Headache Classification and Diagnosis: Focus on Migraine and Tension-Type Headache, Study notes of Neurology

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Headache Diagnosis and

Testing

Headache Diagnosis and

Testing

HEADACHE DIAGNOSIS AND

TESTING

HEADACHE DIAGNOSIS AND

TESTING

Assess the value of diagnostic testing in

primary and secondary headache disorders

Evaluate the prevalence of primary and

secondary headache disorders in the general

population and clinical practice

Identify warning signs of serious secondary

headaches

Review the clinical features, diagnostic

criteria, and differential diagnosis of migraine

Objectives

Headache is, without question, one of the most common symptoms that neurologists evaluate. Not unexpectedly, the differential diagnosis of this highly prevalent symptom is vast, with over 300 different headache types and etiologies. Understanding headache classification and diagnosis is, therefore, a clinical imperative and a requisite for diagnostic testing and treatment.

In 2004, the International Headache Society (IHS) formulated and published the second edition of the headache classification system with operational diagnostic criteria for a broad range of headache disorders. These criteria are based on an international consensus of expert opinion and have been endorsed by the World Health Organization and incorporated into the International Classification of Diseases (ICD-10). These criteria have:

• Established a uniform terminology and consistent diagnostic criteria
• Facilitated epidemiologic studies and multinational clinical trials
• Provided the basis for the current research and treatment guidelines

Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders. Cephalalgia. 2004;24(suppl 1):1:160.

PRIMARY HEADACHE: CLINICAL

PRACTICE VS. POPULATION

PRIMARY HEADACHE: CLINICAL

PRACTICE VS. POPULATION

Other 3%

Dowson A et al. Cephalalgia. 2002.

Primary Care Practice

76%

3% Migrainous

18%

Migraine

16%

Tension-type Headache

78%

Other 6%

General Population

Rasmussen BK et al. J Cin Epidemiol. 1991.

In one international study done in primary care offices, a total of 377 patients returned completed diaries. Of the 94% who consulted their primary care physicians for headache, 76% had migraine and 18% had migrainous headache. Few patients had tension-type headache. If a “Sinus Headache” was diagnosed, it would have been coded as “Other”; therefore, this would represent a small percentage of the total study's population.

For the US patients in this study, the results were almost identical. Of the 162 patients who returned diaries, 75% of those who consulted their primary care physicians with headache had migraine, and 19% had migrainous headache. Few of these patients (4%) had tension-type headache.

However, when surveying the general population, what we see is a larger prevalence of tension-type headache. This suggests that patients with tension-type headache do not frequent primary care physicians for medical care. In contrast, patients with migraine seek medical treatment.

Dowson A, Dahlof C, Tepper S, Newman L. Prevalence and diagnosis of migraine in a primary care setting. Cephalalgia. 2002;22:590-591.

Rasmussen BK, Jensen R, Schroll M, Olesen J. Epidemiology of headache in a general population — a prevalence study. J Clin Epidemiol. 1991;44:1147-1157.

PRIMARY OR SECONDARY

HEADACHES IN EMERGENCY ROOM

PRIMARY OR SECONDARY

HEADACHES IN EMERGENCY ROOM

95% 5%

32% (^) 59% 9%

0% 20% 40% 60% 80% 100%

Met IHS Criteria for Migraine

Diagnoses received

Migraine Headache not specified Other

• 100% took nonprescription medication
• 65% received cocktail (NSAIDs, DA antagonist, antihistamine)
• 49% never taken prescription medication
• 24% received opioids
• 7% received migraine-specific medication
• 100% took nonprescription medication
• 65% received cocktail (NSAIDs, DA antagonist, antihistamine)
• 49% never taken prescription medication
• 24% received opioids
• 7% received migraine-specific medication Blumenthal H et al., Headache. 2003.

This study investigated the diagnosis and clinical outcome of patients who went to the emergency department for treatment of headache. Fifty-seven patients treated for acute primary headache in the emergency department completed a questionnaire. Overall, 95% of the 57 respondents met International Headache Society diagnostic criteria specifically for migraine. However, only 32% received an actual diagnosis of migraine. Fifty-nine percent were diagnosed as having “cephalgia” or “headache NOS” (not otherwise specified). All patients had taken nonprescription medications, 24% received opioids, and 7% received a migraine-specific medication; 65% received a “migraine cocktail” comprised of a variable mixture of a nonsteroidal anti-inflammatory agent, a dopamine antagonist, and/or an antihistamine. Forty nine percent had never taken a triptan.

All 57 patients reported that they had to rest or sleep after being discharged, and they were unable to return to normal function. Additionally, 60% of the patients reported either recurrent or persistent headache 24 hours after being discharge from the emergency department.

Blumenthal HJ, Weisz MA, Kelly KM, Mayer RdL, Blonsky J. Treatment of primary headache in the emergency department. Headache. 2003;43(10):1026-1031.

IHS MIGRAINE AND TENSION-

TYPE HEADACHE

IHS MIGRAINE AND TENSION-

TYPE HEADACHE

g ≥5 attacks lasting 4 to 72 hours g ≥2 of the following 4 n Unilateral n Pulsating n Moderate or severe intensity n Aggravation by routine physical activity g ≥1 of the following n Nausea and / or vomiting n Photophobia and phonophobia g Not attributable to another disorder

g ≥5 attacks lasting 4 to 72 hours g ≥2 of the following 4 n Unilateral n Pulsating n Moderate or severe intensity n Aggravation by routine physical activity g ≥1 of the following n Nausea and / or vomiting n Photophobia and phonophobia g Not attributable to another disorder

Migraine Migraine Tension-Tension-typetype

g ≥10 attacks lasting 30 minutes to 7 days g ≥2 of the following 4 n Bilateral n Not pulsating n Mild or moderate intensity n Not aggravated by routine physical activity g No nausea or vomiting g One or neither photophobia or phonophobia g Not attributable to another disorder

g ≥10 attacks lasting 30 minutes to 7 days g ≥2 of the following 4 n Bilateral n Not pulsating n Mild or moderate intensity n Not aggravated by routine physical activity g No nausea or vomiting g One or neither photophobia or phonophobia g Not attributable to another disorder

The major criteria and associated symptoms required for the IHS diagnosis of migraine are so well known as to be almost intuitive for many clinicians. However, these criteria were established to diagnose headaches, not patients. When used

literally, the sensitivity and specificity may be diminished.

Distinguishing between migraine and tension-type headache (TTH) can sometimes be difficult because the conditions have overlapping features, and patients have more than one type of headache. Their ability to ascribe symptoms to a specific headache on recall may be unreliable.

Although TTH is the most common primary headache disorder, it is the least distinctive, most poorly understood, and most frequently mimicked by underlying diseases. In fact, its clinical diagnosis is based chiefly on the absence of the symptoms that characterize migraine.

What we call TTH may be the lower end in a normal distribution of painful episodic headaches. Whether some TTH is simply a mild migraine or a distinct entity is still an area of debate.

Headache Classification Subcommittee of the International Headache Society. The International Classification of Headache Disorders. Cephalalgia. 2004;24(suppl 1):1:160.

MIGRAINE DIAGNOSTIC

CONSIDERATIONS

MIGRAINE DIAGNOSTIC

CONSIDERATIONS

Recurring moderate-to-severe headache is

migraine until proven otherwise

Russell MB, et al. Cephalalgia. 1996. Pryse-Phillips WEM, et al. Can Med Assoc J. 1997.

No single criterion necessary nor sufficient for diagnosis

Up to 1/3 of patients have a neurological aura

IHS criteria do not require GI symptoms

Vomiting occurs in <1/3 of patients

41% of migraine patients report bilateral pain

50% of the time, pain is nonpulsating

Although research demonstrates that some criteria are more predictive of migraine than others, no single criterion is sufficient. Likewise, no single criterion is essential to confirm a diagnosis of migraine. A common misconception is that aura is the telltale sign of migraine. Eighty five percent of migraine patients do not experience aura. Many, but not all, patients have other symptoms that they recognize as premonitory. Common amongst these are: tiredness, stiff neck, craving for sweets, and yawning. Although nausea is common in migraine patients, vomiting occurs much less frequently. Most migraine patients experience nausea with a large proportion of their headaches, vomiting with a few of their headaches, and neither symptom with some of their headaches. Many migraine patients report never having vomited in association with their headaches. Unilateral pain is a common characteristic of migraine and can be a key symptom in making the diagnosis. However, many migraine patients report headaches that begin bilaterally and then settle on one side or headaches that remain bilateral throughout, but nonetheless meet the other criteria for migraine. Similarly, pulsating or throbbing pain is a common characteristic of migraine but just as many migraine patients will report a penetrating, boring, or stabbing pain. Because approximately 80% of migraine patients also have other headaches and may have more than one headache type at the same time, parsing out migraine symptoms can be challenging. Headache specialists widely believe that moderate-to-severe, recurrent headache is migraine until proven otherwise.

Pryse-Phillips WEM, Dodick DW, Edmeads JG, et al. Guidelines for the diagnosis and management of migraine in clinical practice. Can Med Assoc J. 1997;156(9):1273-1287. Russell MB, Rasmussen BK, Fenger K, Olesen J. Migraine without aura and migraine with aura are distinct clinical entities: a study of four hundred and eighty-four male and female migraineurs from the general population. Cephalalgia. 1996;16(4):239-245.

MIGRAINEURS HAVE MORE

THAN ONE TYPE OF HEADACHE

MIGRAINEURS HAVE MORE

THAN ONE TYPE OF HEADACHE

Migraine 70%

Probable Migraine 7%

Tension-type 23%

Lipton RB et al. Headache. 1999.

N=249 patients 1576 headaches

Perhaps one of the challenges migraine patients have is that their headaches present with a host of different signs and symptoms, some of which meet diagnostic criteria for migraine. Other headaches are either tension-type or probable migraine headaches. It is important for patients and physicians to recognize the differences in these headache types so appropriate care is taken regarding treatment. This study evaluated the efficacy of sumatriptan in treating a host of different headache types. Migraineurs with severe disability, as assessed with the Headache Impact Questionnaire score 250 or greater, were enrolled in a randomized, double-blind, placebo-controlled, crossover study.

Patients treated up to 10 headaches, and headache features, recorded prior to treatment, were used to classify each headache using IHS criteria. Two hundred forty-nine migraineurs treated 1576 moderate or severe headaches: migraine (n=1110), migrainous (n=103), and tension-type (n=363). This study documents that patients with a diagnosis of migraine also may experience other headache types.

Lipton RB, Stewart WF, Cady R, Hall C, O'Quinn S, Kuhn T, Gutterman D. 2000 Wolfe Award. Sumatriptan for the range of headaches in migraine sufferers: results of the Spectrum Study. Headache. 2000;40(10):783-791.

MIGRAINE MOST FREQUENTLY

MISDIAGNOSED AS:

MIGRAINE MOST FREQUENTLY

MISDIAGNOSED AS:

Lipton RB et al. Headache. 2001.

42%

32%

0% 10% 20% 30% 40% 50%

Sinus HA

Tension-type HA

The challenges in sorting through the overlapping features in making a migraine diagnosis are illustrated in this chart.

The American Migraine Study II, published in 2001, replicated a survey conducted a decade earlier, questioned 29,727 respondents about their headaches (1). Self- reported symptoms were assessed to determine whether they met the individual’s IHS diagnostic criteria for migraine. In addition to IHS-defined status, self-reported physician diagnosis was determined. Individuals were assigned to self-reported categories of physician diagnosis based solely on their reported diagnosis and whether they met IHS criteria for migraine (1).

Forty-one percent of male and 51% of female respondents reported receiving a physician diagnosis of migraine. This chart shows the percentage of respondents who met the IHS criteria for migraine who reported receiving a diagnosis other than migraine. Thirty-two percent of undiagnosed migraine respondents reported a diagnosis of TTH. Forty-two percent reported a diagnosis of sinus headache (2). The prevalence in the population of TTH is 78%, and sinus headache is 15% (3).

1. Lipton RB, Stewart WF, Diamond S, et al. Prevalence and burden of migraine in the United States: Data from the American Migraine Study II. Headache. 2001;41(7):646-657.
2. Lipton RB, Diamond S, Reed M, et al. Migraine diagnosis and treatment: Results from the American Migraine Study II. Headache. 2001;41(7):638-645.
3. Rasmussen BK, Jensen R, Schroll M, Olesen J. Epidemiology of headache in a general population – a prevalence study. J Clin Epidemiol. 1991;44:1147-1157.

WHY IS MIGRAINE FREQUENTLY

MISTAKEN FOR SINUS HEADACHE?

WHY IS MIGRAINE FREQUENTLY

MISTAKEN FOR SINUS HEADACHE?

g Pain is often located

over the sinuses

g Migraine is

frequently triggered

by weather changes

g Tearing and nasal

congestion common

during attacks

g Sinus medication

may help migraine

Migraine may be confused with sinus headache because in both conditions pain may localize over the frontal sinuses. With migraine, however, this pain is considered to be referred pain from V1 pathways. Patients report that changes in weather trigger headache, and not realizing that weather changes may be a trigger for migraine, they assume such headaches are sinus headaches. Up to 50% of patients also report autonomic symptoms that resemble sinus disease (rhinitis, tearing, and congestion among others). When these symptoms are present, it is assumed that the patient has sinus disease and sinus headache. However, these symptoms are also associated with migraine.

Barbanti P, Fabbrini G, Pesare M, et al. Unilateral cranial autonomic symptoms in migraine. Cephalalgia. 2002;22:256-259.

DIAGNOSING MIGRAINE IN PATIENTS

COMPLAINING OF HEADACHE

DIAGNOSING MIGRAINE IN PATIENTS

COMPLAINING OF HEADACHE

Strongest predictors of migraine diagnosis

Nausea

Are you nauseated or sick to your stomach when you have a headache?

Disability

Has a headache limited your activities for a day or more in the last 3 months?

Photophobia

Does light bother you when you have a headache?

2 out of 3 symptoms: PPV 93%

3 out of 3 symptoms: PPV 98%

Lipton RB et al. Neurology. 2003.

Because migraine is substantially underdiagnosed, a simple, 3-question, self-administered screening tool called ID Migraine™ was developed to help detect patients with unreported headache complaints in the primary care setting. The questionnaire was developed from a 9- item questionnaire that was in turn designed to evaluate patients based on the criteria for diagnosis of migraine established by the IHS. Of the 9 diagnostic screening questions, it was found that a 3-item subset of disability, nausea, and photophobia had the best performance. The sensitivity and specificity of the questionnaire were similar regardless of sex, age, presence of comorbid headaches, or previous diagnoses.

The predictive ability of these 3 sets of symptoms are reflected in patients’ responses to 3 questions:

**_1. Are you nauseated or sick to your stomach when you have a headache?

2. Has a headache limited your activities for a day or more in the last 3 months?_** 3. Does light bother you when you have a headache?

Lipton RB, Dodick D, Sadovsky R, Kolodner K, Endicott J, Hettiarachchi J, Harrison W. A self-administered screener for migraine in primary care: the ID MigraineTM^ validation study. Neurology. 2003;61:375-

AURA: MIMICS AND SECONDARY

CAUSES

AURA: MIMICS AND SECONDARY

CAUSES

TIA

Carotid artery dissection

Venous sinus thrombosis

Vasculitis

Tumor

Simple partial seizure

AVM

Bousser MG, et al. In: Wolff’s Headache And Other Head Pain. 2001 Campbell JK, Sakai F. In: The Headaches. 2000 Silberstein SD, et al. Headache in Clinical Practice. 2002

Hereditary disorder

Demyelinating disease

Vasculitis

HIV

APL

Although the aura of migraine is a benign and reversible phenomenon, a number of pathologic disease states may closely mimic the migraine aura.

Aura may be present without headache. This is usually seen in the elderly, and the differentiation between migraine and other disorders, such as transient cerebral ischemia, becomes difficult. Late age of onset, short duration or evolution of the focal symptoms, and negative rather than positive visual phenomenon, particularly in a patient with vascular risk factors, should raise concern and prompt further investigations for an underlying vascular etiology.

Visual hallucinations of migraine and occipital lobe epilepsy can sometimes be difficult to differentiate. The visual symptoms of both disorders may be elementary negative hallucinations (scotoma, hemianopia) or positive (phosphenes, sparks, or flashes). Perceptive illusions in which objects appear distorted, such as a change in size (macropsia, micropsia), shape (metamorphopsia), or distance may also occur in both migraine and epilepsy. The distinction between epilepsy and migraine in clinical practice is rarely difficult because of the accompanying headache with migraine and the psychic or overt seizure with epilepsy. In cases where distinction is unclear, electroencephalography may be helpful.

Bousser MG, Good J, Kittner SJ, Silberstein SD. Headache associated with vascular disorders. In: Silberstein SD, Lipton RB, Dalessio DJ, eds. Wolff’s Headache And Other Head Pain. 7th ed. New York: Oxford University Press; 2001:349-392. Campbell JK, Sakai F. Diagnosis and differential diagnosis. In: Olesen J, Tfelt-Hansen P, Welch KMA. The Headaches. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:359-363. Silberstein SD, Lipton RB, Goadsby PJ. Headache in Clinical Practice. London, England: Martin Dunitz; 2002.

TIA

Visual loss Abrupt Simultaneous occurrence

Duration <15 minutes Headache uncommon accompaniment

Migraine

Positive visual symptoms Gradual onset / evolution Sequential progression Repetitive attacks of identical nature Flurry of attacks mid-life Duration up to 60 minutes Headache follows ~ 50%

MIGRAINE AURA VS. TIA MIGRAINE AURA VS. TIA

vs.

Fisher CM. Stroke. 1986

One important clinical concern is distinguishing migraine, especially migraine “equivalents” that occur later in life, from cerebrovascular disease. It is difficult to distinguish the transient ischemic attacks from migraine. These are some of the clinical features that can help distinguish between migraine and a TIA.

Fisher CM. Late-life migraine accompaniments — further experience. Stroke. 1986;17:1033-

MEDICATION-OVERUSE

HEADACHE

MEDICATION-OVERUSE

HEADACHE

Diagnostic criteria:

n Intake on ≥10 days per month on a regular basis for

≥3 months (>15 days for simple analgesics)

n Headache has developed or markedly worsened

during overuse

n Headache resolves or reverts to its previous pattern

within 2 months after discontinuation of ergotamine

Applies to:

n Ergotamine, triptan, analgesic, opioid, and

combination medication-overuse headache

Headache Classification Subcommittee of the International Headache Society. Cephalalgia. 2004.

Diagnostic criteria is defined as taking medications 10 or more days per month on a regular basis for 3 or more months. This includes taking 15 days or more per month of simple analgesics. Additionally, headaches develop or are markedly worsened, during overuse with medications. Most headaches will resolve or revert to their previous pattern within 2 months following discontinuation of ergotamine.

Medication overuse headache has been associated with ergotamine, triptan, analgesic, opioid, and combination medication-overuse headaches.

Headache Classification Subcommittee of the International Headache Society. Cephalalgia. 2004;24(suppl 1):1:160.

EVALUATION STRATEGIES EVALUATION STRATEGIES

Red Flags”

“Investigate

the

Atypical

and the

Laboratory testing is not routinely needed in the evaluation of a headache patient. The necessity for and extent to which laboratory tests are obtained will be determined by the clinical suspicion of a secondary headache disorder, for example, temporal arteritis. A practical suggestion in this setting is to appropriately investigate the atypical, as well as the red flags.

Occasionally, depending on the medications prescribed, a pertinent screening baseline laboratory assessment may be necessary, for example, divalproex sodium levels.

Evans RE, Rozen TD, Adelman JU. Neuroimaging and other diagnostic testing in headache. In: Silberstein SD, Lipton RB, Dalessio DJ, eds. Wolff’s Headache And Other Head Pain. 7th ed. New York: Oxford University Press; 2001:27-49.

Campbell JK, Sakai F. Diagnosis and differential diagnosis. In: Olesen J, Tfelt-Hansen P, Welch KMA. The Headaches. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:359-363.