Headache disorders are among the most prevalent medical problems worldwide. The World Health Organization estimates that 50% to 75% of all adults between the ages of 18 and 65 have headaches. This high prevalence results in significant disability and lost productivity: headache disorders are the third highest cause of years lost to disability worldwide.

Accordingly, headaches are one of the most common reasons patients present to physicians in primary care settings, in the emergency department (ED), or in neurologists’ offices. For clinicians evaluating a patient with headaches, the first responsibility is to diagnose the type of headache correctly, and then treat appropriately, because most headache disorders have excellent treatments available, which can reduce the burden of disability.

Headache disorders fall into two categories, primary headache disorders (those caused by the headache disorder itself, not due to other causes) and secondary headache disorders, those caused by (or “symptomatic of”) another underlying medical problem. The pain can be due to the involvement of pain-sensitive structures in the head, including cranial nerves, cervical nerve roots, blood vessels, meninges, scalp, temporomandibular joint (TMJ), teeth, pericranial and cervical muscles, and paranasal sinuses.

Patients may also have multifactorial headaches, so a detailed history and examination are necessary to identify the contributing factors. Headache disorders may remain refractory to treatment or have an insufficient response to treatment if these comorbidities are not identified and addressed.

DIAGNOSIS

A detailed history and examination are vital in understanding the headache’s cause. There are no biomarkers currently available for primary headache disorders.

KEY POINTS FOR THE HISTORY

When obtaining the history the following information must be elicited:

•Onset

•Precipitants and triggers

•Duration

•Location (unilateral or bilateral; frontal, lateral, vertex, or occipital)

•Quality and severity

•Frequency

•Alleviating and exacerbating factors

•Positional influences (better or worse when supine)

•Waking the patient from sleep, or occurring upon awakening

•Associated with menses

•Associated symptoms

Additional aspects of the history important in evaluating a patient with headache are:

•Analgesic use

•Caffeine use

•Medical history

•Current or recent pregnancy

•Medications (including asking specifically about contraceptive use, over-the-counter treatments, and supplements)

•Social history, including detailed screening for illicit drugs

•Family history

•Sleep, including a history of insomnia and snoring; symptoms of obstructive sleep apnea

The semiology of the headache helps to differentiate a primary from a secondary headache disorder. The history also allows a clinician to identify red flags that suggest a secondary headache disorder (Box 10-1).

KEY POINTS FOR THE NEUROLOGIC EXAM

BOX 10-1. Red Flags

A full neurologic exam should also be performed, with emphasis on the funduscopic exam to assess for papilledema. The cortical sensory exam can suggest cortical dysfunction that may occur with venous sinus thrombosis. Focal neurologic deficits, including field cuts, cranial nerve palsies, weakness, or sensory symptoms, often suggest a secondary headache.

PRIMARY HEADACHE DISORDERS

Primary headache disorders are those not due to another medical condition. Diagnosis is established by history and exam. Migraine is by far the most prevalent primary headache disorder. Table 10-1 outlines common primary headache disorders based on key features of the history. Figure 10-1 shows the common locations of pain in the primary headache disorders, compared to that of headaches caused by sinus disease.

MIGRAINE

Migraine Without Aura

It is estimated that one in seven adults worldwide has migraine. It impacts women more than men in a 2:1 ratio. Migraine may start in childhood and manifest occasionally with abdominal symptoms (“abdominal migraine”). Motion sickness in children is a risk factor for the development of migraine.

Migraine headaches are most likely to develop in adolescence and early adulthood. They can be episodic or chronic. The disability and lost productivity from migraine are substantial, because it impacts people in their prime working years.

Migraine has numerous identified triggers, including weather changes, menses, and caffeine (both withdrawal and overuse). Many patients identify foods and drinks such as alcohol (most commonly red wine), soft cheeses, and nitrite-heavy foods, such as processed meats, as precipitants, but data are sparse in this area and many migraine attacks occur without identifiable triggers.

To diagnose migraine, a patient must have at least five attacks with the following characteristics:

1.The headache lasts for 4 to 72 hours if untreated.

2.It must include at least two of the following features:

a.Throbbing

b.Unilateral headaches

c.Worsening with activity, such as walking

FIGURE 10-1. Location of pain associated with primary headache disorders. Left to right: sinus headache, cluster headache, tension headache, migraine headache. (Used with permission of A.D.A.M.)

Migraine with Aura

Migraine headaches are often preceded by focal neurologic symptoms known as auras. These are also called classic migraine or complicated migraine. Auras are defined as fully reversible neurologic symptoms with a gradual onset, usually followed by a headache. The aura usually lasts for 5 to 60 (often 20) minutes and is typically unilateral. It usually resolves without lingering neurologic deficits. Patients are diagnosed with this disorder when they have an aura followed by a headache that meets the criteria for migraine, as above. Some auras occur without a headache (“acephalgic migraine”), but these symptoms usually require additional investigation for a definite diagnosis.

Visual auras are by the far the most common. Some include a “fortification spectrum” (zigzag lines off the central vision, usually spreading gradually) or a scintillating (or flickering) scotoma (an area of decreased visual acuity surrounded by preserved vision).

Migraine auras can also involve sensory symptoms, most commonly paresthesias (tingling or pins-and-needles sensation). The paresthesias often “march” or spread gradually over the course of several minutes along a limb or extend from an arm to the leg or face.

Migraine auras can also include a gradual onset of weakness, a variant known as hemiplegic migraine when severe. Hemiplegic migraine may be sporadic but there is also a syndrome of familial hemiplegic migraine, sometimes associated with well-characterized genes.

Migraine auras are believed to be due to “cortical spreading depression” in which there is a spread of hyperpolarization of the cortex followed by a wave of depolarization. Imaging studies have shown decreased regional cerebral blood flow in the cortex during migraine aura, but not to the level of worrisome ischemia.

COMPLICATIONS ASSOCIATED WITH MIGRAINE

Status Migrainosus

When migraine lasts for more than 72 hours, the condition is known as status migrainosus. This is often caused by abortive medication overuse (often referred to as rebound headache) and frequently requires intravenous (IV) treatment or a brief course of oral steroids to break the headache cycle.

Stroke Risk Associated with Migraine

Patients with migraine with aura have an increased cardiovascular risk when compared to healthy controls. The use of estrogen-based contraceptives is therefore contraindicated in patients with migraine with aura, as the combination results in a substantially increased stroke risk.

Migraine and Menses

Women of reproductive age frequently have exacerbations of migraine during menses, most commonly 1 to 2 days prior to the onset of bleeding, often persisting for up to 3 days into bleeding. This is thought due to the withdrawal of estrogen that occurs with menses. Some women have migraine at the time of menstruation only, a condition known as pure menstrual migraine. Most, however, have a few episodic headaches at other times of the month, or menstrually related migraine. It is important to identify the relationship of menses to migraine because there are specific treatments that may be helpful for patients with a clear exacerbation around their menses.

Chronic Migraine

Patients who have a headache more than 15 days/month for more than 3 months are diagnosed with chronic migraine. Some patients with chronic migraine do not have typical features of migraine with all headaches, but they must have at least 8 days of headache consistent with migraine to be diagnosed with chronic migraine. If the headaches are not consistent with migraine, other diagnoses must be considered.

Patients often describe a history of gradually progressive episodic migraines that increase in frequency to the point of meeting criteria for chronic migraine. With frequent headaches, many patients with chronic migraine have some component of medication overuse headache (MOH). Importantly, patients with chronic migraine can revert to episodic migraine after effective treatment.

MIGRAINE TREATMENTS

Migraine treatments are divided into two categories.

Abortive Treatments

Abortive treatments, also called rescue medications, are medications used to stop a migraine at the onset. All abortive treatments are most effective if the patient is treated at the onset of the headache. Delay in treatment results in more prolonged disability, so patients must be counseled on the appropriate use of abortive treatments.

There are three primary categories of preventive oral medications: antihypertensives, antiseizure medications, and antidepressants. Within each category, there are specific drugs with the most evidence of efficacy (Table 10-2). In addition to oral therapies, onabotulinum toxin A (often referred to simply as Botox) was also approved as migraine prophylaxis for chronic migraine in 2010. In 2018, a new class of preventative therapy for chronic migraine, Calcitonin Gene-Related Peptide (CGRP) antagonists was approved by the FDA. Erenumabis is an injectable human monoclonal antibody that antagonizes CGRP receptor function..

As with abortive treatments, selecting the right prophylactic medication requires careful consideration of the patient’s comorbidities, concomitant medications, cost, and family planning goals. Patients must be counseled about treatment options and side effects, including teratogenicity and impact on contraceptives. Patients who require preventive therapy also require abortive treatments. Some abortive treatments interact with prophylactic medications (such as antidepressants and triptans) which should be taken into consideration.

Lifestyle Modifications

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