Chapter 20. Common Headache Syndromes

Headache is one of the most common painful conditions for which patients consult physicians. Surveys indicate that in any given year, more than 90% of American adults will suffer some kind of headache or head pain.1 Fortunately, very few headaches are caused by serious organic conditions, and most headaches are actually migraine or tension-type headache. The first step in treating a patient with headache is to establish an accurate diagnosis, and for diagnostic purposes headaches are divided into primary and secondary headache disorders. Secondary headaches have an underlying cause, such as infection, eye disease, tumor, aneurysm, meningitis, and so forth. Primary headache disorders are benign and tend to recur. These headaches are caused by conditions for which the true basis has not yet been established, but altered brain serotonin chemistry clearly plays a role (see Chapter 19 for a discussion of the biology of primary headaches). Chapter 17 outlines diagnostic criteria for migraine, tension-type headache, and cluster headache, the three most common headache syndromes. In this chapter, we comment on differential diagnosis, diagnostic testing, and management of patients with these common primary headache disorders.

The two most common patterns of migraine are migraine with aura, formerly called classic migraine, and migraine without aura, or common migraine. Approximately 18% of women and 6% of men in the United States are plagued by migraine, and 15% will experience an aura with some of their migraine attacks.1

Clinical Features and Differential Diagnosis

Blau has described five phases of migraine that are not universally present and may variably occur during different attacks in the same individual.2 The first phase, or prodrome, occurs in 40% to 60% of migraineurs. It consists of altered mood, irritability, depression or euphoria, fatigue, yawning, excessive sleepiness, craving for chocolate, or other vegetative symptoms, all of which suggest origin of these symptoms in the hypothalamus, perhaps as a result of excessive dopamine stimulation. These symptoms usually precede the headache phase of the migraine attack by several hours or even days, and experience teaches the patient or observant family that the migraine attack has begun.

The second phase of migraine is the aura, consisting of either visual or sensory phenomena (the two most common aura symptoms) or motor weakness, incoordination, or dysphasic symptoms, such as word-finding difficulty. The aura symptoms usually precede the headache phase of the migraine attack, but occasionally occur simultaneously. Sometimes, two aura symptoms occur in the attack, usually visual and sensory symptoms that may occur simultaneously or consecutively. The aura symptoms appear gradually over 5 to 20 minutes and usually subside just before the headache begins.

When examining a patient who has recently experienced only one or two such attacks for the first time, the clinician must determine whether these focal neurologic symptoms represent migrainous aura or manifestations of transient ischemic attack (TIA) or even a focal sensory seizure. Passage of time, repeated identical attacks, and diagnostic testing may be required to obtain certainty, but certain features are more typical of migraine aura. Firstly, visual and sensory symptoms of TIA and seizures usually develop more rapidly than the gradual progression of an aura over 5 to 20 minutes. Secondly, migraine aura is characterized by a combination of negative and positive symptoms; the migraineur experiences a visual scotoma or hole in the vision (negative symptoms) and dazzling, glimmering, scintillating lights (positive phenomena). TIA, such as amaurosis fugax or hemianopic scotoma, usually manifests as a black or blank negative visual loss.

Sometimes, especially in the elderly, the visual aura occurs repeatedly without any headache. C.M. Fisher described these features as “late life migraine accompaniments.”3 Sometimes these elderly patients experienced more typical migraine in youth, with the migraine subsiding for many years only to recur as migraine aura without headache in later life. In this setting, the clinician may be more secure in the diagnosis. However, these late life migraine accompaniments often develop with no previous history of migraine. Patients with new late-life migraine symptoms must be carefully evaluated to rule out cerebrovascular disease, structural hemispheric disease, or even retinal detachments.

The sensory auras of migraine usually consist of numbness (negative sensory symptoms) and positive symptoms of tingling or paresthesia. Again, these sensory symptoms usually progress over 5 to 20 minutes and are followed by the headache phase, which is the third phase of the migraine attack, and usually the most dramatic. It is the headache phase of migraine for which most patients consult physicians.

Chapter 17 outlines the International Headache Society (IHS) system for diagnosing migraine, but it is important to remember that these strict criteria were designed chiefly for purposes of finding a uniform population of migraine patients to be entered into investigational drug trials. The experienced clinician uses the IHS criteria as a guide but is not rigidly bound by them. Furthermore, both migraine and tension-type headache are very common, and when patients report symptoms of both, there may be overlapping features that make it difficult to tell where one ends and the other begins. In fact, the IHS system of classification is being revised and will be more accurate and complete.

Often, the patient does not initially describe the headache characteristics well, and the skillful clinician must take the time to extract important but subtle historical points. For example, some patients spontaneously report only pressure-type occipital pain, but by digging a little deeper, the examiner can help the patient recall that the worst headaches, occurring infrequently, build to an intense throbbing and radiate to one periorbital region. Patients often report that, because they are unsure at the outset whether the headache will build to severe intensity, they fail to take acute-care medication early and, consequently, obtain less effective relief than if they had treated the migraine earlier in the attack.

The headache of migraine is unilateral in 60% of cases and usually alternates sides from one attack to the next.4 Often, patients state that the headache is always on one side but when pressed recall that rarely, perhaps 10% of the time, the headache occurs on the opposite side. This alternating hemicrania, however infrequent, makes the clinician more secure in the diagnosis of migraine. Some migraineurs report that the headache is bilateral, but worse on one side. The headache usually builds over a period of 30 minutes to several hours but may occur with sudden intensity. Although the pain of migraine is usually moderate to severe, many patients report milder headaches, which they refer to as “sinus headaches” or “regular headaches” and which, in reality, are milder migraine attacks. Similar benign recurring headaches are much more likely to be migraine or tension-type headache than sinusitis. Sometimes radiography is needed to settle the issue, and computed tomographic (CT) scanning of the sinuses is superior to sinus x-rays in diagnosing acute sinusitis. Many patients are test oriented and anxious to know what the x-ray showed. They may fail to appreciate the knowledgeable opinion of an experienced clinician in making a diagnosis based on a thorough history and examination.

To be sure, clinicians must always be aware of so-called migraine mimics—paroxysmal headaches caused by arteriovenous malformations, pheochromocytoma, repeated exposure to carbon monoxide, transient increased spinal fluid pressure resulting from a colloid cyst of the third ventricle, adult-onset of headaches caused by type II Arnold-Chiari malformations, or other structural brain disease. In the elderly, temporal arteritis must always be considered. Primary central nervous system angiitis may manifest as frequent headaches before other symptoms, such as encephalopathy, seizures, or infarctions, occur as a result of the vasculitis.

The following danger signals warn the clinician that a headache may be more serious than a migraine1:

• 1. Headache that is changing or different from previous headaches may herald a brain tumor superimposed on a long-standing primary headache disorder, such as migraine or tension-type headache.
• 2. Headache with progressive worsening over 24 hours or several days suggests a mass lesion or infectious disease such as meningitis, abscess, subdural or intracerebral hematoma, or vasculitis.
• 3. Headache precipitated by exertion, bending over, coughing, or sneezing may result from transient blockage of cerebrospinal fluid (CSF) flow or increased intracranial pressure.
• 4. Sudden onset of headache during exercise or sexual activity can occur with subarachnoid hemorrhage or with benign exertional headache.
• 5. Vomiting may result from a brain tumor or other mass lesion with increased intracranial pressure.
• 6. Early morning headache can occur with obstructive sleep apnea and hypertension.
• 7. Any abnormal physical or neurologic finding must be considered suspect: fever, stiff neck, rash, lymphadenopathy, scalp tenderness, altered sensorium, or focal neurologic signs. Any patient who presents with his or her “first or worst headache” is cause for alarm.

The young, healthy patient with a textbook history of migraine and a normal examination seldom requires diagnostic investigation. If, however, the patient fails to respond as expected to treatment efforts, diagnostic testing may be wise.

Unsuspected granulomatous inflammations, such as sarcoidosis, meningeal malignancy, and cryptococcal, tuberculous, or Lyme meningitis, can be diagnosed only by CSF examination. Elevated opening spinal fluid pressure may confirm the diagnosis of pseudotumor cerebri.

For structural brain lesions, magnetic resonance imaging (MRI) is much more sensitive than CT scanning and is always preferable unless bone windows are desired. CT scanning is more sensitive for demonstrating subarachnoid hemorrhage in the first 24 hours, whereas MRI becomes more sensitive after 48 hours. Unenhanced CT scans made within 24 hours of subarachnoid hemorrhage demonstrate blood in 90% of cases, but in only 70% of cases after 5 days.5 If a small subarachnoid hemorrhage, a so-called sentinel bleed, is being considered, CSF examination is essential even when results of the CT are normal. The presence of fresh blood or xanthochromia should prompt angiography. Cerebral angiography is also required if primary central nervous system granulomatous angiitis is suspected. Magnetic resonance angiography is sensitive for identifying unruptured aneurysms as small as 3 to 4 mm, and angiography would be definitive.5

There is a high familial incidence of aneurysms; unsuspected asymptomatic intracranial aneurysms were found in 9% of 396 persons having a first-degree relative with an aneurysm.5 A careful family history is, therefore, important if a warning leak is suspected.

CT scanning of the sinuses is more sensitive than sinus x-rays or MRI. Clinicians must not omit dental disease or jaw dysfunction as a cause of head or facial pain, or localized eye disease, such as glaucoma, which can cause unilateral orbital pain. Cervical spine disease or lesions at the foramen magnum may cause suboccipital pain, and plain x-rays or imaging may be considered.

Certain medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs), estrogen, progestins, selective serotonin reuptake inhibitors (SSRIs), or certain calcium channel blockers, may cause headache. Because these drugs are often used to treat headaches, sorting out the diagnosis may be difficult.

Blau’s fourth phase of migraine is the headache termination. Sleep, even a brief nap of 1 or 2 hours, is the most common natural method of resolution, but biofeedback and relaxation exercises may also be beneficial. Today, pharmacologic treatment is the most common medical treatment to terminate an acute migraine attack.

The fifth phase of migraine, the postdrome, was reported by 94% of Blau’s patients, but these symptoms have not been widely studied. Postdrome symptoms may last about 24 hours and range from feeling drained or exhausted to an unusual sense of elation or euphoria.

Management

The first step in treating the patient with migraine, or any medical condition, is to establish an accurate diagnosis. The diagnosis must be conveyed to the patient who, very often, is fearful of a tumor or aneurysm, or may erroneously believe he or she has a chronic sinus or psychiatric condition. Many of these patients have been discouraged in the past by physicians who have ignored their complaints of headache. Headache is a common complaint, and many busy physicians are reluctant to take the time required to obtain an adequate headache history, or they may view headache as being a result of nervousness or stress. The busy physician may choose to direct the brief office visit at management of hypertension, diabetes, or arthritis. Headache treatment begins with educating patients about the nature of migraine and reassuring them that this is a biologic disorder caused by altered brain biochemistry with secondary vascular changes, and usually a hereditary predisposition. Patients often are relieved that at last they have found a physician who is knowledgeable about headaches and who expresses an interest in helping them. Sometimes it is helpful to have the spouse present and explain to the couple that headaches, and migraine in particular, are often provoked by hormonal changes, certain food triggers, missing meals, irregular sleep patterns, travel, or specific environmental changes. Visual and sensory stimuli, such as bright lights, excessive noise, cigarette smoking, certain perfumes or smells, or other stimuli, may act as migraine triggers. Some medications, both over-the-counter and prescription drugs, can precipitate headache (Table 20-1). There is also an underlying predisposition for people with migraine to have a biochemical comorbidity of depression or anxiety.

In a specialty headache practice, there is usually a nurse or physician’s assistant who instructs the patient to keep a headache calendar, watch for repeated food or environmental triggers, and record the amount of medication taken and response to treatment. Having this accurate record enables the physician to alter pharmacologic treatment depending on the response. The power of the written word cannot be overemphasized, and publications are available that describe and explain many of these issues for the migraineur. Information is available free of charge from the American Council for Headache Education (ACHE) (by phone at 1-800-255-ACHE) and the National Headache Foundation (NHF), (1-800-843-2256).

Some headache patients express a desire to be treated without “drugs” or prefer to take “natural” substances, such as herbal or vitamin supplements. Biofeedback training can be beneficial in conjunction with, or without, medication. Biofeedback training teaches relaxation skills as part of overall headache management. Patients learn to be aware of skeletal muscle status and how to relax general and specific muscle contraction and tension; they learn breathing techniques and how to enhance blood flow to the peripheral vessels, producing hand warming. With practice, patients can alter autonomic nervous function to produce measurable temperature changes in the hands. These exercises may reduce afferent sensory volleys from peripheral muscular pain and modulate sensory impulses ascending through cervical segments into the trigeminal nerve complex in the brainstem.

Cognitive therapy using behavioral modification can help patients deal with the headache condition in a positive way. If the patient spontaneously asks about stress or if he or she recognizes anxiety or depression as significant problems, consultation with a behavioral psychologist may be very helpful.

Physical therapy with heat or cold applications, ultrasound, myofascial release, and massage therapy are beneficial for some patients. Attention to nutrition and observing for food triggers, especially alcohol, are important. Nutrasweet (Equal, aspartame) has been reported to trigger migraine, and one of the authors has observed this in patients who chew gum containing aspartame.6 Correcting irregular eating and sleep habits may be beneficial.

Some of these measures, such as keeping an accurate headache calendar, practicing biofeedback exercises, and paying attention to diet, have the added benefit of insisting that the patient play an active role in the treatment program. Many patients express a sense of empowerment and satisfaction that they are contributing to management of the headache condition and gaining more control over their lives and the condition.

Pharmacotherapy

Pharmacotherapy of migraine may be divided into three types: (1) treatment with nonspecific analgesics; (2) acute care treatment with drugs that have specific pharmacologic affinity to bind to certain serotonin receptors and alter the neurochemical, inflammatory, and vascular process of migraine; and (3) the daily use of preventive medications.

Common analgesics, such as aspirin, acetaminophen, or NSAIDs (e.g., naproxen sodium), often provide relief to patients with mild migraine headaches, especially if taken early in the attack. A double-blind placebo-controlled study found Excedrin Extra-Strength was significantly superior to placebo in treating mild to moderate migraine headaches.7 Combination analgesics, such as Midrin, Fioricet, and Fiorinal, also may be beneficial in migraine if taken early. These compounds have the potential to cause analgesic rebound headache (see later discussion) if taken too often, such as more than three times a week, but they can be very helpful and are safe to take for migraine attacks that occur three or four times a month. Analgesic rebound is more likely related to frequent dosing of these drugs, rather than to the total number of doses that may be taken safely in a week.

Treatment of acute migraine attacks was revolutionized in the United States in 1993 with the introduction of sumatriptan and, in 1998, by other triptans—zolmitriptan, naratriptan, and rizatriptan. Dihydroergotamine has been available since 1945 as an injection, but was not widely used until 15 years ago; a nasal spray formulation, Migranal NS, was introduced in 1997.

All of these drugs are effective because of their specific pharmacologic affinity to bind to certain serotonin (5-HT) receptors and interrupt the neurochemical, inflammatory, and vascular changes that occur in migraine. There are differences in lipophilicity of some of the triptans, with variable ability to cross the blood-brain barrier, but whether any central action contributes to meaningful migraine relief remains uncertain. The slight molecular differences of each triptan confer different pharmacologic properties, such as bioavailability, time to peak concentration, onset of action, metabolic half-life, and excretion, among others (Table 20-2). Sumatriptan, zolmitriptan, and rizatriptan are metabolized by the enzyme monoamine oxidase-A (MAO-A) and, therefore, these drugs are contraindicated in patients taking MAO inhibitors. Naratriptan and dihydroergotamine are not so contraindicated.

Triptan metabolism may be inhibited by cimetidine, birth control pills, and propranolol, resulting in higher blood levels and, in turn, increasing the risk of adverse effects. All the triptan drugs share similar adverse effect profiles. Most effects are mild and transient; however, the primary concern with this class of drugs, as well as with dihydroergotamine, is that these compounds can cause coronary vasoconstriction. A few serious and life-threatening cardiac events have been reported in patients using triptans, and caution should be exercised in prescribing any of the triptans or dihydroergotamine for patients with cardiac risk factors. In vitro studies of isolated human coronary artery segments demonstrate that these drugs are unlikely to cause myocardial ischemia at therapeutic concentrations in healthy subjects8; they are safe for young, otherwise healthy patients with migraine. The triptans and dihydroergotamine have improved the lives of people formerly disabled and suffering several times a month from migraine pain. Because of the pharmacologic differences, one triptan may be very helpful to a patient even if treatment with another triptan has failed.

Rescue treatment refers to using potent opioid analgesia or sedation for an acute attack when specific acute care treatment for migraine fails. Markley provides a thorough review of the appropriate use of opioid analgesics and recommends sound guidelines for the use of these drugs.9 We agree that it is unfair and unethical to deny patients with intractable pain effective analgesia; however, clinicians must also assume the responsibility to monitor the patient’s response and use of these drugs. Once again, we emphasize the importance of having the patient keep an accurate headache calendar, recording the number and severity of headaches and number of and response to acute care medications, including opioids.

Preventive medications may be taken daily by migraineurs who suffer frequent attacks (e.g., three or four times a month or more) or by those who become disabled for 48 to 72 hours even once or twice a month. Only the patient can determine if he or she wishes to take daily preventive medication. Some patients express concerns about side effects or habituation, and clinicians must always monitor continuous medication use in young women who might become pregnant, and caution them of potential harm to the fetus. Patients who are reluctant to take daily medication should be reassured that the drug can be discontinued if they experience any adverse reactions or if their migraine symptoms are not reduced after a trial period. We explain that we begin with a low dose, which can gradually be increased, but at least 4 to 6 weeks of treatment should occur before deciding on effectiveness. The most commonly used preventive medications are listed in Table 20-3. The exact mechanism of action of these medications is not always known, but most have in common the pharmacologic affinity to bind to and downregulate the 5-HT2 family of serotonin receptors.

Choice of preventive medicine should be determined by consideration of the patient’s overall medical condition and any comorbid illnesses. For example, a beta blocker would be a good choice if the patient has mild untreated hypertension, has coexisting essential tremor, or is nervous and excitable. On the other hand, beta blockers should be avoided if the patient has asthma or depression or is taking other vasoactive antihypertensive drugs. Tricyclic antidepressants might be especially helpful for patients who have sleep disturbance, depression, or loss of appetite. Divalproex sodium might be the drug of choice for migraine patients who have obsessive-compulsive traits or are bipolar or who have had seizures in the past, but this drug should be avoided if the patient has known liver disease.

Cluster headache is so distinctive a condition that once the clinician is aware of the characteristic features, there seldom is any difficulty in making the diagnosis. Of all the headache disorders, cluster headache pain is probably the most excruciating and disabling. The reference to cluster in the name of this disorder derives from two characteristics of the condition: firstly, the number of headache attacks can occur in a bunch up to eight times per day; and secondly, the frequent headache attacks are clustered, or grouped, over a defined period of several weeks or months, followed by long headache-free intervals for months or years. Another hallmark of cluster headache is its periodicity; hence the British term for this condition, periodic migrainous neuralgia. These headaches tend to occur on a regular schedule, often at night, awakening the patient from a sound sleep at precisely the same time every night, seldom deviating by even 5 minutes. Furthermore, the attacks usually have a seasonal periodicity, occurring at the same time every year, often in late autumn or springtime. This may be related to long and short photoperiods of available light, which occur around June 21 and December 21.

The onset of cluster headache is almost immediate, with rapidly escalating intensity. There is no throbbing quality, as in migraine, but instead a deep boring or searing pain, which is often described as being “like a red-hot poker” or squeezing of the eyeball. This intense pain is usually deep in the eye, occasionally in the temple. The duration of cluster headache is briefer than that of migraine, lasting from 15 to 180 minutes, most often 45 to 90 minutes. Unlike migraine, nausea, vomiting, and visual symptoms are absent, despite the orbital location. The associated symptoms of cluster headache are very different from those of migraine and are quite characteristic of each patient’s attack. These symptoms, caused by autonomic dysfunction, include ipsilateral oculorrhea, nasal congestion, scleral injection, ptosis, miosis, and forehead sweating. Behavior during the acute cluster headache attack is very different from that during migraine; patients in severe pain with cluster headache cannot lie still, are up walking or sitting, rocking back and forth with pain, or may beat their head with their fists or bang their head on the wall. Patients with migraine want to lie still in a dark quiet room, because movement exacerbates the pain.

The location of the headache and these autonomic features point to the trigeminal nerve and sphenopalatine ganglion anatomic location, but the neurovascular or neuropeptide changes which set in motion this painful periodic disorder remain unknown. Abnormalities of the carotid body and lowered oxygen tension may be causally related.

Cluster headache is always unilateral, and every recurrence over many years is usually on the same side. There are rare reports of the opposite side being involved on occasion.

Like migraine, which has a strong familial tendency, cluster headache has a hereditary predisposition, with first-degree relatives having a 14-fold increase compared with the random population. Only 0.07% of the population is affected, compared with 12% for migraine.4 Cluster headache occurs chiefly in men, 5:1 over women.1 These men usually have a characteristic physiognomy: they are athletic or mesomorphic, with prominent thick facial features, furrowed brow, and deep nasolabial folds. They often have an orange-peel–like skin and telangiectasias of the nose and cheeks. They are often intense, busy, dynamic people, heavy smokers and heavy drinkers who have an increased incidence of coronary and gastrointestinal problems. During a cluster headache siege, patients quickly learn that alcohol will trigger a headache attack, but between clusters they may drink with impunity.

Ninety percent of cluster headache sufferers have episodic cluster headache (ECH), with the cluster period lasting 4 to 12 weeks. The pain stops spontaneously, only to return in several months or years. Chronic cluster headache (CCH) develops in 10% of sufferers.10 This condition usually evolves from ECH, as episodic headaches begin to lengthen in duration and appear more frequently until the attacks are continuous (secondary chronic cluster headache). Rarely, CCH appears at the outset (primary chronic cluster headache). CCH is more resistant to treatment that ECH, and patients suffer terribly.

Differential Diagnosis

Cluster headache must be differentiated from tic douloureux (trigeminal neuralgia), chronic and episodic paroxysmal hemicrania, and SUNCT syndrome (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing). There are rare reports of focal structural lesions, such as ateriovenous malformations, pituitary tumors, intracranial aneurysms, vertebral dissection, or temporal fossa meningiomas, mimicking cluster headache.4 Occasionally, we encounter patients with cluster headache related to trauma or surgery, especially in the fifth cranial nerve distribution; these cases are often resistant to treatment.

In typical cases of cluster headache in which examination is normal, neuroimaging is not necessary unless the patient fails to respond to treatment and the condition continually worsens.

Management

In cluster headache, unlike migraine, nonpharmacologic treatment is seldom effective. There are two approaches to management of cluster headache: treatment of the acute attack and preventive pharmacotherapy.

Approximately 70% of patients with cluster headache obtain prompt relief within a few minutes by breathing 100% oxygen through a nonrebreathing mask at a flow rate of 7 liters per minute for 15 minutes.4 Kudrow advocates the patient sitting while leaning forward with arms on knees during the oxygen inhalation. The oxygen cylinder is often kept at the bedside, and prompt relief is achieved for nocturnal cluster attacks.

Subcutaneous injection of sumatriptan is remarkably and rapidly effective for 74% of patients; response is usually consistent without tolerance or loss of effectiveness throughout the cluster period.11 Sumatriptan nasal spray is sometimes effective. There is less experience with use of the newer triptans in treatment of cluster headache. Migranal NS (dihydroergotamine) or ergotamine tablets or suppositories may be helpful if sumatriptan is ineffective. Lidocaine, 4% nose drops, may be effective but is not a first-line acute care treatment.

As noted, patients with cluster headache are usually middle-aged men who are heavily muscled and frequent smokers, so potent vasoconstrictor medications must be given with caution and are contraindicated if there is a suspected history of heart disease or poorly controlled hypertension.

Preventive treatment may reduce the frequency and severity of individual headaches during the cluster attack and will often interrupt the cluster cycle itself. If this is successful, treatment is usually continued for 3 or 4 weeks and then gradually tapered, but it can be resumed if the headaches return.

A brief tapering course of corticosteroids may break the headache cycle, and can be repeated if the cluster recurs. Calcium-channel blockers are frequently effective; verapamil in high doses has been studied the most thoroughly. Lithium carbonate is often helpful, especially for patients with CCH. Blood levels of lithium, electrolytes, and renal function must be monitored in patients receiving lithium therapy, because high doses may be nephrotoxic. Concurrent use of lithium with diuretics or carbamazepine should be avoided. Indomethacin and the ergot derivatives, such as ergotamine tartrate and methysergide, are often effective for both ECH and CCH. Divalproex sodium, acetazolamide, and intranasal capsaicin have been used successfully. Often, combinations of these medications are required and knowledge of potential toxicity and side effects must be communicated to the patient.1,4,10

The pain of cluster headache is intense and disabling, and the fact that 90% of patients can be helped makes treatment of this condition especially gratifying. For the 10% of patients who respond poorly to outpatient medical management, inpatient intensive therapy or surgery may be offered; successful procedures include glycerol injection, radiofrequency thermocoagulation of the trigeminal ganglion, gamma knife procedures involving the trigeminal nerve root entry zone, or sphenopalatine ganglionectomy.4

Tension-type headache (TTH) is the most common kind of headache, experienced by almost everyone at some time in his or her lifetime. Most people take an aspirin or acetaminophen and never even think about consulting a physician for these occasional mild headaches. TTH is less intense than migraine and seldom disabling; these are nonthrobbing, pressure or tight bandlike global headaches that do not have associated symptoms such as nausea or light sensitivity. Chapter 17 includes the IHS criteria used to diagnose TTH. When TTH occurs for more than 15 days per month over a period of more than 6 months, it is termed chronic tension-type headache (CTTH), distinguishing it from less frequent episodic tension-type headache (ETTH).

Before publication of the IHS system of headache classification in 1988,12 TTH was called tension headache or muscle contraction headache. We commonly see patients whose symptoms begin with suboccipital tightness or nuchal pressure; these mild headaches can often be relieved with simple analgesics or physical therapy. If not treated early, however, this TTH may progress to assume characteristics of migraine, such as throbbing intensity, and radiating to become localized over one orbital region, accompanied by nausea or photophobia. Modern headache investigators increasingly speculate that migraine and TTH may have a common origin resulting from inflammation, altered pain-regulating neuropeptide dysfunction in the brainstem, and changes in cranial blood vessels.13 In support of this view are recent reports about response of TTH to sumatriptan in patients who also have migraine.14 Sumatriptan is believed to decrease migraine symptoms because of its pharmacologic specificity for serotonin 1B and 1D receptors. In any case, patients with ETTH seldom consult physicians, and when they do, treatment is usually straightforward, involving reassurance, simple analgesics, biofeedback, anxiolytics, or tricyclics. Most patients respond to over-the-counter medication, but some require prescription medications such as Fiorinal or Midrin. Occasional use of acetaminophen (Tylenol) with codeine, Fiorinal with codeine, or even butorphanol nasal spray (Stadol NS) may afford rapid relief; these drugs can safely be used up to two times a week, but the patient must be cautioned against dose escalation and increased frequency of use, which can lead to analgesic rebound headaches.

More problematic is the patient with CTTH. Confounding this diagnosis is growing criticism that the IHS classification is not comprehensive; revision of the IHS criteria to subdivide CTTH into patients with or without medication overuse has been proposed.15 Mathew coined the term “transformed migraine” to describe the phenomenon of patients with infrequent migraine in their teens who develop frequent, milder headaches in their twenties and thirties. They often take increasing doses of medications, both prescription and over-the-counter preparations, which usually contain caffeine, barbiturates, aspirin, or acetaminophen.1,4 People with a predisposition to headaches often develop a pharmacologic tolerance to these drugs, and as the medicine is metabolized, a rebound headache occurs, usually in the early morning, several hours after the last dose. The patient then takes more of the offending medicine, resulting in a vicious cycle in which the drug is perpetuating and worsening the headache condition and the episodic migraine is transformed into a chronic, daily headache. Patients with analgesic rebound headaches are very difficult to treat, and hospitalization is often necessary for detoxification and development of a more rational and effective comprehensive treatment program. These patients usually report taking Tylenol with codeine, Fiorinal, and other prescription drugs but often neglect to mention the 12 to 16 Excedrin, Anacin, aspirin, or Tylenol they take every day, so the clinician must remember to ask specifically about nonprescription medications.

CTTH must be differentiated from other chronic headache conditions with different natural histories and perhaps different underlying mechanisms; these include transformed migraine, hemicrania continua, and new, daily, persistent headaches. Several investigators were unable to satisfactorily classify 35% to 40% of their chronic headache patients according to existing IHS diagnostic criteria.15

A relatively new phenomenon is the increasing popularity of alternative, “natural” substances that patients are reluctant to disclose to the physician. Among these are megadoses of herbal sedatives, such as valerian root; St. John’s wort (which can potentiate serotonin reuptake inhibitors); ginkgo biloba (which, combined with aspirin or NSAIDs, can cause serious bleeding); and other herbs and vitamin supplements. These products are sold without FDA regulation or approval as food supplements rather than drugs. Some studies suggest the benefits of oral magnesium, vitamins B2 and B6, and, possibly, less for patients with TTH.

Management

Needless to say, treatment of patients with chronic headaches is more difficult than that of patients with straightforward, infrequent, and uncomplicated migraine. The patient-physician relationship, described by Rapoport and Sheftell as having mutuality of input and decision making, is essential more than ever in these situations.1

For the difficult patient with chronic headaches, behavioral modification is important, and a cornerstone of treatment must include the patient becoming an active participant rather than a passive consumer of medications. Behavioral treatment begins with the patient keeping an accurate headache diary, which records the following characteristics: (1) when the headaches occur—on weekends, after sleeping late, always at the end of a hectic workday, menstrual-related, and so on; (2) potential food and environmental triggers; and (3) medications, doses, and response to treatment.

Pharmacotherapy begins with the patient being able to distinguish TTH from migraine. Early treatment of TTH may afford complete relief. Aspirin, acetaminophen, Excedrin, and NSAIDs are often effective. Patients must be cautioned about possible gastric irritation, and close monitoring of medication is very important. Combinations of over-the-counter and prescription drugs may be preferred. For example, 2 tablets of naproxen sodium plus 2 tablets of Midrin may give satisfactory headache relief. Daily preventive medications, such as cyclobenzaprine or tricyclic antidepressants, may be helpful in conjunction with the nonpharmacologic treatment methods outlined earlier. The tricyclics are especially effective if the patient complains of sleep disturbance or is depressed. If weight gain or excessive daytime drowsiness develops, reducing the dose or replacing tricyclics with SSRIs may be beneficial. Comorbidity of other conditions should always be considered.

Opiates can be helpful for patients with severe TTH but must be used properly, and limitations must be set and followed by patients.