Nonneurologists who treat patients with headaches should be able recognize common headache types and to initiate therapy for tension-type headaches and migraines. Patients with complicated headache scenarios should be referred to a neurologist for consultation.
Key points
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Patients with common primary headache types can be treated by multiple different specialists, including otolaryngologists.
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Most patients with chronic headaches who present for medical evaluation and treatment will have migraine headaches, which afflict approximately 36 million Americans.
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Brain imaging, which is not necessary for most patients with primary headaches, may show unrelated incidental findings.
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Migraines can be treated with lifestyle modification, triptans for acute treatment of pain, and preventive medications to decrease the frequency and severity of headaches.
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Multiple types of side-locked headaches, including those with ipsilateral autonomic symptoms, can be prevented by indomethacin.
Overview
Headache is a virtually universal experience, with almost everyone experiencing headaches at some time. As a very common complaint causing patients to seek medical consultation, physicians of almost every specialty will hear about headaches from patients, friends, or family. Many of the millions of chronic headache sufferers can and should be treated by nonneurologists, including otolaryngologists. Primary care practitioners, including internists and gynecologists, treat most patients with episodic or uncomplicated headaches. Patients often consult otolaryngologists before any other specialty because they interpret their head or facial pain of migraine as a symptom of sinus or ear disease. Otolaryngologists should be aware of the different common headache types of their patients, as they can often diagnose and initiate treatment. Ophthalmologists are also often consulted initially, when chronic headaches are interpreted as being due to eye strain. Patients may consult with dentists because they interpret headaches or facial pain as being caused by temporomandibular joint pain or tooth disorders. The primary headache types of migraine headache, cluster headache, paroxysmal hemicrania, and hemicrania continua may present to dentists with the chief complaint of tooth pain.
Overview
Headache is a virtually universal experience, with almost everyone experiencing headaches at some time. As a very common complaint causing patients to seek medical consultation, physicians of almost every specialty will hear about headaches from patients, friends, or family. Many of the millions of chronic headache sufferers can and should be treated by nonneurologists, including otolaryngologists. Primary care practitioners, including internists and gynecologists, treat most patients with episodic or uncomplicated headaches. Patients often consult otolaryngologists before any other specialty because they interpret their head or facial pain of migraine as a symptom of sinus or ear disease. Otolaryngologists should be aware of the different common headache types of their patients, as they can often diagnose and initiate treatment. Ophthalmologists are also often consulted initially, when chronic headaches are interpreted as being due to eye strain. Patients may consult with dentists because they interpret headaches or facial pain as being caused by temporomandibular joint pain or tooth disorders. The primary headache types of migraine headache, cluster headache, paroxysmal hemicrania, and hemicrania continua may present to dentists with the chief complaint of tooth pain.
Primary and secondary headache types
The International Headache Society (ISH) has published systematic definitions of different types of headaches in an effort to improve the diagnosis and treatment of headaches. Headaches are divided broadly into primary and secondary, depending on their cause. Primary headaches, the most common type, are not associated with anatomic or physiologic abnormalities. Most patients who present for medical evaluation will have a type of primary headache, usually migraine headaches. The major primary headaches are migraine (with or without aura), tension-type headache, and trigeminal autonomic cephalgias (TACs). Other more unusual types of primary headache may occur less frequently, especially in individuals prone to headaches of multiple types. Health care providers other than neurologists, including otolaryngologists, can diagnose and treat many patients with primary headache types.
Secondary headaches are due to a distinct and known anatomic, physiologic, inflammatory, vascular or infectious cause. Although they are much less common than primary headaches, secondary headaches should be considered in all patients who present for evaluation of headaches. Patients who have a primary headache disorder may also develop unrelated secondary headaches, so primary headache patients should be questioned about a change in character or frequency of headache that may indicate another superimposed headache type. There are many types of secondary headaches, most of which should be treated in consultation with a neurologist. The underlying disease causing the headaches may require urgent diagnosis and treatment because of the potential complications that can expand beyond head pain. Even if the underlying disease causing secondary headaches does not itself mandate immediate intervention, secondary headaches generally will not resolve until their specific cause is diagnosed and treated. Box 1 lists some, but not all, red flags indicating that patients should be rapidly triaged to a neurologist for evaluation. Causes of secondary headache include mass lesions, such as tumors and vascular lesions, which are generally accompanied by focal neurologic signs and symptoms. Infections, including meningitis, encephalitis, and brain abscess, may present with headache, fever, alteration in consciousness, and focal neurologic signs and symptoms pointing toward the diagnosis of a secondary headache. Headaches with persistent focal neurologic complaints and/or abnormalities on neurologic examination should be referred to a neurologist for further evaluation.
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Abnormal neurologic examination, including papilledema
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Focal neurologic symptoms that last longer than 30 minutes or do not disappear after headache is over
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A severe headache that is of peak intensity at the very onset of pain
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Unexplainable worsening of previously existing headaches
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Change in character of the patient’s typical chronic headaches
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Side-locked headaches that are never bilateral or contralateral
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Headaches that are clearly positional (eg, worse when recumbent), or severely worse with coughing, sneezing, or Valsalva
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New-onset headaches at an older age (>50 years)
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New-onset headaches in patients with active cancer or human immunodeficiency virus infection
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Headache associated with systemic illness (eg, fever, rash, stiff neck)
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Chronic headaches, transient visual changes, pulsatile tinnitus in an obese woman
Brain imaging in headache disorders
The issue of appropriate brain imaging in headache patients is problematic, as not all patients with headaches should be imaged. If more than half of all Americans have a headache each year and headache disorders in the United States already result in more than $31 billion in annual direct and indirect economic costs, imaging the entire population with headaches, even once in their lifetime, would be impractical and an exorbitant waste of resources. Estimating which patients are likely to have a clinically relevant brain lesion causing headaches is crucial to avoid both unnecessary imaging and unnecessary detection of incidental and clinically irrelevant lesions. In patients without focal neurologic abnormalities on examination who appear to have a primary headache, the yield of brain imaging for significant or correlating intracranial findings is generally low. Most patients who present with a history of chronic headaches do not need brain imaging. If the history and physical examination are consistent with migraine or tension-type headache (TTH), and if there are no red flags indicating a possible secondary headache, an imaging procedure is not needed. However, if the patient with a migraine or TTH does not respond as expected to the appropriate treatment, imaging, generally a magnetic resonance imaging (MRI) scan of the brain without contrast, is appropriate.
Indiscriminate imaging often reveals findings without clinical significance that increase anxiety. The incidence of incidental, yet not clinically relevant, brain and head and neck imaging findings in a young healthy population, such as those with primary headaches, is high. An analysis of the MR images obtained from 203 healthy young adult volunteers (mean age 21.9 years; range 18–35 years) found a high prevalence of incidental brain and head and neck abnormalities (9.4% and 36.7%, respectively). All incidental brain findings were clinically silent, not requiring follow-up, and included cysts (pineal gland, arachnoid, Rathke cleft), widened bifrontal subarachnoid space, white matter lesions, and Chiari I malformations. The high occurrence of abnormal findings (36.7%) in the upper head and neck region was mainly explained by simple sinus disease which, when excluded, reduced the incidence to 14.4%. The most common incidental head and neck findings were hyoplastic frontal or maxillary sinuses, sinonasal retention cysts or polyps, mucosal swelling, lymphadenopathy, cystic lesions in the parotid gland, and bilateral osteomeatal abnormality. In older adults, incidental findings on brain MRI are likely to include increased white matter hyperintensities attributable to ischemic small-vessel disease, silent brain infarcts, and incidental neoplasms such as meningiomas. The detection of incidental findings of MRI increases with high resolution sequences in comparison with standard resolution.
Migraine is associated with a significantly increased risk of brain lesions found on MRI, including subclinical infarcts in the posterior circulation, white matter lesions, and brainstem hyperintense lesions. The number of migraine attacks, frequency of migraines, migraine severity, type of migraine headaches, and migraine therapy are not associated with lesion progression. Increase in deep white matter hyperintensity volume on MRI in migraine patients was not significantly associated with worsening cognition. The patient with chronic headaches who is found to have white matter lesions on MRI should be advised that these lesions are unlikely to be of clinical significance. However, referral to a neurologist for consultation may be indicated, in order to discuss the MRI findings and to evaluate for the rare overlap between migraines and other neurologic diseases that can cause white matter lesions in the brain.
Although the likelihood of finding a causal lesion in a suspected primary headache disorder is very small, imaging may be indicated in some patients with primary headache to rule out primary-headache mimics. Criteria that may be used to justify imaging in suspected migraine patients include: unilateral headaches that are always on the same side; headaches associated with aura lasting longer than an hour or an aura that persists after the headache has resolved; and motor or dysphasic auras. In most cases for patients with a suspected migraine or TTH, if a brain imaging study is considered appropriate, an MRI scan of the brain without contrast should be ordered to rule out a causal lesion. When there is a suspicion of a secondary headache, brain imaging with an MRI scan of the brain (or a computed tomography [CT] scan of the head if an MRI is contraindicated) should be obtained, often with contrast enhancement. Patients with most secondary headaches should be treated in consultation with a neurologist .
A 30-year-old man consulted an otolaryngologist for right frontal throbbing head pain and nasal congestion. The pain, which was never on the left or bilateral, was improved by rest in a quiet, dark room and acetaminophen/aspirin/caffeine combination medication. He had photophobia and phonophobia, but no nausea. He had a chronic cough. He denied ringing in the ears, dizziness, or hearing loss. Nasopharyngeal endoscopy revealed a septum severely deviated to the left, with a large spur with nasal obstruction and inferior turbinate hypertrophy. The neurologic examination was normal. A CT scan of the head and sinuses was interpreted as being normal ( Fig. 1 A–D). Migraine was diagnosed and the patient was treated with a triptan. The throbbing headaches with sensitivity to light and sound increased in frequency. A more detailed history revealed that the patient had bilateral dull headaches behind his eyes without nausea or photophobia/phonophobia, starting as a young boy. Then for about 3 to 4 years he had different headaches with right facial pain lasting up to 2 minutes. About 2 years after his initial evaluation, a neurologist found a left visual field deficit and pupillary asymmetry, with a possible skew deviation and papilledema. An MRI scan showed a large sellar/suprasellar mass pressing on the chiasm (see Fig. 1 E, F). A right supraorbital craniotomy for lesional biopsy diagnosed a prolactin-secreting pituitary adenoma.
Migraine
Most patients who present to a physician for evaluation and treatment of chronic headaches will have migraine headaches. Although headaches span the human life cycle, with infants and centenarians both suffering the effects of migraines, the peak age for migraines is 25 to 55 years. The prevalence of migraine in adults is 18.5%, migraine with aura 4.4%, and chronic migraine 0.5%. The rate of migraine in children is 10.1%, and migraine with aura 1.6%. Most patients who seek medical attention from nonneurologists for chronic headaches producing functional disability have migraine headaches. Migraine headaches are severely disabling, with a distinct pathophysiologic mechanism. While the IHS criteria for the diagnosis of migraine headache ( Box 2 ) are useful for research studies on migraine, they are too stringent to encompass all headaches in migraine sufferers. Migraine headaches can be unilateral or bilateral, with pain that can be throbbing or constant. Accompanying gastrointestinal symptoms and sensitivities can occur with some, but not all, migraine headaches. Migraine headaches may be triggered in susceptible individuals by environmental stimuli that are innocuous to those who do not suffer migraines. The most common migraine triggers are change in sleep habits (too much or too little sleep), stress or relief from stress, alcohol, change in weather, and hunger and/or dehydration. Some foods may trigger some headaches in some people at some time; in general, however, strict food avoidance has little effect on migraine prevention for most patients. In many women their menstrual period is a very strong trigger for their most severe migraine headaches. Identification of individual triggers by a patient is the first step in migraine prevention.
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At least 5 attacks fulfilling criteria B–D
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Headache attacks lasting 4 to 72 hours (untreated or unsuccessfully treated)
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Headache has at least 2 of the following 4 characteristics:
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Unilateral location
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Pulsating quality
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Moderate or severe pain intensity
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Aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
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During headache at least 1 of the following:
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Nausea and/or vomiting
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Photophobia and phonophobia
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Not better accounted for by another ICHD-3 diagnosis
Most migraines are not accompanied by any focal neurologic symptoms, but some migraine sufferers may have some headaches with a neurologic aura. Migraines with aura are migraine headaches with focal neurologic symptoms (binocular visual disturbances, unilateral numbness, unilateral weakness, speech difficulty) that occur before or during the head pain. The aura generally lasts less than 60 minutes. There may be a symptom-free period of up to 60 minutes before the headache pain begins. The most common aura is binocular alteration in vision, with a scintillating scotoma in the unilateral peripheral vision that increases in size over time (ie, gradual onset) and then suddenly disappears (ie, sudden offset). Specialists other than neurologists are often consulted by patients with migraine, with ophthalmologists seeing patients in whom the new-onset visual aura is more distressing than the accompanying headache.
Vertigo is very common in migraneurs, both in association with headache and occurring in isolation. A temporal overlap between vestibular symptoms, such as vertigo and head-movement intolerance, and migraine symptoms, such as headache, photophobia, and phonophobia, may indicate the diagnosis of vestibular migraine. The symptoms of imbalance and vertigo may overshadow the head pain, leading to initial consultation with an otolaryngologist. Vestibular migraine, recurrent attacks of vertigo caused by migraine, presents with attacks of spontaneous or positional vertigo lasting seconds to days, accompanied by migrainous symptoms. When headache is absent during acute attacks, other migrainous features are identified by history in a patient with a predilection for headaches. By contrast, vestibular testing serves mainly for the exclusion of other diagnoses. Once underlying otologic disease is ruled out, treatment is targeted at the underlying migraine. Caffeine cessation, nortriptyline, and topiramate, treatments used successfully for chronic migraine both with and without aura, have been shown to be effective treatments for migraine-related vertigo.
Patients with frequent or prolonged migraine with aura should generally have a consultation with a neurologist, as patients with migraine with aura have an increased risk of stroke and cardiovascular disease. These patients should be screened for vascular risk factors to determine their individual risk. An increased risk for ischemic stroke has been found in patients with migraine, with the greatest and most consistent increase found in younger women who have migraine with aura. Relative estimates of increased risk range from 3.8 to 8.4. However, the absolute risk of ischemic stroke is small; the estimated attributable risk ranges from 18 to 40 additional cases of ischemic stroke per 100,000 women per year. Oral contraceptives and smoking increase the risk for ischemic stroke in women with migraine with aura. An increased prevalence of patent foramen ovale in patients with migraine with aura has an unclear significance in the pathogenesis of migraine-associated stroke.
Migraine Treatment
Lifestyle modification
Patients have often lived with chronic headaches for years, thinking that having headaches is a normal part of life for which there is no treatment. Recognition of the disability of patients’ chronic headaches along with a compassionate, consistent approach to their care can markedly improve the quality of their lives.
The general principles of headache treatment are:
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Establish an accurate diagnosis
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Educate patients about their condition, its treatment, and its natural history
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Establish realistic expectations about headache treatment
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Encourage patients to participate in their own management
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Adjust lifestyle to avoid headaches
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Discuss treatment/medication preferences
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Cooperate with medical treatment recommendations
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Keep a headache diary to monitor the frequency and severity of headaches
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The first step in decreasing the frequency and severity of headaches is a regulated lifestyle with avoidance of headache triggers. Recommended lifestyle modification includes all of the following: regular meals minimizing alcohol and processed foods; the same number of hours of refreshing sleep every night; adequate hydration with water; regular, pleasurable exercise; and avoidance of stress. Any physician consulted by a patient who appears to have migraine headaches can counsel patients on lifestyle modification. Box 3 lists lifestyle suggestions that can be offered to patients as an initial nonpharmacologic approach to the management of migraine headaches. For many patients, lifestyle management in combination with an effective acute pain medication significantly decreases the disability associated with chronic migraines.
