A recurring headache that is of moderate or severe intensity and is triggered by migraine-precipitating factors usually is considered to be migraine. Precipitating factors can include stress, certain foods, weather changes, smoke, hunger, fatigue, hormones, and so on. Migraine without aura is a chronic idiopathic headache disorder with attacks lasting 4–72 h. Status migrainosus applies to migraine headaches that exceed 72 h. Migraine features often include a unilateral location and a throbbing or pulsating nature to the pain. There may be associated nausea, photophobia, phonophobia, or dizziness (Table 1.1). Further characteristics include a positive relationship with menses, decreased frequency during pregnancy, increased pain with physical activity, and history of migraine in first-degree relatives. Between 70 and 75 % of migraine patients report that they have a first-degree relative with a history of migraines [3].

Patients who suffer from migraines often have colder hands and feet compared with controls, and the prevalence of motion sickness is much higher in migraine patients. Although most patients will not have all of these characteristics, there are certain diagnostic criteria that have been established by the International Headache Society for the definitive diagnosis of migraine [4]. Distinguishing a milder migraine without aura from a moderate or severe tension headache may be difficult, and it is not surprising when “pure” migraine medications are effective for severe tension-type headaches.

Approximately 25 % of patients experience visual or other neurologic symptoms preceding or during the migraine; these auras may be as disturbing to the patient as the migraine pain itself. The visual symptoms usually last 15–20 min, and most often will be followed by the migraine headache. Most migraine sufferers experience the same aura with each migraine, but, occasionally, one person may have several types of auras. “The light of a flashbulb going off” is the description many patients give to describe their aura. The visual hallucinations seen most often consist of spots, stars, lines (often wavy), color splashes, and waves resembling heat waves. The images may seem to shimmer, sparkle, or flicker. These visual occurrences are referred to as photopsia. Fortification spectra are seen much less often than photopsia. They usually begin with a decrease in vision and visual hallucinations that are unformed. Within minutes, a paracentral scotoma becomes evident and assumes a crescent shape, usually with zigzags. There often is associated shimmering, sparkling, or flickering at the edges of the scotoma. Patients may experience a “graying out” of their vision, or a “white out” may occur. Some patients suffer complete visual loss, usually for some minutes. Photopsia may be experienced at the same time as the gray out, white out, or visual loss.

Numbness or tingling (paresthesias) commonly are experienced by patients as part of a migraine. These are experienced most often in one hand and forearm, but may be felt in the face, periorally, or in both arms and legs. Like the visual disturbances, they often last only minutes preceding the pain, but the numbness may continue for hours, and at times the paresthesias are severe. The sensory disturbances usually increase slowly over 15–25 min, differentiating them from those with a more rapid pace that are seen in epilepsy.

Paralysis of the limbs may occur, but this is rare. This occasionally is seen as a familial autosomal dominant trait, which is termed familial hemiplegic migraine. With the weakness, aphasia or slurred speech may also occur, and sensory disturbances are seen ipsilateral to the weakness.

Vertigo occasionally is experienced during migraine, and may be disabling.

“Migraine-associated vertigo” has become a common diagnosis. Ataxia may occur, but it is not common. Rarely, multiple symptoms of brain stem dysfunction occur, with the term migraine with brainstem aura (previously called basilar migraine) being applied to this type of syndrome. The attack usually begins with visual disturbances (most often photopsia), followed by ataxia, vertigo, paresthesias, and other brain stem symptoms. These severe neurologic symptoms usually abate after 15–30 min and are followed by a headache. This type of migraine often stops over months or years, and the patient is simply left with migraine headaches without neurologic dysfunction.

As noted, when patients present with a long history of typical migraine attacks, and the headaches are essentially unchanged, scans of the head may not be necessary. Whether to do any testing at all depends on the physician’s clinical suspicion of organic pathology. Sound clinical judgment, based on patient history and a physical exam, is crucial in deciding which exams a given patient needs.

In addition to the MRI and CT scan, tests that are generally useful for diagnosis of headache include lumbar puncture, IOP testing, CT scan of the sinuses, and blood tests. A magnetic resonance angiogram (MRA) allows the detection of most intracranial aneurysms.

The problems that need to be excluded in a patient with new-onset migraine include sinus disease, meningitis, glaucoma, brain tumor, arteritis, subarachnoid hemorrhage, idiopathic intracranial hypertension, hydrocephalus, pheochromocytoma, stroke or transient ischemic attack, internal carotid artery dissection, and systemic illness.

With migraine and chronic daily headache sufferers, avoidance of triggers should be emphasized. The most common triggers are stress (both during and after stress), weather changes, perimenstruation, missing meals, bright lights or sunlight, under- and oversleeping, food sensitivity, perfume, cigarette smoke, exercise, and sexual activity. Some foods can be headache triggers, but foods tend to be overemphasized. In general, headache patients do better with regular schedules, eating three or more meals per day, and going to bed and awaking at the same time every day. Many patients state that “I can tell the weather with my head.” Barometric changes and storms are typical weather culprits, but some patients do poorly on bright “sun-glare” days.

Regarding stress as a trigger, it is not so much extreme stress but rather daily hassles that increase headaches. When patients are faced with overwhelming daily stress, particularly when they are not sleeping well at night, headaches can be much worse the next day.

Psychotherapy is extremely useful for many headache patients with regard to stress management, coping, life issues, family-of-origin issues, and so on. Although psychotherapy may be recommended, it is crucial to legitimize the headaches as a physical condition; headaches are not a “psychological” problem but rather a physical one that stress may exacerbate. If a person inherits the brain chemistry for headache, these triggers come into play; without the inherited genetics, most people may have stress/weather changes/hormonal changes but not experience a headache.

Managing stress with exercise, yoga, and Pilates, often will reduce the frequency of headaches. The ideal would be for the patient to take a class weekly, then do the stretches and breathing for 10 min/day. Relaxation techniques such as biofeedback, deep breathing, and imaging also can be helpful for daily headache patients, particularly when stress is a factor.

Many migraine patients have accompanying neck pain. Physical therapy may help, and acupuncture or chiropractic treatments occasionally help as well. Certain physical therapists “specialize” in head and neck pain. Massage may be effective, but the relief often is short-lived. Temporomandibular disorder (TMD), with clenching and/or bruxing, may exacerbate migraine. For patients with TMD, physical therapy, a bite splint, and/or onobotulinum toxin A (Botox) injections may help. It often “takes a village” to help a person with pain, and we recruit other “villagers,” such as physical therapists and psychotherapists.

The most common first-line treatment for migraines includes triptans. More than 200 million patients worldwide have used triptans. The most effective way to use triptans is to take them early in the headache—the earlier a patient takes these agents, the better the effect. Sumatriptan is an extremely effective migraine-abortive medication with minimal side effects. It is effective for approximately 70 % of patients and has become the gold standard in abortive headache treatment. The usual dose is one tablet every 3 h, as needed; maximum dose, two tablets per day. However, clinicians do need to limit triptan use (ideally, 3 days per week) to avoid rebound headaches or medication overuse headaches (MOH).

Triptans are helpful for moderate as well as more severe migraines. Certain patients may tolerate one triptan better than others, and it is worthwhile for patients to try several. Triptans are an excellent choice for migraine patients who are not at risk for coronary artery disease (CAD). Patients in their 50s or 60s can use these drugs, but they should be prescribed cautiously, and only in those patients who have been screened for CAD. Over the 23 years that triptans have been available, serious side effects have been few; they appear to be much safer than was previously thought in 1993.

As noted, if patients do not do well with one triptan (lack of efficacy or side effects), it is usually worthwhile for them to try at least one or two other triptans. While they are all very similar, the minor chemical differences between them mean that some patients do well with one, and not another.

The usual triptan side effects may include pressure (or tightness) in the chest/neck (or other muscle areas), tingling, and fatigue. These are usually transient, lasting 10–30 min. If a patient experiences moderate to severe chest/throat/neck pressure (or pain), we usually discontinue the triptan or substitute a milder one (naratriptan/frovatriptan). The chest symptoms are rarely cardiac in nature, which is the primary concern with chest symptoms.

There are a number of triptan choices. Sumatriptan, zolmitriptan, rizatriptan, and naratriptan are available in generic formulations. Eletripton (Relpax) is a very effective triptan and almotriptan (Axert) is useful for many patients (Fig. 1.1).

Treximet is a combination of sumatriptan and naproxen. Frovatriptan (Frova) is a “slow onset,” milder triptan, which has a longer half-life. Zolmitriptan (Zomig) nasal spray is not generic, but it is very effective, with a quick onset of action. The sumatriptan injections (available in various forms) remain the most effective migraine abortives.

For patients who cannot tolerate triptans, there are a number of other effective nontriptan first-line approaches, including diclofenac potassium powder (Cambia), Excedrin Migraine, naproxen, ketorolac, ibuprofen, and Prodrin (similar to Midrin, but without the sedative). We often combine two first-line approaches—for example, a triptan and a nonsteroidal anti-inflammatory drug (NSAID).

In general, drugs containing ergotamine (also called ergots) are effective second-line therapy for migraines. They were the first antimigraine drugs available, but they have many side effects, and, at most, should be used only 2 days per week. Dihydroergotamine (DHE) is the safest ergot derivative. Intravenous DHE primarily is a “venoconstrictor” with few arterial effects. This renders it very unlikely to cause cardiac problems. Indeed, since its introduction in 1945, DHE has been remarkably safe. Intravenous DHE can be administered in the office or emergency room. Nasal (Migranal Nasal Spray) and inhaled forms of DHE (soon to be released) have been found to be safe and effective as well.

Barbiturates and opioids have been studied and are effective, but because of the risk for addiction, they should be used sparingly. For severe prolonged migraines, corticosteroids (oral, intravenous (IV), or intramuscular) often are effective. It is important to use low doses of steroids.

Many patients use 3–6 abortives: a triptan, NSAID, Excedrin, an antinausea medication, and a painkiller (opioid/butalbital). Patients will use each medication in different situations, for different types and degrees of headache. Tables 1.5, 1.6, and 1.7 review all the first- and second-line migraine-abortive medications.

Muscle relaxants (carisoprodol, diazepam) or tranquilizers (clonazepam, alprazolam) occasionally are useful, primarily to aid in sleeping. Intravenous valproate sodium (Depacon) is safe and can be effective. The atypical antipsychotics, such as olanzapine (Zyprexa) or quetiapine (Seroquel), occasionally may be useful on an as-needed basis. In the emergency room, IV administration of antiemetic agents such as prochlorperazine (Compazine, others) or metoclopramide (Reglan) may be useful (Table 1.8). Certain preventive medications, such as valproic acid, or divalproex sodium (Depakote), topiramate (Topamax), and amitriptyline—in low doses every 4–6 h—may be useful on an as-needed basis. The antihistamine diphenhydramine occasionally is useful when administered intramuscularly. At times, patients may have injections for home use (ketorolac, orphenadrine, sumatriptan, diphenhydramine, promethazine, etc.). Transcranial magnetic stimulation (TMS) will soon be available. Patients will have a hand-held device, placed over the occiput, for only a minute. Several quick magnetic pulses are delivered, which are generally very safe. This may be effective for many people. TMS has been around for some time (for depression), in much higher doses, and has been fairly safe and well tolerated.

Much is written about MOH, with many patients diagnosed with this condition. Often a patient will be overusing abortive agents but will not be suffering “rebound/withdrawal” headaches (medication overuse, but not MOH). Up until recently, all NSAIDS were lumped under “medications that cause MOH,” and this simply is not correct. For some patients, opioids, butalbital, and medications containing a lot of caffeine cause MOH. Triptans are implicated occasionally as well. However, preventives may not be effective for most patients with chronic migraine (daily or near-daily headaches), and they use abortives to help themselves get through the day.