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## Complete Summary

#### GUIDELINE TITLE

Diagnosis and classification of primary headache disorders. In:
Standards of care for headache diagnosis and treatment.

#### BIBLIOGRAPHIC SOURCE(S)

Martin V, Elkind A. Diagnosis and classification of primary headache
disorders. In: Standards of care for headache diagnosis and treatment.
Chicago (IL): National Headache Foundation; 2004. p. 4-18. \[35
references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

### COMPLETE SUMMARY CONTENT

 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Primary headaches including:

-   Migraine (with or without aura)

-   Tension-type headache (TTH)

-   Cluster headache

-   Chronic daily headache (CDH)

-   Chronic tension-type headache (CTTH)

-   New daily persistent headache

-   Hemicrania continua

#### GUIDELINE CATEGORY

Diagnosis

#### CLINICAL SPECIALTY

Family Practice\
Internal Medicine\
Neurology

#### INTENDED USERS

Health Care Providers\
Physicians

#### GUIDELINE OBJECTIVE(S)

-   To improve the medical treatment of headache

-   To review standard taxonomy for classifying headache

-   To outline a rational approach to headache diagnosis in clinical
    practice

-   To help physicians and other health care professionals to:

    -   Rule out secondary headache and establish a primary headache
        diagnosis

    -   Recognize indications for appropriate and timely referrals to
        specialists

#### TARGET POPULATION

Patients with primary headache disorders

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Diagnosis**

1.  Classification of headaches according to the International Headache
    Society (IHS) classification scheme

2.  Physical examination and recording of patient history

3.  Laboratory studies

    -   Hepatic profile

    -   Appropriate endocrinologic tests as necessary

    -   Erythrocyte sedimentation rate (in patients over the age of 50)

4.  Neuroimaging

    -   Computed tomography (CT)

    -   Magnetic resonance imaging/angiography (MRI/MRA)

5.  Lumbar puncture

6.  Electroencephalography (rarely necessary)

7.  Thermography (considered, but not recommended)

8.  Transcranial Doppler studies (considered, but not recommended)

9.  Specialist referral

#### MAJOR OUTCOMES CONSIDERED

-   Sensitivity and specificity of diagnostic tests

-   Predictive value of diagnostic tests

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

Not stated

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Expert Consensus

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

Not applicable

#### METHODS USED TO ANALYZE THE EVIDENCE

Review

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

Not stated

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

The guidelines presented in this monograph represent the consensus of an
advisory panel of practitioners chosen by the National Headache
Foundation (NHF) for their expertise. In addition to incorporating the
US Headache Consortium\'s recommendations, their conclusions reflect
clinical experience and the most recent medical literature.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Not applicable

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Not stated

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

Not applicable

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

**Diagnosis and Classification of Primary Headache Disorders**

The ability to make a rapid and accurate diagnosis is crucial to the
successful management of any headache disorder. Since head pain can have
many causes, a rational approach will facilitate differential diagnosis
and may increase the likelihood of a positive therapeutic outcome. This
chapter reviews standard taxonomy for classifying headache and outlines
a rational approach to headache diagnosis in clinical practice.

**Classification of Headache**

Headache disorders are generally classified as either primary or
secondary, and these classifications are further divided into specific
headache types. The primary headache disorders - those *not* associated
with an underlying pathology - include migraine, tension-type, and
cluster headache. Secondary headache disorders - those attributed to an
underlying pathologic condition - include any head pain of infectious,
neoplastic, vascular, drug-induced, or idiopathic origin (see table 1.1
of the original guideline document). The vast majority of patients who
present with headache have one of the primary disorders, as serious
secondary causes for presentation with head pain are rare.

A number of diagnostic schemata for headache have been proposed. As
early as 1962, for example, the Ad Hoc Committee on Classification of
Headache listed the features that are typically present during certain
types of headache, but it failed to indicate which features or
combinations of features were required to establish a diagnosis. By
1988, recognizing the need for improvement in headache classification,
the International Headache Society (IHS) published a new system. The
updated IHS criteria outline the specific characteristics necessary to
confirm a broad range of headache disorders.

Please refer to the original guideline document for a discussion of the
history of headache classification schemes up to and including the
International Headache Society\'s current criteria.

**Types of Primary Headache**

*Migraine*

Migraine is a chronic neurologic disorder characterized by episodic
attacks of head pain and associated symptoms. Methodologically identical
epidemiologic studies conducted 10 years apart show that the prevalence
and distribution of migraine have remained stable over the last decade
in the United States, with about 18% of women and 6% of men satisfying
diagnostic criteria for the condition. Studies done outside the United
States show an agreement in overall migraine prevalence rates. For
example, in data from the Netherlands, the 1-year prevalence of migraine
in adults is estimated at 10 to 12% in men and 15 to 18% in women. Even
though it is widespread, migraine remains underdiagnosed; only 48% of
Americans who satisfy criteria for migraine reported receiving a
physician diagnosis of migraine.

[With Aura]{.underline}

The IHS recognizes 6 variants of migraine, but the most common types
seen in primary care practice are migraine with aura (formerly
\"classic\" migraine), migraine without aura (formerly \"common\"
migraine), and probable migraine, formerly called migrainous headache.
Providers should suspect migraine with aura whenever a headache is
preceded by one of the following neurologic symptoms:

-   Visual symptoms, such as blind spots, flashes of light, zigzag
    lines, or other distortions

-   Motor weakness

-   Sensory symptoms, such as paresthesias

-   Aphasia

-   Signs of brainstem dysfunction, such as diplopia, ataxia, or vertigo

The symptoms should be fully reversible, developing over 5 to 20 minutes
and lasting less than 60 minutes. The table below summarizes the IHS
criteria for migraine with aura. It is commonly observed in clinical
practice that not all auras are followed by a headache or a headache
that is associated with characteristics of migraine. If aura occurs
without subsequent headache, then the condition is a typical aura
without headache; if a nonmigraine headache follows aura, then it is
classified as a typical aura with a nonmigraine headache.

**Table: Adapted IHS Criteria for Migraine with Typical Aura**

+---+---------------------------------------------------------------------+
| * | At least 2 attacks fulfilling criteria B                            |
| * |                                                                     |
| A |                                                                     |
| * |                                                                     |
| * |                                                                     |
+---+---------------------------------------------------------------------+
| * | Aura consisting of at least one of the following, but no motor      |
| * | weakness:                                                           |
| B |                                                                     |
| * | -   Fully reversible visual symptoms including positive features    |
| * |     (e.g., flickering lights, spots, or lines) and/or negative      |
|   |     features (i.e., loss of vision)                                 |
|   |                                                                     |
|   | -   Fully reversible sensory symptoms including positive features   |
|   |     (i.e., pins and needles) and/or negative features (i.e.,        |
|   |     numbness)                                                       |
|   |                                                                     |
|   | -   Fully reversible dysphasic speech disturbance                   |
+---+---------------------------------------------------------------------+
| * | At least two of the following:                                      |
| * |                                                                     |
| C | -   Homonymous visual symptoms and/or unilateral sensory symptoms   |
| * |                                                                     |
| * | -   At least one aura symptom develops gradually 5 minutes or more  |
|   |     and/or different aura symptoms occur in succession over 5 or    |
|   |     more minutes                                                    |
+---+---------------------------------------------------------------------+
| * | Headache fulfilling criteria for migraine without aura beginning    |
| * | during the aura or follows the aura within 60 minutes               |
| D |                                                                     |
| * |                                                                     |
| * |                                                                     |
+---+---------------------------------------------------------------------+

[Without Aura]{.underline}

Migraine without aura is characterized by head pain that is virtually
indistinguishable from the pain experienced by patients with aura; the
difference, as the name implies, is that in migraine without aura, no
aura precedes a migraine attack. Because migraine without aura does not
have a single distinguishing feature, the IHS criteria for migraine
without aura require the presence of a constellation of symptoms (see
table below).

**Table: Adapted from IHS Criteria for Migraine without Aura\***

+------------------------------------------+---------------------------+
| **Headache Descriptions (Any 2)**        | **Associated Symptoms     |
|                                          | (Any 1)**                 |
+==========================================+===========================+
| -   Unilateral                           | -   Nausea and/or         |
|                                          |     vomiting              |
| -   Pulsatile quality                    |                           |
|                                          | -   Photophobia and       |
| -   Moderate to severe pain intensity    |     phonophobia           |
|                                          |                           |
| -   Aggravation by or causing avoidance  |                           |
|     of routine physical activity         |                           |
+------------------------------------------+---------------------------+
| \*Must have 5 attacks fulfilling the     |                           |
| above criteria and no signs of a         |                           |
| secondary headache disorder. The         |                           |
| headaches last 4--72 hours               |                           |
+------------------------------------------+---------------------------+

Despite the existence of specific criteria, clinicians frequently
misdiagnose migraine. One reason for error is the criteria themselves.
The IHS criteria do not include all symptoms frequently observed in
episodes of migraine. Consequently, migraine associated with muscle or
neck pain, a non-IHS migraine diagnostic criterion, is often diagnosed
as tension-type headache (TTH), or migraine associated with nasal
symptoms such as rhinorrhea and nasal congestion, also not included as
IHS diagnostic criteria, is diagnosed as a \"sinus\" headache. In both
cases, research demonstrates that these headaches are usually migraine.

In addition, clinicians often focus on the presence of a single symptom
to make a migraine diagnosis. In one study, providers who diagnosed
migraine only based on the presence of unilateral head pain diagnosed
31% of patients incorrectly, and those who used vomiting as the hallmark
symptom missed 50% of all cases. Similarly, many patients who present
with tension headache, stress headache, and \"sinus headaches\" may be
IHS migraineurs. A recent study of patients with self-reported sinus
headaches found that 63% met IHS diagnostic criteria for migraine
without aura and 33% met IHS criteria for migrainous headaches (in which
patients lack one of the IHS diagnostic criteria for migraine). In other
work, Stang compared the outpatient headache diagnoses of primary care
physicians with the headache diagnoses of a validated algorithm. Using
the validated algorithm as the gold standard, primary care physicians
missed the diagnosis of migraine headache in 55% of migraine patients.
Coexisting TTH in 50% of these patients was the most significant
negative predictor of a diagnosis of migraine headache. A recent study
by Lipton found that the occurrence of 3 primary criteria (disability,
nausea, and sensitivity to light) were highly predictive for migraine in
a primary care setting. Using this combination of migraine symptoms,
their 3-item ID Migraine Screener yielded a sensitivity of 0.81 (95%
confidence interval \[CI\], 0.77 to 0.85), a specificity of 0.75 (95%
CI, 0.64 to 0.84), and a positive predictive value of 93.3 (95% CI, 89.9
to 95.8). The sensitivity and specificity of these attributes is similar
to other common screening tools, such as the Primary Care Evaluation of
Mental Disorders (PRIME-MD) for depression or a prostate-specific
antigen (PSA) test for prostate cancer.

An especially significant complicating factor in the diagnosis of
migraine is the existence of comorbid illness. Migraine has been
associated with a number of psychiatric and medical-neurologic
illnesses. Therefore, providers should not be surprised to find an
increased incidence of affective and anxiety disorders among migraine
patients. Bipolar psychiatric disturbances and phobias are also noted.
The incidence of stroke, epilepsy, essential tremor, mitral valve
prolapse, and Raynaud\'s disease also are increased in migraine patients
compared with their nonmigraine counterparts.

*Tension-Type Headache*

TTH is the most common type of primary headache. In the general
population, estimates of the prevalence of episodic TTH vary widely,
from 30 to 80%. The prevalence of chronic tension-type headache (CTTH)
was 2% in men and 5% in women in a Danish study. Compared with migraine,
the pain of TTH tends to be less severe, bilateral, nonpulsating, and
not aggravated by routine physical activity. Symptoms associated with
migraine attacks, such as nausea, phonophobia, or photophobia, are
rarely present, but there can be symptomatic overlap. Studies have shown
that 25% of TTH patients also have migraine, and 62% of migraineurs have
TTH. Moreover, epidemiologic research suggests that TTH, when it
coexists with migraine, might represent a segment on the continuum of
the same disorder. The IHS criteria for TTH, listed in the table below,
outline a range of specific characteristics that distinguish TTH from
migraine.

**Table: Adapted IHS Criteria for General Diagnosis of Episodic TTH\***

+------------------------------+---------------------------------------+
| **Headache Description (Any  | **Associated Symptoms (Any 1)**       |
| 2)**                         |                                       |
+==============================+=======================================+
| -   Pressing or tightening   | -   No nausea or vomiting             |
|                              |                                       |
| -   Mild to moderate         | -   Photophobia or phonophobia (1     |
|     intensity                |     allowed)                          |
|                              |                                       |
| -   Bilateral location       |                                       |
|                              |                                       |
| -   No worsening with        |                                       |
|     exertion                 |                                       |
+------------------------------+---------------------------------------+
| \*Must have had \>10         |                                       |
| previous headache episodes   |                                       |
| and no evidence of a         |                                       |
| secondary headache disorder. |                                       |
| Episodic TTH occur \<15      |                                       |
| days/month and CTTH occur    |                                       |
| \>15 days/month. CTTH also   |                                       |
| allows the presence of mild  |                                       |
| nausea                       |                                       |
+------------------------------+---------------------------------------+

In the 2004 IHS diagnostic criteria, episodic TTH is a condition without
associated symptoms other than photophobia or phonophobia. Although this
further separates migraine and tension headache, it leaves more headache
presentations somewhere in between these two extremes. Much of the void
is filled by a diagnosis of \"probable migraine,\" which represents a
headache that lacks one diagnostic criterion for migraine headache.

*Cluster Headache*

Findings from prevalence studies of cluster headache are controversial,
but one survey calculated a prevalence of 0.24% in the general
population. Patients with cluster headaches generally rate the intensity
of their pain as among the worst imaginable, and cluster headache may be
the most severe of the primary headache disorders. Most often, cluster
headache occurs once every 24 hours for 6 to 12 weeks at a time, with
remission periods typically lasting 12 months. Typical age of onset for
both men and women is 27 to 31 years. However, cluster headaches are one
of the few headache syndromes that are more frequent in men than in
women. Research shows male to female ratios that range from 5.0:1 to
6.7:1, but there is evidence that the gap may have narrowed in the
1990s. Two recent studies found sex ratios that still showed greater
frequency in men, but the ratios were only 3.5:1 and 2:1. Cluster
attacks have several differentiating features. Most important of these
is the presence of transient autonomic symptoms. These include signs of:

-   Sympathetic hypofunction, such as miosis and ptosis

-   Parasympathetic hyperfunction, such as rhinorrhea and lacrimation

These associated symptoms are always unilateral and ipsilateral to the
headache; systemic symptoms, such as bradycardia, hypertension, and
increased gastric acid production, may also accompany an attack. Another
unique feature is that cluster episodes are \"side-locked\" - the
headaches are *always* on the same side, even when long intervals
separate headache episodes. See the table below for the IHS criteria for
cluster headache.

**Table: Adapted IHS Criteria for the General Diagnosis of Cluster
Headache\***

+---------------------------------------+------------------------------+
| **Headache Description (All 4)**      | **Autonomic Symptoms (Any    |
|                                       | 2)**                         |
+=======================================+==============================+
| -   Severe headache                   | -   Rhinorrhea               |
|                                       |                              |
| -   Unilateral                        | -   Lacrimation              |
|                                       |                              |
| -   Duration of 15--180 min           | -   Facial sweating          |
|                                       |                              |
| -   Orbital, periorbital, or temporal | -   Miosis                   |
|     location                          |                              |
|                                       | -   Eyelid edema             |
|                                       |                              |
|                                       | -   Conjunctival injection   |
|                                       |                              |
|                                       | -   Ptosis                   |
+---------------------------------------+------------------------------+
| \*No evidence of a secondary headache |                              |
| disorder. Episodic cluster headaches  |                              |
| occur for \<1 year and chronic        |                              |
| headaches occur for \>1 year.         |                              |
+---------------------------------------+------------------------------+

*Chronic Headache*

Population-based studies suggest that 4 to 5% of the population suffer
from chronic daily headache (CDH). CDH is generally defined as more than
15 days per month with headache of any kind. The IHS has recognized the
diagnosis of certain chronic headache disorders, but investigators have
recommended that a separate classification for CDH be added to the IHS
diagnostic criteria. The table below lists the proposed classification
of CDH. The newly published IHS criteria, while not adding a separate
category of CDH, recognized and added to the migraine classification the
disorder of chronic migraine (see Table 1.7 in the original guideline
document). Patients with chronic migraine have headaches on 15 or more
days per month, and the headaches retain much of the typical
characteristics of episodic migraine without aura.

**Table. The Proposed Classification of Chronic Daily Headache**\*

  -----------------------------------------------------------------------
  Chronic migraine

  Chronic tension-type headache

  New daily persistent headache

  Hemicrania continua

  \*Headaches can occur both with and without rebound headache
  -----------------------------------------------------------------------

[Chronic Tension-Type Headache]{.underline}

The characteristics of the head pain are similar in chronic and episodic
TTH. Proposed diagnostic criteria for CTTH include more than 15 headache
days per month, a history of episodic TTH, and headache characteristics
similar to episodic IHS TTH. Patients may also have mild accompanying
nausea and still receive a diagnosis of chronic tension type headaches,
which represents a change from past criteria.

[New Daily Persistent Headache]{.underline}

The IHS defines new daily persistent headache as headache that is daily
and unremitting from very soon after onset (within 3 days at most). The
pain is typically bilateral, pressing or tightening in quality, and of
mild to moderate intensity. There may be photophobia, phonophobia, or
mild nausea.

[Hemicrania Continua]{.underline}

Hemicrania continua is a continuous headache, which is strictly
unilateral, associated with ipsilateral autonomic symptoms (similar to
cluster headache), and responsive to indomethacin. The pain must be at
least intermittently severe and present for 1 month.

**Diagnosis in Clinical Practice**

Historically, the recognition of primary headache syndromes was based on
a pattern of symptoms, such as those defined by the IHS. Yet most
office-based evaluations of headache occur when patients are
asymptomatic. This fact in itself rules out most urgent pathologies and
allows a physician to rely on impact-based recognition of migraine. On
those occasions when a person is being evaluated during a headache, it
is best to rely on IHS criteria (see table below).

**Table: Characteristics of Primary Headache Disorders**

  ------------------------------------------------------------------------------
  ** **                  **Migraine**      **Tension-Type\***    **Cluster**
  ---------------------- ----------------- --------------------- ---------------
  **Location**           Unilateral        Bilateral             Strictly
                                                                 unilateral

  **Intensity**          Moderate/severe   Mild/moderate         Severe

  **Duration**           4 to 72 hours     30 min to 7 days      15 to 90 min

  **Quality**            Throbbing         Pressing/tightening   Severe

  **Associated           Yes               No                    Yes \--
  symptoms**                                                     autonomic

  **Gender**             Female \> male    Female \> male        Male \> female
  ------------------------------------------------------------------------------

\*Episodic

Given the constraints of clinical practice, however, primary headache
disorders can be quickly and reliably recognized by asking the following
4 questions:

1.  **How do headaches interfere with your life?**

> Recurrent headaches that produce significant disability should be
> considered migraine until proven otherwise. Allowing a patient to
> explain the impact of headaches helps define disability, establishes
> rapport, and directs therapeutic interventions. For example, headaches
> that restrict important activities such as work or home
> responsibilities suggest a need for aggressive therapy, whereas
> headaches with less impact may be adequately treated with
> nonprescription medications. In this way, the clinician provides the
> appropriate tools for managing the spectrum of headache activity that
> a patient is experiencing. This paves the way for patient involvement,
> and it allows clinicians to focus on managing patients rather than
> just headaches.

2.  **How frequently do you experience headaches of any type?**

> The frequency of headaches alerts the clinician to chronic headache
> disorders and migraine transformation. Daily or near-daily headache
> patterns should alert the provider to the possibility of medication
> overuse. Frequent headaches raise the issue of adding a preventive
> medication to the patient\'s treatment regimen.

3.  **Has there been any change in your headache pattern over the last 6
    months?**

> An affirmative answer indicates the need for a more in depth
> evaluation of possible warning signs, or \"red flags\" (see table
> below), whereas a negative response reassures the physician and the
> patient that serious underlying disease is unlikely.

+-----------------------------------------------------------------------+
| **Red Flags for a Secondary Headache Disorder**                       |
+=======================================================================+
| -   A new or different headache                                       |
|                                                                       |
| -   \"Thunderclap\" headache (peak intensity within seconds to        |
|     minutes)                                                          |
|                                                                       |
| -   Worst headache ever                                               |
|                                                                       |
| -   Focal neurologic signs or symptoms, such as papilledema, motor    |
|     weakness, memory loss, papillary abnormalities, or sensory loss   |
|                                                                       |
| -   Change in existing headaches                                      |
|                                                                       |
| -   New onset headache after age 50                                   |
|                                                                       |
| -   Headache associated with systemic symptoms (fever, weight loss,   |
|     jaw claudication)                                                 |
+-----------------------------------------------------------------------+

4.  **How often and how effectively do you use medication to treat
    headaches?**

> Generally, patients should not use acute treatment medications more
> than 2 days a week. This is an ideal goal and, at present, for many
> patients it is not possible to attain. Being pain free and fully
> functional within 2 to 4 hours is the goal of acute headache therapy.
> An inability to achieve this goal suggests the need to change
> medication. It is essential to query patients about prescription and
> nonprescription medication usage. Overuse of any acute headache
> remedy, prescription or nonprescription, may promote more frequent
> headaches. Self-management efforts can be assessed, and therapy can be
> modified to meet a broad spectrum of a patient\'s needs.

*Comfort Signs*

Primary headache conditions are associated with certain clinical
attributes that can be identified through the clinical history. These
characteristics, when present, increase diagnostic comfort (see table
below). For example, when migraine seems a likely diagnosis, query about
prodrome, aura, postdrome, family history, and menstruation, which
correlate highly with migraine. Although many headaches will not
precisely fulfill the IHS diagnostic criteria for a given diagnosis, it
can be reassuring when they do. In addition, patients should have normal
vital signs, physical examination, and screening neurologic examination.
Any abnormalities require further evaluation. Response to a treatment
plan over time supports a diagnosis of primary headache. Therefore,
scheduling follow-up visits allows the physician to identify the rare
secondary cause of headaches, assess treatment response, screen for
comorbidities, such as depression or irritable bowel syndrome, and
monitor use of medication.

+-----------------------------------------------------------------------+
| **Migraine Comfort Signs**                                            |
+=======================================================================+
| -   Positive family history of migraine                               |
|                                                                       |
| -   Headache related to menstrual cycle                               |
|                                                                       |
| -   Headaches preceded by typical aura                                |
|                                                                       |
| -   Headaches remaining periodic and stable over time                 |
|                                                                       |
| -   Normal physical and neurologic findings                           |
+-----------------------------------------------------------------------+

**Diagnostic Testing**

*Laboratory Studies*

Laboratory studies, including complete blood count (CBC), blood
chemistries, and urine analysis, are generally of little value for
diagnosis. Baseline laboratory studies, such as a hepatic profile, may
be valuable in patients who are to start pharmacologic agents that may
be contraindicated in those with suspected or unsuspected liver disease
(e.g., divalproex sodium). Secondary headache can rarely be caused by
endocrinopathy (pituitary tumors, thyroid disease, etc), and appropriate
endocrinologic tests may be ordered if a disorder is suspected. Always
assess erythrocyte sedimentation rate in patients over 50 years of age
to rule out giant cell arteritis.

*Neuroimaging*

Neuroimaging, including computed tomography (CT) and magnetic resonance
imaging/angiography (MRI/MRA) will detect most structural abnormalities
that can result in headache symptoms. Magnetic resonance imaging is more
sensitive for specific abnormalities of the posterior fossa and
cervical/medullary pathology as well as white matter disturbances. The
US Headache Consortium guidelines for neuroimaging suggest routine
testing is not warranted in recurrent headache (specifically migraine)
without neurologic findings, a history of seizures, or changes in
headache history. Atypical headache history may also warrant
neuroimaging.

*Lumbar Puncture*

Lumbar puncture is indicated when the following diseases are suspected:

-   Viral or bacterial meningitis

-   Subarachnoid hemorrhage

-   Carcinomatous meningitis

-   Pseudotumor cerebri

-   Encephalitis

-   Systemic diseases known to affect the central nervous system (i.e.,
    sarcoidosis, lupus, vasculitis)

In the case of suspected subarachnoid hemorrhage, a noncontrast computed
tomography will miss approximately 10% of bleeds; lumbar puncture is
essential to rule out xanthochromia. Keep in mind that complications of
lumbar puncture may include headache.

*Other Tests*

Electroencephalography is rarely necessary or useful in the routine
evaluation of a headache patient. It may be useful in patients with
headache and a seizure history, head trauma with signs of alteration of
consciousness, or syncope. Thermography has not been found to be useful
in the diagnosis or management of individuals with headache.
Transcranial Doppler studies have also not demonstrated value in the
evaluation of headache patients.

**Specialist Referral**

Knowing when to refer a headache patient is an important aspect of
managing patients optimally in the primary care setting. The table below
lists the appropriate reasons to refer a headache patient to a
specialist.

+-----------------------------------------------------------------------+
| **Reasons to Refer Headache Patients**                                |
+=======================================================================+
| -   Diagnostic uncertainty                                            |
|                                                                       |
| -   Treatment failure                                                 |
|                                                                       |
| -   Suspicion of a secondary headache syndrome                        |
|                                                                       |
| -   Rebound or chronic daily headache                                 |
|                                                                       |
| -   Reassurance for patient or provider                               |
+-----------------------------------------------------------------------+

#### CLINICAL ALGORITHM(S)

None provided

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of evidence supporting the recommendations is not specifically
stated.

In addition to incorporating the US Headache Consortium\'s
recommendations, the conclusions reflect clinical experience and the
most recent medical literature.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

Since head pain can have many causes, a rational approach will
facilitate differential diagnosis and may increase the likelihood of a
positive therapeutic outcome.

#### POTENTIAL HARMS

Not stated

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

Drug therapy is constantly evolving as new research, clinical trials,
case reports, and opinions are published. Many of the drugs recommended
in the original guideline document are not approved by the US Food and
Drug Administration (FDA) for treatment of headache, nor are they
necessarily the same as those therapies recommended by the manufacturer
for labeled indications. Their use in headache, however, may be
supported by the scientific literature and by the authors\' clinical
experiences. While efforts have been made to ensure accuracy, the
authors and publisher do not assume responsibility for the consistent
updating of available information for the original guideline document,
nor for any errors or omissions, nor for any consequences thereof. The
onus is on the practitioner to evaluate recommendations in light of the
clinical condition of the patient and recent medical literature. The
authors advise the practitioner to consult other sources, especially the
manufacturers\' warnings and precautions, before prescribing any drug
with which they are unfamiliar. Practitioners are also advised that
while these guidelines will address the needs of many patients, there
will be circumstances calling for exceptions to these recommendations.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Chart Documentation/Checklists/Forms\
Foreign Language Translations\
Patient Resources\
Slide Presentation

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness\
Patient-centeredness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Martin V, Elkind A. Diagnosis and classification of primary headache
disorders. In: Standards of care for headache diagnosis and treatment.
Chicago (IL): National Headache Foundation; 2004. p. 4-18. \[35
references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2004

#### GUIDELINE DEVELOPER(S)

National Headache Foundation - Private Nonprofit Organization

#### SOURCE(S) OF FUNDING

National Headache Foundation

#### GUIDELINE COMMITTEE

Not stated

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Authors*: Vincent Martin, MD, and Arthur Elkind, MD

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: None available

Print copies: Available from the National Headache Foundation, 820 N.
Orleans, Suite 218, Chicago, IL 60610; Phone: (888) NHF-5552; Web
address: [www.headaches.org](http://www.headaches.org/)

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   Headache screening questionnaire. Chicago (IL): National Headache
    Foundation. 2 p. Electronic copies available in Print Documentation
    Format (PDF) from the [National Headache Foundation Web
    site](http://www.headaches.org/professional/educationindex.html).

-   The complete headache chart. Chicago (IL): National Headache
    Foundation (NHF); 2 p. Electronic copies available in Portable
    Document Format (PDF) from the [National Headache Foundation Web
    site](http://www.headaches.org/consumer/presskit/hachart.pdf)

-   National Headache Foundation fact sheet. Chicago (IL): National
    Headache Foundation (NHF); 2004 Oct. 2 p. Electronic copies
    available in Portable Document Format (PDF) from the [National
    Headache Foundation Web
    site](http://www.headaches.org/consumer/presskit/factsheet.pdf).

Print copies: Available from the National Headache Foundation, 820 N.
Orleans, Suite 218, Chicago, IL 60610; Phone: (888) NHF-5552; Web
address: [www.headaches.org](http://www.headaches.org/)

#### PATIENT RESOURCES

The National Headache Foundation (NHF) has created a variety of
educational resources for patients, including informative brochures, a
patient diary for migraines, Power Point presentations, and patient
guides; many of these resources are available in both Spanish and
English. Some of these items are available as print copies for purchase
through the [NHF online
store](http://www.headaches.org/consumer/storeindex.jsp). Electronic
versions of other resources are available through the consumer education
section of the [NHF Web
site](http://www.headaches.org/consumer/educationindex.html).

Print copies: Available from the National Headache Foundation, 820 N.
Orleans, Suite 218, Chicago, IL 60610; Phone: (888) NHF-5552; Web
address: [www.headaches.org](http://www.headaches.org/).

Please note: This patient information is intended to provide health
professionals with information to share with their patients to help them
better understand their health and their diagnosed disorders. By
providing access to this patient information, it is not the intention of
NGC to provide specific medical advice for particular patients. Rather
we urge patients and their representatives to review this material and
then to consult with a licensed health professional for evaluation of
treatment options suitable for them as well as for diagnosis and answers
to their personal medical questions. This patient information has been
derived and prepared from a guideline for health care professionals
included on NGC by the authors or publishers of that original guideline.
The patient information is not reviewed by NGC to establish whether or
not it accurately reflects the original guideline\'s content.

#### NGC STATUS

This NGC summary was completed by ECRI on April 7, 2005. The information
was verified by the guideline developer on April 26, 2005.

#### COPYRIGHT STATEMENT

These guidelines are for reference purposes only and are not to be mass
produced. This information is copyrighted by the National Headache
Foundation, 2005.

### DISCLAIMER

#### NGC DISCLAIMER

The National Guideline Clearinghouse™ (NGC) does not develop, produce,
approve, or endorse the guidelines represented on this site.

All guidelines summarized by NGC and hosted on our site are produced
under the auspices of medical specialty societies, relevant professional
associations, public or private organizations, other government
agencies, health care organizations or plans, and similar entities.

Guidelines represented on the NGC Web site are submitted by guideline
developers, and are screened solely to determine that they meet the NGC
Inclusion Criteria which may be found at
<http://www.guideline.gov/about/inclusion.aspx> .

NGC, AHRQ, and its contractor ECRI Institute make no warranties
concerning the content or clinical efficacy or effectiveness of the
clinical practice guidelines and related materials represented on this
site. Moreover, the views and opinions of developers or authors of
guidelines represented on this site do not necessarily state or reflect
those of NGC, AHRQ, or its contractor ECRI Institute, and inclusion or
hosting of guidelines in NGC may not be used for advertising or
commercial endorsement purposes.

Readers with questions regarding guideline content are directed to
contact the guideline developer.

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Date Modified: 11/10/2008