Approach to the Patient with Headache

Amy A. Pruitt

A complaint of headache raises numerous diagnostic possibilities. Fortunately, less than 1% of cases of headache that come to medical attention represent serious intracranial disease. Still, headache poses a diagnostic challenge for the primary physician, who must distinguish between the rare headache that represents a potentially life-threatening process and the vast majority, which are harmless. The primary physician’s most immediate task is to identify efficiently by history and physical examination the occasional patient who requires aggressive workup, including expensive neuroimaging studies. Additional priorities are to provide symptomatic relief and to diagnose the type of headache and its cause systematically while formulating a long-term plan for management.

PATHOPHYSIOLOGY AND CLINICAL PRESENTATION (1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11 and 12)

Headache may originate in either intracranial or extracranial structures, with the mechanisms and presentations of pain depending on the source.

Intracranial Sources

Intracranial sources of pain referable to the head include fibers of the fifth, ninth, and tenth cranial nerves and the upper cervical nerves, the venous sinuses, parts of the dura at the base of the skull, the dural arteries (anterior and middle meningeal), and the large arteries at the base of the brain that give rise to the circle of Willis. Brain parenchyma is not pain sensitive. Postulated mechanisms include (a) traction resulting from direct or indirect displacement of intracranial structures, (b) distention of intracranial arteries, (c) inflammation of pain-sensitive structures, and (d) obstruction of cerebrospinal fluid flow by a mass lesion distorting the brain contents. If the intracranial source of pain is above the tentorium, pain is usually felt in the distribution of the fifth cranial nerve. Pain from a site in the posterior fossa is usually felt in the posterior half of the head, conveyed by the glossopharyngeal and vagus nerves and also by the upper cervical spinal roots.

Mass Lesions

Mass lesions can cause headache by displacing a pain-sensitive structure. About one third of patients with a mass lesion have headache as an early symptom, often with the pain localized to the side of the lesion. Presentations vary widely, with none particularly diagnostic. The headache may be mild or severe, intermittent or persistent, aching, sharp, pressure-like, or even throbbing in quality. Characteristically, the headache remains in the same location but is progressive, increasing in duration and severity over time, in conjunction with changes in mental status or the development of focal neurologic deficits. As intracranial pressure increases, lying down may exacerbate the headache, as may straining at stool, coughing, or bending over, and a more generalized headache may develop. Nocturnal awakening is common but not diagnostic. Projectile vomiting is a late complication.

Subarachnoid Hemorrhage.

This potentially catastrophic source of acute headache due to arterial bleeding may present initially without focal neurologic deficits. In such patients (with no history of trauma) presenting with a headache reaching maximum intensity in less than one hour, Canadian investigators identified several characteristic, predictive features: “thunderclap” onset (instantly peaking), neck pain or stiffness, nuchal rigidity on examination, onset during exertion, witnessed loss of consciousness, and age of 40 or more years.

Brain Tumor and Brain Abscess.

In brain tumor, persistent, localized headache may be the sole initial complaint, unaccompanied by focal neurologic deficits. However, as the condition progresses, neurologic deficits usually ensue. Brain abscess may present as a mass lesion causing headache, especially in its later stages.

Chronic Subdural Hematoma.

Chronic subdural hematoma, another important mass lesion, typically presents in a subtle fashion, with antecedent head trauma followed by a symptomfree interval. The injury may be forgotten, but changes in mental status and, eventually, focal neurologic deficits begin to develop.

Pseudotumor Cerebri.

This condition can mimic the clinical presentation of tumor. Characteristic features include onset of headache most often in an obese young woman and papilledema on funduscopic examination.

Nonmigrainous Cerebrovascular Sources

Ischemic events may be associated with acute headache. The pain most often occurs on the side of the lesion but may be frontal or diffuse. In some instances, the headache is a consequence of the ensuing cerebral edema. Arteriovenous malformation and berry aneurysm are much-feared causes of vascular intracranial headache. Acute rupture of an aneurysm produces a headache of sudden onset that reaches maximum intensity immediately and is often accompanied by meningeal irritation. In the absence of any rupture, 10% to 15% of patients with an arteriovenous malformation may experience a chronic headache characterized by unilateral (always the same side) throbbing pain. Unlike migraine, such headaches are not associated with prodromal or other symptoms. Berry aneurysms are silent until they rupture
unless they are larger than 2 cm, in which case they may present with headache similar to that caused by a mass lesion.

Migraine Headache

Migraine affects about 12% of American adults and is roughly three times as common among women. Incidence peaks between ages 20 and 24 among women and between ages 15 and 19 among men. Cumulative lifetime incidence is an astonishing 43% among American women and 18% among US males. In 50% of cases, onset occurs before age 25 and in three quarters of patients before age 35. Despite the fact that migraine is associated with high rates of disability, most patients with migraine have never had their condition diagnosed by a doctor or treated with prescription medications. Although epidemiologic studies suggest that approximately 38% of migraineurs qualify for daily preventive therapy, only 3% to 13% currently use it. In 2007, the American Migraine Prevalence and Prevention study confirmed the stability of migraine epidemiologic profile over the last 15 years. Family history is present in nearly two thirds of cases, especially in patients who have a history of migraine with aura. Migraine headaches usually begin in childhood or young adult life, although in about 16% of women afflicted by migraine, the headaches first develop at the time of menopause. The condition tends to improve in many women during pregnancy, although oral contraceptives have been known to precipitate migraine or convert migraine without aura into migraine with aura. In roughly one in seven women with migraine, headache occurs only during the first few days of menses, although many women experience an exacerbation at this time. Medical conditions associated with migraine include depression, cardiovascular disease, and roughly twofold increased stroke risk in patients who experience migraine with aura.

Mechanisms.

Migraine is a complex, genetically influenced, chronic brain condition with secondary systemic manifestations. The neurogenic-inflammatory hypothesis explains migraine as a primary neuronal event, with secondary neurotransmittermediated changes in vasculature and blood flow. Neuropeptides act as neurotransmitters at trigeminal nerve branches, precipitating an inflammatory process with vasodilation. Serotonin receptors are believed to be important in mediating these events, which can be triggered by a variety of stimuli (mechanical, electrical, or chemical). The clinical phenomenon of aura is now believed to be due to a slowly spreading band of “cortical depression” or decreased neuronal function.

Classification (Tables 165-1 and 165-2).

Two forms of migraine are recognized: with aura and without aura (replacing the terms classic and common). Both types of migraine are accompanied by nausea and photophobia. The classification system requires that at least two of the following be present to establish a diagnosis of migraine headache: unilateral location, pulsating quality, moderate to severe intensity, and exacerbation by physical activity. At least one of the following must accompany the headache: nausea or vomiting, photophobia, and phonophobia.

TABLE 165-1 International Headache Society Definition of Migraine Without Aura (Common Migraine)

Diagnostic Criteria
	At least five attacks
	Headache lasting 4—72 h (untreated or unsuccessfully treated)
	Headache with at least two of the following characteristics:
		Unilateral location
		Pulsating quality
		Moderate or severe intensity (inhibits or prohibits daily activities)
	Aggravated by walking stairs or similar routine physical activity
	During headache, at least one of the following:
		Nausea and/or vomiting
		Photophobia and phonophobia
History and physical examination do not suggest organic disorder, or such disorder is unrelated to the timing of the migraine attacks.
Adapted from International Classification Committee of Headache Disorders II. Cephalalgia 2004;24(Suppl 1):9-160, with permission. Copyright © 2004, International Headache Society.

TABLE 165-2 International Headache Society Definition of Migraine with Aura (Classic Migraine)

Previously used terms: classic migraine; ophthalmic, hemiparesthetic, hemiplegic, or aphasic migraine
Diagnostic Criteria
	At least two attacks
	At least three of the following four characteristics:
		One or more fully reversible aura symptoms are present, indicating focal cerebral cortical or brainstem dysfunction.
		At least one aura symptom develops gradually over 5 min or more, or two or more symptoms occur in succession.
		No aura symptom lasts >60 min; if more than one aura symptom is present, the accepted duration is proportionally increased.
		Headache follows aura with a free interval of <60 min (it may also begin before or simultaneously with the aura).
Adapted from International Classification Committee of Headache Disorders II.
Cephalalgia 2004;24(Suppl 1):9-160, with permission. Copyright © 2004, International Headache Society.

Precipitants and Complications.

Precipitants of migraine include emotional upset, menstruation, and, in some people, ingestion of tyramine- or tryptophan-rich foods (e.g., ripe cheeses, red wine, chocolate). Headache may occur shortly after or just before a period of psychological stress. Some patients experience a seemingly paradoxical flare-up on weekends or vacations.

One concern is stroke risk. The absolute stroke risk is low, but there is a twofold increased stroke risk confined to patients who have migraine with aura. In women of childbearing age with migraine, the risk of stroke increases with the use of oral contraceptives and is exacerbated by smoking and hypertension. Of some concern is the frequency of white matter lesions (especially in the posterior fossa) noted on brain magnetic resonance imaging (MRI) in persons with migraine, suggesting that migraine may be associated with increased risk of multiple subclinical strokes. However, while confirming increased prevalence of such lesions in women with migraine (but not men), long-term communitybased study finds no evidence of progression or increased risk of cognitive decline.

Clinical Presentation.

Attacks may comprise as many as five phases: prodrome, aura, headache, termination, and postdrome. The prodrome is characterized by lassitude, irritability, difficulty concentrating, and nausea. Patients with aura often report visual phenomena (scintillating scotomata, zigzag patterns, hemianopsia, diplopia), vertigo, aphasia, or even hemiplegia preceding the onset of the headache, which is typically unilateral and throbbing, although it may begin as a dull sensation and take a while to reach maximum intensity. Headache termination usually occurs within 24 hours but sometimes not until after 48 hours. The postdrome phase includes feelings of fatigue, sleepiness, or irritability. Epidemiologic studies suggest that migraineurs suffer a median of about 12 attacks per year. Many have headaches both with and without aura.

Variants.

A number of variants are seen. A relatively uncommon variant is acephalgic migraine, in which a focal neurologic deficit may evolve during an aura but is not succeeded by headache. In rare instances, the symptoms of acephalgic migraine may persist for 1 to 2 days, simulating a stroke. Basilar migraine produces focal symptoms referable to the posterior circulation; it is more common in children. Vertiginous migraine relates to patients who have an aura of dizziness preceding typical migraine attacks.

Meningitis

Infection or hemorrhage produces pain that is acute in onset, severe, generalized, and constant. Symptoms may be particularly intense at the base of the skull and aggravated by forward flexion of the neck or by leg raising in conjunction with knee extension and foot dorsiflexion. Meningitis in young people is often meningococcal or viral in origin. A major independent risk factor for meningococcal disease in such persons is living in a college dormitory. Administration of quadrivalent polysaccharide vaccine to such persons could markedly reduce risk.

Postconcussion Headache

Postconcussion headache occurs when the wrenching and displacement of pain-sensitive structures in the central nervous system during head trauma have been severe enough to cause concussion. Postconcussion syndrome (posttraumatic nervous instability) is a complicated, poorly characterized state manifested by chronic refractory headache, neck pain, nervousness, emotional lability, crying spells, and inability to concentrate. The symptoms are suggestive of an agitated depression following trauma; the syndrome probably represents a variant of tension-type headache. The correlation between severity of symptoms and seriousness of the injury is minimal.

Extracranial Sources

Extracranial sites of headache include the skin, fascia, muscles, and blood vessels of the scalp; the extracranial arteries; mucous membranes of the nasal and perinasal spaces; the external and middle ear; teeth; and muscles of the scalp and facial region. Problems involving the eyes, sinuses, cervical spine, temporal mandibular joints, or cranial nerves can be important sources.

Tension-Type Headache

Tension headache ranks among the leading types of chronic and recurrent headache. More than 90% are bilateral and are often described as a feeling of pressure or a band-like sensation about the head. The pain is dull and steady in most instances, characteristically worsening as the day progresses and sometimes accompanied by occipital and nuchal soreness. The headache may last days, weeks, or even months. Recording of myographic potentials from head and neck muscles reveals vigorous contractions in some but not all patients with this type of headache. Vasoconstriction can also be detected and may account for the migraine-like symptoms (nausea, throbbing pain) experienced by some patients.

Precipitants include anxiety, depression, and situational stress. Patients with underlying psychopathology often describe their headache pain in vivid terms (e.g., “feels like an ax” or “lightning” or “something exploding”), yet they do so without demonstrating any apparent discomfort. So psychologically engaging is the headache that many of these patients are unaware of their underlying emotional problems. Tension-type headaches may also occur secondary to muscle strain from cervical spondylosis or temporomandibular joint (TMJ) disease (see later discussion). It is important to note that the majority of patients who visit physicians with a primary complaint of headache have features suggestive of migraine and not tension headache.

Sinusitis

True sinusitis produces a headache that characteristically is acute in onset and worse on awakening. It gets better on arising, only to worsen again as the day progresses. The patient reports a purulent nasal discharge, and pain and skin sensitivity are predominant over the involved sinus (see Chapter 219). Many patients with other forms of headache (e.g., frontal muscle contraction headaches) mistakenly attribute their problem to “sinusitis” and self-treat with decongestants to no avail. Because the pain of sinusitis is sometimes described as throbbing in quality and can worsen when the patient bends over, it may be mistaken for migraine or the headache of an intracranial mass lesion.

Giant Cell Arteritis

Also referred to as temporal arteritis or cranial arteritis, this disease of older persons (almost all are older than age 50 years) affects medium and large arteries (especially those of the extracranial vasculature) and can cause blindness if it spreads to the ophthalmic artery. The headache may begin as a throbbing discomfort and progress to a dull, aching pain. Some patients describe burning, and others note bouts of lancinating pain. Scalp tenderness (especially when the hair is combed) localized to the involved vessel(s) is characteristic. However, the inflamed artery may not always be tender or palpable, and although the temporal artery is commonly involved, it need not be. Jaw (masticatory muscle) claudication is often part of the clinical picture, and the condition is strongly associated with polymyalgia rheumatica (see Chapter 161).

The most-feared complication is blindness, which occurs when arteritis results in occlusion of the ophthalmic artery, usually about 1 to 2 months after the onset of headache. Diplopia may precede and is predictive of visual impairment (50% risk). Once visual impairment sets in, it progresses quickly over several hours to total visual loss (see Chapter 161).

Temporomandibular Joint Dysfunction

TMJ has received much attention in the lay press as a common, sometimes overlooked cause of chronic refractory headache. Occasionally, the problem is caused by joint changes resulting from malocclusion. However, the problem in most cases is not malocclusion but rather tension-induced jaw clenching and nocturnal teeth grinding (bruxism). Such chronic involuntary oral habits lead to masticator muscle fatigue and spasm. Chronic dull, aching, unilateral discomfort may be described about the jaw, behind the eyes and the ears, and even down the neck into the shoulders. Jaw pain, clicking sounds, and difficulty opening the mouth in the morning are characteristic. Chewing may exacerbate symptoms; locking of the jaw is common. On physical examination, masticatory muscle tenderness, mandibular hypomotility, and joint clicking and deviation on opening are noted. Molar prominences may be flat from chronic grinding of the teeth (see Chapter 225).

Cluster Headache

The pathophysiology of cluster headache is obscure, although extracerebral vasodilation appears to be a component. It occurs predominantly in middle-aged men and is the only type of headache more common in men than in women. Cluster headache is distinguished by its location, timing, and periodicity. In the most typical presentation, seen in more than 50% of cases, patients describe an intense, nonthrobbing, unilateral headache “behind the eye” that is searing, stabbing, or burning and accompanied by ipsilateral lacrimation, nasal stuffiness, and facial flushing. In 20% to 40% of cases, ipsilateral ptosis and miosis are also present. Headache typically begins a few hours after the patient goes to bed and lasts for 30 to 90 minutes. Attacks occur nightly for 2 to 3 months and then disappear, only to return several months to years later. About 10% of these patients have chronic cluster headache, in which attacks occur daily for 1 to 2 years; periodicity is not noted. These headaches are sometimes confused with migraine, being unilateral and severe, though not throbbing and lacking most other features of migraine.

Indomethacin-Responsive Headache

Headaches in this group include chronic paroxysmal hemicrania, ice pick headache, and hemicrania continua. Chronic paroxysmal hemicrania is a rare variant occurring predominantly among women and characterized by a similarly severe unilateral facial pain; unlike cluster, these headaches occur in short spasms of 10 to 20 minutes up to 20 or 30 times per day. Horner syndrome and ipsilateral tearing are often noted. Ice pick headache occurs in patients suffering from migraine, although not exclusively. The headache is brief, sharp, and jabbing. Hemicrania continua differs from the other types in causing continuous unilateral aching discomfort, although it is accentuated by ipsilateral jabbing pain.

Systemic Infection, Fever and Other Vascular Causes

These conditions are among the most common causes of cranial vasodilation and diffuse, throbbing headache. The headache that frequently accompanies a viral syndrome is typical. Numerous metabolic disturbances and drugs may lead to vasodilation and headache. A pounding headache is a prominent symptom of early carbon monoxide poisoning and a common complaint of patients who take nitrates for angina or vasodilators for other conditions.

Hypertension

Moderate to severe hypertension (diastolic pressures >110 mm Hg) sometimes results in occipital headaches. The mechanism of the headache is unknown. The discomfort is worse in the morning and recedes as the day progresses. This headache resolves with correction of the hypertension. It should not be confused with the muscle contraction and psychogenic headaches that account for most of the headaches occurring in hypertensive patients or with the headache caused by the increased intracranial pressure that accompanies malignant hypertension.

Ocular Sources

Headaches are often attributed to eye problems, especially when they are felt about the orbit. Eyestrain is often blamed for headaches, although in most instances, the attribution is incorrect, and refraction fails to improve the problem. However, in an occasional patient, astigmatism can cause difficulty when the eyes are used for close work for prolonged periods. It produces ocular muscle imbalance and sustained contraction of extraocular, frontal, and temporal muscles; aching discomfort about the orbit and the frontotemporal region results. Refraction corrects the problem. Acute glaucoma may produce an orbital headache of sudden onset accompanied by cloudy vision (see Chapter 207).

Cervical Radiculopathy

Headache is commonly the first symptom of cervical radiculopathy. The pain arises during mechanical irritation of an upper cervical root. Findings on radiography of the cervical spine are variable, ranging from normal to spondylotic. The pain is often localized to one side of the occiput or base of the skull, in conjunction with tenderness to palpation. It may start in the neck and, at times, even radiate to the forehead or eye. The discomfort is described as nagging or aching and is aggravated by neck movement. The headache tends to be worse on awakening, perhaps because of unconscious neck motion during sleep. Entrapment of upper cervical nerve roots as they course toward the occiput through irritated nuchal ligaments and muscles is believed responsible.

Occipital neuralgia is a particularly common variant. Patients often describe sudden lancinating pain in the distribution of the greater occipital nerve, which may be precipitated by turning over in bed and relieved by sitting up; the posterior head pain of occipital neuralgia may also present as a dull pressure sensation.

Trigeminal Neuralgia (Tic Douloureux)

Trigeminal neuralgia (tic douloureux) is one of the most severe pain syndromes known. Paroxysms of lancinating facial or cranial pain occur in middle-aged or elderly patients; these may last only a few seconds but can be excruciating and recurrent. The jaw, gums, lips, or maxillary region may be involved. Characteristically, a trigger zone is located within the region of pain (see Chapter 176).

Syndrome of Chronic Daily Headache

Patients presenting with chronic daily headache (CDH) pose a clinical challenge. The International Headache Society defines the CDH syndrome as the presence of headache on more than 15 days per month for greater than 3 months. The entities comprising it are a diverse group and include chronic migraine, chronic tension-type headache, new daily persistent headache, chronic cluster headache, and hemicrania continua. Transformation of episodic migraine to CDH is most often due to medication overuse.

Only gold members can continue reading. Log In or Register to continue