Common Headache Disorders: Diagnosis and Management, Part I
Author: Glen D. Solomon, MD, FACP, Professor and Chair of Medicine, Wright State University Boonshoft School of Medicine, Dayton, OH.
Peer Reviewer: Dara G. Jamieson, MD, Associate Professor of Clinical Neurology, Weill-Cornell Medical College, New York, NY.
Headaches are one of the most common problems of patients presenting to primary care physicians. Workup and management can be both frustrating and demanding. The physician always needs to be wary of serious conditions such as brain tumors and giant cell arteritis, which would be disastrous to miss but are rather uncommon.
Headache is the seventh most common presenting complaint for outpatient physician visits in the United States. The problem of headache generates 18.3 million ambulatory encounters in the United States per year.1
Headaches generally are differentiated into primary (idiopathic) headache syndromes (see Table 1), which include migraine, tension-type, and cluster headache, and secondary headaches caused by underlying disease. This review will focus on the common primary headache syndromes.
Migraine is a syndrome of intermittent, moderate- to severe-intensity headaches lasting from four to 72 hours.2 The headaches typically are unilateral, throbbing in quality, and associated with nausea or vomiting, and sensitivity to light and/or noise. Aura, usually visual with scintillating scotomata or fortification spectra, precedes the headache in about 15-20% of people.
Tension-type headache typically is described as a band-like pressure headache without associated symptoms. The International Headache Society (IHS)2 defines tension-type headache as a bilateral headache having a pressing or tightening quality of mild to moderate severity. Unlike migraine, it is not aggravated by physical activity, nor is it associated with vomiting. By definition, chronic tension-type headache occurs at least 15 days per month for at least 6 months, 2 although in clinical practice, it usually is a daily or almost daily headache.
The clinical presentation of cluster headache is a unilateral headache of excruciating severity, accompanied by certain autonomic phenomena that is more common in men and strikingly periodic in occurrence.2 Cluster headache is marked by cycles of headache lasting one to four months, separated by remissions of six to 24 months. The cluster headache attacks are unilateral and located around the eye, temple, or upper jaw. Associated symptoms may include conjunctival reddening and tearing of the eye, drooping of the eyelid, nasal stuffiness, and rhinorrhea. The attacks generally last from 15 minutes to three hours (average 45 minutes), occur every other day to eight times daily, and often awaken the patient after 90 to 120 minutes of sleep. Ten to 15% of cluster patients suffer with chronic cluster headache that lasts more than one year without remission.
Prevalence of Headache
In order to determine the overall prevalence of headache in the general population, Rasmussen and colleagues3 examined 740 persons randomly chosen to constitute a representative sample of the population of Copenhagen, Denmark. The group was aged 25-64 years old and was representative of the Danish population in terms of sex, age distribution, and marital status. Subjects underwent a structured interview, examination by a neurologist, and laboratory evaluation. Headache disorders were classified according to the International Headache Society (IHS) criteria.2 They reported a lifetime prevalence of headache of 96%, which was significantly higher among women (99%) than among men (93%). Men aged 55-64 had the lowest lifetime and last year prevalence of headache. Headache at the time of examination (point prevalence rate) was twice as common in women as in men.
The overall lifetime prevalence of migraine was 16% (25% among women and 8% among men). The male/female ratio was about 1:3. There were no significant differences in migraine prevalence rates according to age. Of migraine sufferers, 15% had migraine 8-14 days per year, and 9% had it more than 14 days per year. Eighty-five percent of migraineurs reported severe pain intensity.
The lifetime prevalence of tension-type headache was 78% (88% among women and 69% among men). The male/female ratio was about 4:5. Men aged 55-64 had the lowest lifetime prevalence of tension-type headache. Among women, there was a significant decrease in prevalence of tension-type headache with increasing age. Of tension-type headache sufferers, 23% had headache 8-14 days per year, and 36% had it several times per month. Chronic tension-type headache (tension-type headache occurring > 180 days/year) was noted by 3% of the population. Only 1% of tension-type headache patients reported severe pain intensity, while moderate pain was noted by 58%, and mild pain by 41%.
To determine trends in the prevalence of chronic migraine and the impact on disability and utilization of medical care, the National Health Interview Survey4 (NHIS) collected data through personal interviews conducted with a representative sample of the U.S. population to determine the prevalence of migraine. This study compared migraine prevalence for 1980 and 1989. The NHIS study showed that more than 80% of female and 70% of male migraine sufferers had at least one physician contact per year because of migraine headaches. Eight percent of female and 7% of male migraine patients were hospitalized at least once per year for migraine. Long-term limitations in functional capacity due to migraines were reported by 3% of females and 4% of males.
"Chronic daily headache," a term not included in the ICHD classification, encompasses a group of headache disorders lasting longer than 6 months and marked by a duration of four hours or more for at least half of the days of the month.5 The most common daily headache syndromes include chronic tension-type headache with or without medication overuse, chronic migraine, or medication overuse headache in patients with frequent migraine attacks. Factors associated with the development of chronic daily headache were higher baseline headache frequency, obesity, arthritis, diabetes, and snoring.6
Population-based studies in the United States, Europe, and Asia report that approximately 4% of the general population suffers from chronic daily headache.7,8,9,10,11,12 In addition, 0.5% of the population has severe headache on a daily basis.13
Population-based studies report that the most common cause of chronic daily headache is chronic tension-type headache (1.4-2.7%), followed by chronic migraine (1.0-1.7%).14 In tertiary headache centers, the most common cause of chronic daily headache is chronic migraine. Studies report that chronic daily headache is roughly twice as prevalent in women as in men.14 The average age at onset of chronic daily headache is in the thirties. The prevalence of chronic daily headache appears consistent across age groups from childhood and adolescence, adults, and the elderly.14 This is in contrast to migraine, which decreases in prevalence after the sixth decade of life.
Evaluation of the Headache Patient
Headache is one of the most common pain complaints of humankind. Headache may be categorized simply as a primary headache disorder or secondary (organic) headache. Secondary headaches present as a symptom of an underlying disease (i.e., giant cell arteritis, meningitis), while benign headaches are classified further based on clinical symptomatology, i.e., temporal patterns and accompanying symptoms, into syndromes such as migraine, tension-type headache, and cluster headache.
The Headache History
The headache history is the key to determining whether a headache is migraine, cluster, tension-type, or whether it represents a symptom of underlying disease. Most often a careful and complete history will provide a presumptive diagnosis, which then can be verified by physical examination and, when necessary, confirmatory laboratory or radiographic studies.15
Evaluating factors such as age of onset, temporal pattern, quality and location of pain, and trigger factors usually allows the physician to diagnose the headache problem and initiate therapy. (See Table 2.)
Duration. The duration of the headache problem often is a key indicator to probable underlying cause. Severe headache of sudden onset, especially if associated with focal neurological signs or changes in level of consciousness, suggests serious illness such as subarachnoid hemorrhage (SAH) or meningitis. Recurrent intermittent headaches occurring for many years more likely represent migraine headaches. A long history of daily headaches without associated symptoms is suggestive of chronic tension-type headache.
The patient's initial migraine headache, unless preceded by a characteristic aura, may be confused with serious neurological problems such as meningitis or subarachnoid hemorrhage.
Among the most difficult headaches to interpret are those developing over weeks or months. These may be benign or may arise from conditions as diverse as sinusitis, ocular disease, subdural hematoma, mass lesion, or in the patient older than 60 years giant cell arteritis.
Timing. After establishing the frequency and duration of the headache, the timing with respect to other physiological events can be helpful in the diagnosis of recurrent headache. One should inquire as to the time of day the headache occurs and its relationship to puberty, menses, pregnancy, menopause, or the use of hormones.
The initial onset of migraine in women often is during puberty. Migraine may resolve following menopause. It may occur irregularly for months to years or it may follow a regular pattern of occurring with menses. An acute migraine attack can last from four to 72 hours, with headache-free intervals between attacks.
Episodic cluster headache follows a pattern of cyclic bouts of attacks lasting from two weeks to several months. These bouts are separated by headache-free periods lasting from months to years. During a cluster cycle, severe headaches lasting from 15 minutes to three hours may occur from one to four times a day, often awakening the patient from sleep at night.
The duration of the cluster headache attack distinguishes it from trigeminal neuralgia, which presents with recurrent jabs of pain lasting less than a minute. Trigeminal Autonomic Cephalgias or TACs (see Table 3), such as chronic paroxysmal hemicrania, show a pain pattern similar to cluster headache, but attacks are more frequent and predominantly occur during the day.2,16
Chronic tension-type headaches show no periodicity and have few prolonged headache-free intervals. The patient typically describes a daily or almost daily headache.
Location. Location of the head pain can aid in the diagnosis of cluster headache or trigeminal neuralgia, but it is less specific in migraine and tension-type headache. Although migraine is unilateral two-thirds of the time, it is bilateral one-third of the time. Chronic tension-type headache usually is bilateral but may be unilateral. Cluster headache and trigeminal neuralgia are almost always unilateral. Migraine usually alternates sides with different attacks but may be predominantly unilateral throughout life. Cluster headache is invariably unilateral and affects only one side during a series of attacks. Trigeminal neuralgia usually occurs in the V2 or V3 distribution, while TACs usually occur in the V1 distribution.17
Quality. Migraine usually is throbbing or pulsatile, whereas a constant pressure suggests tension-type headache, and deep, boring, intense pain points to cluster headache. Trigeminal neuralgia is marked by short, intense, shock-like jabs.
The intensity of pain in cluster headache and trigeminal neuralgia is invariably described as excruciating so that the cluster headache patient usually cannot remain still. The migraine sufferer, by contrast, often chooses to rest in a dark, quiet room.
Associated Symptoms. If the patient tells of an aura or warning signs, this generally means migraine with aura (classic migraine). Visual or neurological symptoms commonly precede the headache by four to 60 minutes (typically 20 minutes). Premonitory symptoms, which can include euphoria, fatigue, yawning, or craving for sweets, may occur 12-24 hours before an attack.
Associated symptoms that may accompany migraine include photophobia, phonophobia, osmophobia, anorexia, nausea, vomiting, and focal neurological signs. Symptoms seen with cluster headache and other TACs include partial Horner's syndrome, constricted pupils, injected conjunctiva, and unilateral lacrimation and rhinorrhea.
Neck stiffness or other signs of meningeal irritation can signal meningitis or subarachnoid hemorrhage. A mass lesion, hydrocephalus, or encephalitis may be suggested by decreased level of consciousness or obtundation. Seizures can reflect cortical irritation resulting from a mass lesion, such as a tumor or arteriovenous malformation. Fever and sweating may suggest an infectious process.
Precipitating Factors. Fatigue, particularly loss of sleep, may trigger either migraine or tension-type headache. Stress may exacerbate tension-type headache, whereas migraine may occur after stressful events, often on weekends or vacations. Migraine sufferers may associate their headaches with menses, missing meals, or consuming foods such as red wine, chocolate, or aged cheese. Alcohol may trigger a cluster attack during a series but will have no effect during a quiescent period. Weather changes can be associated with migraine. Commonly associated with chronic tension-type headache are symptoms of depression, such as sleep and appetite disturbances.
One also should assess possible exposure to occupational toxins, chemicals, or infectious agents. Carbon monoxide poisoning, for example, often manifests as headache. Certain chemicals such as nitrates induce withdrawal and reintroduction headache. Exposure to infectious agents in immunosuppressed or AIDS patients may induce meningitis unaccompanied by classic fever and stiff neck.
Past History. Migraine is a familial disorder, with a positive family history in two-thirds of cases. Cluster headache is familial in only about 3% of patients.18 In tension-type headache, a family history of depression or alcohol abuse is common.
The patient's medical-surgical history and history of current and previous medications can aid in diagnosis. Head trauma, for instance, may suggest subdural hematoma or skull fracture. Certain medications can trigger the onset of headache or exacerbate headache in patients with an underlying headache disorder.19,20 Medication-induced headache has been reported commonly with the following medications: indomethacin (Indocin), nifedipine (Procardia, Adalat), cimetidine (Tagamet), atenolol (Tenormin), trimethoprim-sulfamethoxazole (Bactrim, Septra), nitroglycerin, isosorbide dinitrate (Isordil), ranitidine (Zantac), isotretinoin (Retin-A), captopril (Capoten), piroxicam (Feldene), granisetron (Kytril), erythropoetin (Epogen), metoprolol (Lopressor, Toprol), and diclofenac (Voltaren). Medications that may aggravate existing migraine include vitamin A, its retinoic acid derivatives, and hormonal therapy, such as oral contraceptives, clomiphene, and postmenopausal estrogens. Migraine and cluster headaches may be exacerbated by vasodilators such as nitrates, hydralazine (Apresoline), minoxidil (Loniten), nifedipine (Procardia, Adalat), and prazocin (Minipress).
Reserpine can cause depression, migraine, and tension-type headaches. Indomethacin, while useful in treating some TACs such as paroxysmal hemicrania or hemicrania continua, can cause a generalized headache. Frequent or chronic use of some prescription and over-the-counter medications used to treat headache, including opioids, barbiturates, caffeine, triptans, and ergots, can lead to medication overuse (rebound) or withdrawal headaches.21
After evaluating the headache history, the physician should perform a targeted physical examination. This should include a mental status exam (often performed as part of obtaining the history), blood pressure and pulse measurement, examination of the cranial nerves, fundoscopic exam, palpation of the head and neck, evaluation of motor and balance, and palpation of peripheral pulses (particularly if vasoconstrictor medications are to be prescribed). (See Table 4.)
Diagnostic testing of the headache patient should be based on the results of the history and physical examination. In a patient with a typical headache history of several years' duration and a normal neurological examination, no further evaluation is usually needed. All patients older than 60 with new-onset headache or change in their headache pattern should have a sedimentation rate or C-reactive protein measurement to evaluate for giant cell arteritis. If elevated, a temporal artery biopsy may be obtained to confirm the diagnosis.
Laboratory screening with complete blood count, urinalysis, and chemistry profile adds little diagnostic information. These studies may help to rule out diseases that present with headache if the history and/or examination suggest secondary headache.
Several commonly ordered tests have little or no value in the headache evaluation.22 Electroencephalography (EEG) may be abnormal in some migraine patients, but EEG changes are neither specific for nor diagnostic of migraine. As a screening test to localize organic lesions, EEG has been supplanted by more specific CT and MR imaging. Evoked potentials (visual, auditory, and somatosensory) fail to show specific findings in migraine. Like EEG, evoked potentials have no utility as a screening test for headache. Cervical spine X-rays rarely are useful in the diagnosis and management of headache patients.
Neuroradiology has little role in the diagnosis of headache beyond ruling out occult lesions such as neoplasm, hemorrhage, vascular malformations, brain abscesses, hydrocephalus, or congenital malformations (i.e., Arnold-Chiari malformations). MRI and CT will not pick up other organic etiologies of headache, such as idiopathic intracranial hypertension (pseudotumor cerebri), glaucoma or eye disease, and metabolic or toxic causes of headache. It is critical that the physician obtain a complete history and examination and not rely solely on the MRI or CT to eliminate organic causes of headache.
Most patients suffering with newly developed, acute onset, severe headaches should undergo imaging with CT or MRI to rule out the organic causes listed above. (See Table 6.) Because organic causes of headache are rare (estimated at less than 1% in headache clinics), these tests generally will be unrevealing. The benefits of a normal CT or MRI in reassuring the patient and doctor should be considered, however. Some patients (and physicians) may be unwilling to embark on a course of therapy for a benign headache disorder without the reassurance of a normal scan.
A controversy in headache management is the issue of repeated evaluations to look for organic etiologies of headache. Most physicians agree that repeating the pertinent headache history and the targeted physical examination is important to pick up clues for new diagnoses and diagnoses missed at initial evaluation. The issue of obtaining additional CT or MRI scans is unsettled. It generally is prudent to repeat neuroradiographic imaging in a patient whose condition is deteriorating, or where history or physical examination has revealed new neurological abnormalities. There appears to be little yield to obtaining repeated studies in patients whose headache course is stable, even if the patient is not responding well to treatment.
In summary, the appropriate screening for the outpatient with recurrent headaches includes: a complete headache history; both physical and neurologic examination; lab tests only if indicated from history and physical; CT or MRI, if the headaches are of recent onset, if indicated by troubling neurologic symptoms in the history, or if associated with abnormalities on the neurologic examination.
In primary care, most headaches are not caused by underlying disease. It is important to recognize, however, that headache can be the presenting symptom of several diseases. Fever, regardless of etiology, is probably the most common medical problem that causes headache. Less common causes include pheochromocytoma, chronic renal failure, hyperthyroidism, and malignant hypertension.21 Rheumatologic diseases may have headache as an early manifestation. Headache is common in systemic lupus erythematosus, polyarteritis nodosa, and giant cell arteritis. About two-thirds of patients with fibromyalgia report headache, usually tension-type headache. Many types of vasculitis can also present with headache.21 Headache upon awakening may be the initial symptom of sleep apnea syndrome. The headache often will improve as the day progresses. Associated symptoms include snoring, daytime somnolence, hypertension, and arrhythmias.21
Once a primary (benign) headache diagnosis is made, the major role of the physician is to initiate a therapeutic plan. Important goals of the therapeutic plan are to limit disability caused by headache and to prevent the development of analgesic overuse headache.
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