month), it is a
likely cause of chronic headache. According to the
ICHD-2, neither chronic migraine (1.5.1) nor
medication-overuse headache (8.2) can be diagnosed
with confidence until the overused medication has
been withdrawn: improvement within 2 months is
expected if the latter diagnosis is correct (and is necessary to confirm it), not if the former is present.
Meanwhile, the codes to be assigned are that of the
antecedent migraine (usually migraine without aura,
1.1) plus probable medication-overuse headache
(8.2.7) plus probable chronic migraine (1.6.5).
Status migrainosus (1.5.2) refers to an attack of
migraine with a headache phase lasting 72 hours.40
The pain is severe (a diagnostic criterion) and debilitating. Non-debilitating attacks lasting 72 hours
are coded as probable migraine without aura (1.6.1).
Persistent aura without infarction (1.5.13) is diagnosed when aura symptoms, otherwise typical of
past attacks, persist for 1 week. Investigation
shows no evidence of infarction. It is an unusual but
well-documented complication of migraine that is
now being introduced into the IHS classification.40 If
aura symptoms last more than 1 hour (typical aura),
but less than 1 week (persistent aura without infarction), a code of 1.6.2 (probable migraine with aura),
specifying the atypical feature (prolonged aura),
should be assigned.
Migrainous infarction (1.5.4) is an uncommon occurrence. One or more otherwise typical aura symptoms persist beyond 1 hour and neuroimaging
confirms ischemic infarction. Strictly applied, these
criteria distinguish this disorder from other causes of
stroke, which must be excluded41: the neurologic deficit develops during the course of an apparently typical attack of migraine with aura, and exactly
mimics the aura of previous attacks.
Migraine and epilepsy are comorbid disorders.42
Headaches are common in the postictal period, but
epilepsy can be triggered by migraine (migralepsy).
The criteria for migraine-triggered seizure (1.5.5) require that a seizure fulfilling diagnostic criteria for
one type of epileptic attack occurs during or within 1
hour after a migraine aura.
1.6 Probable migraine. Between 10% and 45% of
patients with features of migraine fail to meet all
criteria for migraine (or any of its subtypes).42 If a
single criterion is missing (and the full set of criteria
for another disorder are not met), the applicable code
is probable migraine (1.6). Epidemiologic studies
demonstrate that probable migraine is common and
associated with temporary disability and reduction
in health-related quality of life.43
2. Tension-type headache. TTH is the most common type of primary headache, with 1-year period
prevalences ranging from 31 to 74%.44 The 1988 classification distinguished two subtypes, ETTH (15
attacks per month) and chronic TTH (CTTH) (15
attacks per month).3 The revised classification dis-

Table 4 ICHD-2 criteria for episodic tension-type headache

(TTH)
2.1 Infrequent episodic TTH
A. At least 10 episodes occurring on 1 day per month on
average (12 days per year) and fulfilling criteria BD
B. Headache lasting from 30 minutes to 7 days
C. Headache has at least two of the following characteristics:
1. bilateral location
2. pressing/tightening (non-pulsating) quality
3. mild or moderate intensity
4. not aggravated by routine physical activity such as
walking or climbing stairs
D. Both of the following:
1. no nausea or vomiting (anorexia may occur)
2. no more than one of photophobia or phonophobia
E. Not attributed to another disorder
2.2 Frequent episodic TTH
A. At least 10 episodes occurring on 1 but 15 days per
month for at least 3 months (12 and 180 days per year)
and fulfilling criteria BE.
2.3 Chronic TTH
A. Headaches happening on 15 days per month on average
3 months (180 days per year and fulfilling criteria BD).
B. Headache lasts hours or may be continuous
C. Headache has at least two of the following characteristics:
1. bilateral location
2. pressing/tightening (non-pulsating) quality
3. mild or moderate intensity
4. not aggravated by routine physical activity such as
walking or climbing stairs
D. Both of the following:
1. no more than one of photophobia, phonophobia, or mild
nausea
2. neither moderate nor severe nausea or vomiting
E. Not attributed to another disorder

tinguishes three subtypes: infrequent ETTH (2.1)

(headache episodes on 1 day per month), frequent
ETTH (2.2) (headache episodes on 1 to 14 days per
month), and CTTH (2.3) (headache on 15 days per
month, perhaps without recognizable episodes).9
The diagnostic criteria for TTH are presented in
table 4. In contrast to migraine, the main pain features of TTH are bilateral location, non-pulsating
quality, mild to moderate intensity, and lack of aggravation by routine physical activity. The pain is not
accompanied by nausea, although just one of photo- or
phonophobia does not exclude the diagnosis.
CTTH evolves from ETTH in most cases. This feature of evolution is described in the notes but is not
part of the formal criteria. Like chronic migraine,
CTTH cannot be diagnosed in patients overusing
acute medication (see Controversies in the classification of chronic daily headache, below). Such patients
often meet criteria for, and in fact have, medication-

overuse headache (8.2), although withdrawal of the

medication is required to confirm this diagnosis. A
recently recognized disorder that phenotypically resembles CTTH, but is nosologically distinct from it
(as far as is known), does not evolve from an episodic
headache but is daily and unremitting from onset or
within 3 days of onset. This condition is separately
classified as new daily-persistent headache (4.8).
2.4 Probable tension-type headache. This code is
used when headache fulfills all but one of the criteria
for TTH and does not fulfill criteria for migraine
without aura.
3. Cluster headache and other trigeminalautonomic cephalalgias. The addition of the term
TACs to the classification reflects the observation
that CH is one of a group of primary headache disorders characterized by trigeminal activation coupled
with parasympathetic activation.45 The revised classification includes several disorders not quoted in
the previous edition.
3.1 Cluster headache. The diagnostic criteria for
CH have not substantially changed. This disorder
manifests as intermittent, short-lasting, excruciating
unilateral head pain accompanied by autonomic dysfunction. The pain of CH is described variously as
sharp, boring, drilling, knife-like, piercing, or stabbing, in contrast to the pulsating pain of migraine. It
usually peaks in 10 to 15 minutes but remains excruciatingly intense for an average of 1 hour within a
duration range of 15 to 180 minutes. During this
pain, patients find it difficult to lie still, exhibiting
often marked agitation and restlessness, and autonomic signs are usually obvious. After an attack, the
patient remains exhausted for some time.46
CH is classified into two subtypes. Attacks of episodic CH (3.1.1) occur in cluster periods lasting from
7 days to 1 year separated by attack-free intervals of
1 month or more. Approximately 85% of CH patients
have the episodic subtype. In chronic CH (3.1.2), attacks recur for 1 year without remission, or with
remissions lasting 1 month. Chronic CH can evolve
from episodic CH, or develop de novo,46 and may
revert to episodic CH.
Patients have been described who have both CH
and trigeminal neuralgia, and received the denomination of cluster-tic syndrome.45 In the IHS scheme
they should receive both diagnoses.
3.2 Paroxysmal hemicrania. As a group, the paroxysmal hemicranias have three main features: at
least 20 frequent (more than five per day) attacks of
short-lasting (2 to 30 minutes), severe and strictly
unilateral orbital, supraorbital, or temporal pain;
symptoms of parasympathetic activation ipsilateral
to the pain (as in CH); absolute response to therapeutic doses of indomethacin.47,48
The 1988 classification included chronic paroxysmal
hemicrania only. The revised classification includes episodic paroxysmal hemicrania (3.2.1) and chronic paroxysmal hemicrania (CPH) (3.2.2). Like CH, these
disorders are distinguished by the presence or absence
of attack-free intervals lasting 1 month or more.
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Some patients with both CPH and trigeminal neuralgia have been described (CPH-tic syndrome)49;
they should receive both diagnoses.
3.3 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and
tearing. Short-lasting unilateral neuralgiform
headache attacks with conjunctival injection and
tearing (SUNCT) syndrome (3.3) is a very rare primary headache. The diagnostic criteria require at
least 20 high-frequency attacks (3 to 200 per day) of
unilateral orbital, supraorbital, or temporal stabbing
or pulsating pain, lasting 5 to 240 seconds and accompanied by ipsilateral conjunctival injection and
lacrimation. The attacks are characteristically dramatic, with moderately severe pain peaking in intensity within 3 seconds and prominent tearing.50,51
3.4 Probable trigeminal autonomic cephalalgia.
Headache attacks believed to be a subtype of TAC
but fulfilling all but one of the diagnostic criteria for
it are diagnosed as probable TAC.
4. Other primary headaches. This group of miscellaneous primary headache disorders includes
some mimics of potentially serious secondary headaches, which need to be carefully evaluated by imaging or other appropriate tests. Some, like hypnic
headache, primary thunderclap headache, HC, and
new daily-persistent headache were not included in
the first IHS classification.
4.1 Primary stabbing headache. Episodic localized stabs of head pain occurring spontaneously in
the absence of any structural cause (formerly referred to as jabs and jolts) are diagnosed as primary stabbing headache (4.1). Pain is exclusively or
predominantly in the distribution of the first division
of the trigeminal nerve (orbit, temple, and parietal
area). It lasts for up to a few seconds and recurs at
irregular intervals with a frequency ranging from
one to many per day. Other features such as autonomic signs are lacking.52,53
4.2 Primary cough headache. This headache is
brought on suddenly by coughing, straining, or Valsalva maneuver, and not otherwise, in the absence of
any underlying disorder such as cerebral aneurysm
or, especially, Arnold-Chiari malformation.54 Diagnostic neuroimaging, with special attention to the
posterior fossa and base of the skull, is mandatory to
differentiate secondary and primary forms of cough
headache.55
4.3 Primary exertional headache. This disorder is
triggered by physical exercise, and not otherwise, and
is distinguished from primary cough headache (4.2)
(above) and headache associated with sexual activity
(4.4) (below). Primary exertional headache is pulsating
and lasts from 5 minutes to 48 hours. After the first
occurrence of any exertional headache of sudden onset,
appropriate investigations must exclude subarachnoid
hemorrhage and arterial dissection.56
4.4 Primary headache associated with sexual activity. Headache precipitated by sexual activity
usually begins as a dull bilateral ache as sexual excitement increases, and suddenly becomes intense at
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orgasm.57 Two subtypes are classified: pre-orgasmic

headache (4.4.1), a dull ache in the head and neck,
and orgasmic headache (4.4.2), explosive and severe
and occurring with orgasm. Diagnosis of the latter
requires exclusion of subarachnoid hemorrhage and
arterial dissection.58
4.5 Hypnic headache. This primary headache
disorder of the elderly59 is characterized by shortlived attacks (typically 30 minutes) of nocturnal
head pain that awaken the patient at a constant
time each night, in many cases on more nights than
not. It does not occur outside sleep. Hypnic headache
is usually bilateral (although unilaterality does not
exclude the diagnosis60) and usually mild to moderate, very different from the unilateral orbital or periorbital knife-like intense pain of CH. Autonomic
features are absent.
4.6 Primary thunderclap headache. Severe headache of abrupt onset, which mimics the pain of a
ruptured cerebral aneurysm, is classified as primary
thunderclap headache (4.6), although this code is not
applied to thunderclap headache meeting the criteria
for 4.2, 4.3, or 4.4 (above). Intensity peaks in less
than 1 minute. Pain lasts from 1 hour to 10 days and
may recur within the first week after onset but not
regularly over subsequent weeks or months.61 This
diagnosis can be established only after excluding
subarachnoid hemorrhage.
4.7 Hemicrania continua. This daily and continuous strictly unilateral headache is defined by its
absolute response to therapeutic doses of indomethacin. Pain is moderate, with exacerbations of severe
pain, and autonomic symptoms accompany these exacerbations although less prominently than in CH
and CPH.62,63 Some bilateral or alternating-side
cases have been reported.64
4.8 New daily-persistent headache (see Controversies in the classification of chronic daily headache,
below). The essence of this headache, which other-

Figure 1. Algorithm for headache diagnosis (modified

from reference 69).

Table 5 Red flags in the diagnosis of headache (modified from reference 69)
Red flag

Consider

Possible investigation(s)

Sudden-onset headache

Subarachnoid hemorrhage, bleed into a

mass or AVM, mass lesion (especially
posterior fossa)

Neuroimaging
Lumbar puncture (after neuroimaging evaluation)

Worsening-pattern headache

Mass lesion, subdural hematoma,

medication overuse

Neuroimaging

Headache with systemic illness (fever, Meningitis, encephalitis, Lyme disease,

neck stiffness, cutaneous rash)
systemic infection, collagen vascular
disease, arteritis

Neuroimaging
Lumbar puncture
Biopsy
Blood tests

Focal neurologic signs, or symptoms

other than typical visual or
sensory aura

Mass lesion, AVM, collagen vascular disease Neuroimaging

Collagen vascular evaluation

Papilloedema

Mass lesion, pseudotumor, encephalitis,

meningitis

Neuroimaging
Lumbar puncture (after neuroimaging evaluation)

Triggered by cough, exertion, or

Valsalva

Subarachnoid hemorrhage, mass lesion

Neuroimaging
Consider lumbar puncture

Headache during pregnancy or

postpartum

Cortical vein/cranial sinus thrombosis

Neuroimaging

Carotid dissection
Pituitary apoplexy
New headache type in a patient with
Cancer

Metastasis

Neuroimaging, lumbar puncture

Lyme disease

Meningoencephalitis

Neuroimaging, lumbar puncture

HIV

Opportunistic infection, tumor

Neuroimaging, lumbar puncture

AVM arteriovenous malformation.

wise resembles CTTH (2.3), is that it is daily and

unremitting from or very soon (3 days) after onset.
There is no history of evolution from episodic headache.38 If a history of progression from ETTH is
present, a diagnosis of CTTH should be assigned.
Diagnosis is not confirmed until it has been present
for 3 months, and cannot be made if this manner of
onset is not clearly recalled by the patient. Nor can it
be made in the presence of medication overuse. New
daily-persistent headache (NDPH) is typically bilateral, pressing or tightening in quality, of mild to
moderate intensity, and unaffected by routine physical activity, although the diagnostic criteria require
only any two of these features. There may be any but
not more than one of photophobia, phonophobia, or
mild nausea.
Controversies in the classification of chronic daily
headache. Chronic daily headache (CDH) of long
duration is a clinical syndrome not included in the
ICHD-2, defined by headaches that occur for 4
hours a day on 15 days a month over 3 months.37
CDH is one of the more common headache presentations to headache specialty care centers65 and afflicts
4 to 5% of the general population.66 Several studies
have reported difficulties using the 1988 system to
classify CDH sufferers.67 As a consequence, several
separate proposals for their classification have
emerged.68 The Silberstein and Lipton (S-L) criteria
have been most widely used.37
The S-L criteria divide primary CDH into trans-

formed migraine (TM), CTTH, NDPH, and HC, and

subclassify each of these into subtypes with medication overuse or without medication overuse. Of these,
the 1988 classification included only CTTH while the
revised classification has detailed diagnostic criteria
for all four types of primary CDH of long duration.
The terms TM and chronic migraine (CM) have
been used synonymously in the past but this is no
longer appropriate: CM has a specific definition in
the ICHD-2, which does not recognize TM as a separate entity. Three main differences exist between the
IHS and S-L systems. First, the IHS criteria for CM
require that headaches meeting criteria for migraine
without aura (1.1) occur on 15 days a month. To
classify TM, the S-L criteria require only one of three
links with migraine: a prior history of migraine
meeting 1988 IHS criteria; a period of escalating
headache frequency; concurrent and superimposed
attacks of migraine that fulfill the IHS criteria. Second, IHS reserves the diagnosis of CM (1.5.1) from
patients overusing acute medications (simple analgesics on 15 days a month or ergotamine, triptans,
opioids, or combination analgesics on 10 days a
month), applying instead probable chronic migraine
(1.6.5) plus probable medication-overuse headache
(8.2.7), and coding of the antecedent episodic migraine. The different approach of the S-L system
adds with medication overuse to the diagnosis and
defines the amounts of medication taken. Third,
headache with migrainous features cannot be classiAugust (1 of 2) 2004

NEUROLOGY 63

433

on average monthly frequency of the headaches, into

those of low-to-moderate frequency (15 headache
days per month), or those of high frequency (15
headache days per month). Second, based on average
duration, classify the headache as shorter-duration
(4 hours a day) or longer-duration (4 hours). Lowto-moderate frequency headaches of long duration
include migraine and ETTH; high frequency headaches of long duration include CM, CTTH, NDPH,
and HC.
Third, for shorter-duration headache of low or high
frequency, consider whether it is triggered by coughing, straining, or Valsalva maneuver or by exertion or
sexual activity or sleep (hypnic headache). Highfrequency, short-duration headaches not triggered by
these include episodic and chronic CH, episodic and
chronic paroxysmal hemicrania, hypnic headache, and
SUNCT syndrome. Headaches triggered by cough, exertion, and sexual activity include the disorders named
for these triggers.
The approach to differential diagnosis and the precise
criteria for each disorder are presented elsewhere.5,69
These algorithms should help neurologists generate differential diagnoses based on the second edition of the IHS
criteria.

Figure 2. Algorithm for primary headache diagnosis.

ETTH episodic tension-type headache; CCH chronic
cluster headache; ECH episodic cluster headache;
CPH chronic paroxysmal hemicrania; EPH episodic
paroxysmal hemicrania; CM chronic migraine; CTTH
chronic tension-type headache; NDPH new daily persistent headache; HC hemicrania continua.

fied as NDPH according to the IHS criteria while the

S-L classification allows this diagnosis in patients
with headache features of migraine or ETTH if the
disorder arises abruptly.
Using the IHS criteria in neurologic practice.
Headache diagnosis using the ICHD-2 should proceed in an orderly fashion. In this discussion, we
assume one headache disorder is present. If multiple
headache disorders occur together the conceptual
process needs to be repeated for each.
First, one needs to distinguish primary from secondary headaches. The approach is to spot red
flags that suggest the possibility of secondary headache (figure 1), to conduct the workup indicated by
those red flags (table 5), and thereby to diagnose any
secondary headache disorder that is present.69
In the absence of secondary headache, the clinician proceeds to diagnosing a primary headache disorder. If headache is atypical or difficult to classify,
the possibility of secondary headache should be reconsidered, although the modifying effect of any
treatment being taken should be kept in mind. We
propose the approach to differential diagnosis based
on figure 2.
The first step is to divide primary headache based
434

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August (1 of 2) 2004

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