• Chronic headache disorder with episodic manifestation characterized by recurrent paroxysms of headache capable of altering daily function lasting from 4–72 hours (1)[C]. Pre-headache symptoms are nonspecific, may occur hours to days before headache. Most frequent subtypes are as follows:
  • Without aura: (Common migraine); defining >80% of attacks, often associated with nausea, vomiting, photophobia, and phonophobia.
  • With aura: Visual or other types of neurological phenomenon precede the headache.
• Other subtypes:
  • Transformed migraine: Chronic headache pattern evolving from episodic migraine. Migraine-like attacks are superimposed on a daily or near-daily headache pattern (e.g., tension headache).
  • Medication overuse headache: Daily or near daily use of acute medication perpetuating the headache pattern
  • Basilar migraine: Occipital headache, with aura symptoms of dysarthria, vertigo, tinnitus, ataxia, and bilateral paresis or bilateral paresthesias
  • Hemiplegic migraine: Aura consisting of hemiplegia and/or hemiparesis
  • Ophthalmoplegic: Palsy of the ipsilateral 3rd cranial nerve during the headache phase:
    • Some published case studies of abducens nerve palsy (2)
  • Retinal: Symptoms of retinal vascular involvement during migraine
  • Menstrual-related migraine: Associated with onset of menstrual period
  • Childhood periodic syndromes (migraine equivalents): Recurrent, often cyclic, episodes of symptoms
  • Status migrainous: Persistent migraine that does not resolve spontaneously
  • Migrainous stroke: Persistent or permanent neurological deficits persisting beyond migraine attack usually with neuroimaging changes

• Female > Male (3:1) menarche to adult
• Female > Male (2:1) postmenopausal women

Prevalence
Adults: Women, 18%; Men, 6%

• Foods, alcohol, missing meals, menstrual cycle, excessive sleep, fatigue, emotional stress, let down (relief of stress)
• Medications:
  • Cyclic estrogen replacement
  • Birth control pills
  • Vasodilators
• Family history of migraine
• Female gender
• History of childhood cyclic vomiting, cyclic abdominal pain, motion sickness

Genetics

• >80% of patients have positive family history
• Familial hemiplegic migraine has been shown to be linked to both chromosomes 19 and 1.

• Avoid precipitants of attacks.
• Biofeedback, education, and psychological intervention
• Prophylactic therapy if attacks frequent, interfere with lifestyle or are not controlled by acute interventions
• Exercise may reduce the intensity of migraines (3)[C].

• Sensory neurons in the brainstem inappropriately activated
• No longer believed to be primarily vascular in etiology

Largely unknown; serotonin, dopamine, calcitonin-gene related peptide may have role

• Depression
• Panic disorder
• Sleep disturbance
• Cerebral vascular disease
• Cardiac anatomic abnormalities (PFO)
• Peripheral vascular disease
• Seizure
• Irritable bowel syndrome

History

• Headache usually begins with mild pain that escalates into a unilateral (30–40% bilateral), throbbing (40% non-throbbing) pain of 4–72 hours duration.
• Intensified by movement and associated with systemic manifestations: Nausea (87%), vomiting (56%), diarrhea (16%), photophobia (82%), phonophobia (78%), muscle tenderness (65%), lightheadedness (72%), vertigo (33%)
• May be preceded by aura:
  • Visual disruptions are most common, including scotoma, hemianopsia, fortification spectra, geometric visual patterns, and occasionally hallucinations.
  • Somatosensory disruption in face or arms
  • Speech difficulties

Physical Exam
Full neurological exam should be performed to help exclude other neurological etiologies.

Imaging
Initial Imaging Approach
CT scan preferred over MRI; obtain if:

• New-onset in patient >50 years of age
• Change in established headache pattern
• Atypical pattern or symptoms
• Prolonged or bizarre aura
• Progressive headache
• Unremitting/Progressive neurological symptoms

Diagnostic Procedures/Other
Lumbar puncture if suspicion of other CNS causes including subarachnoid bleed or meningitis

• Other primary headache syndromes (tension, cluster)
• If focal neurological signs/symptoms present, consider TIA, CVA.
• Secondary headaches: Tumor, infection, vascular pathology, prescription or illicit drug use
• Drug-seeking patients
• Psychiatric disease
• Rarely, atypical forms of epilepsy

ALERT
Geriatric Considerations

• Rare onset of non cephalalgic migraine (aura without subsequent headache) >40 years of age; possible relationship to transient global amnesia
• Late onset requires diagnostic evaluation.

Pediatric Considerations

• Attacks may be shorter and headache description atypical
• Recurrent abdominal pain and cyclic vomiting may be migraine equivalent

Pregnancy Considerations

• May decrease in 2nd and 3rd trimesters.
• No treatment drug has US Food and Drug Administration approval during pregnancy.
  • Ergotamines are contraindicated.
  • Early data for sumatriptan suggest no increase in birth defect to date (4)[B].
  • Sumatriptan and zolmitriptan recommended “pump and dump” of milk (5)[B]

First Line

• Combination of acetaminophen, aspirin, and caffeine as effective as other agents (5-HT-1 agonists (triptans)) for mild to moderate migraine with fewer adverse events; if given at onset of symptoms (6)[A]
• 5-HT-1 agonists (triptans) intervention during the mild phase of headache; contraindicated with coronary heart disease (7)[A]
• Ergotamines:
  • Dihydroergotamine (DHE): Drug of choice in status migrainous (6)[B]:
    • Most effective ergotamine; available as IV, IM, or SC injection; and nasal spray
  • Ergotamine tartrate (6)[B]: preparations contain 1 mg of ergotamine and 100 mg of caffeine.
• Nonsteroidal anti-inflammatories (NSAIDs) (6)[B]:
  • No superiority in efficacy established for any specific agent; early use improves efficacy.
• Anti-emetics: Consider anti-nausea medications that antagonize dopamine receptors.
  • Metoclopramide, prochlorperazine
• Contraindications to treatments:
  • Avoid 5-HT-1 agonists (triptans) in coronary heart disease, peripheral vascular disease, uncontrolled hypertension, complex migraine (e.g., basilar or hemiplegic migraine).
  • 5-HT-1 agonists should not be used within 24 hrs of an ergot derivative or other triptans.
  • Avoid NSAIDs if danger of gastric erosion or renal or hepatic disease.
  • Avoid narcotics or butalbital in addiction-prone patients and with frequent migraines.
  • Avoid vasoconstrictors in uncontrolled hypertension, coronary heart disease, and peripheral vascular disease.
  • Avoid sumatriptan, zolmitriptan, and rizatriptan within 2 weeks of MAOI usage.
• Precautions:
  • Frequent use of acute-treatment drugs may lead to increase in migraine patterns and medication overuse headache.

Second Line

• Use of opioids in migraine:
  • Some advocate the use of long-acting opioids in patients with refractory migraine (8)[C]
  • Shorter-acting opioids may be effective for acute relief of severe migraine (8)[C]
  • Recent retrospective study found narcotics were the most frequently used drugs for migraine in the ED with other migraine treatment used only 2% of the time (9)

General Measures

• Cold compresses to area of pain
• Rest with pillows comfortably supporting head or neck in area devoid of sensory stimulation, including light, sound, and odors
• Withdrawal from stressful surroundings
• Sleep is desirable.
• Most patients manage attacks with self-care.
• Avoid precipitants of migraine

Issue for Referral

• Obscure diagnosis, concomitant medical conditions, significant psychopathology
• Unresponsive to usual treatment
• Analgesic-dependent headache patterns

Additional Therapies

• Recent study suggests that “rescue” (parenteral) therapy in clinic reduces ED visits and total health care costs (10)[B]
• For people who have frequent migraine, consider prophylactic therapy with beta-blockers, calcium channel blockers, anti-depressants, or some anti-convulsants (11)

• Riboflavin 400 mg per day possibly effective as preventive agent
• Feverfew no more effective than placebo for the prevention of migraines (12)

Initial Stabilization
Monitor vital signs, patient comfort

Admission Criteria
Consider if diagnosis not clear, if appropriate may need to exclude acute bleeds, TIA, CVA.

IV Fluids

• Consider in setting of acute onset, severe headache
• Consider if associated with nausea/vomiting

Discharge Criteria
Judgment based on patient overall clinical status, patient's ability to tolerate PO medications

Early intervention when migraines begin

Patient Monitoring

• Early intervention to assist management
• Monitor frequency of attacks, pain behaviors, and medication usage.
• Encourage lifestyle modifications

Educate patients as to migraine triggers

• With age, reduction in severity, frequency, and disability of attacks
• Most attacks subside within 72 hours.

• Rare:
  • Status migrainous
  • Cerebral ischemic events
• Iatrogenic effects of treatment

Goadsby PJ, Lipton RB, Ferrari MD. Migraine: Current understanding and treatment. NEJM 2002;346:257–250.

See Also (Topic, Algorithm, Electronic Media Element)
Algorithm: Headache, Chronic

• 346.90 Migraine, unspecified without mention of intractable migraine
• 346.00 Classical migraine without mention of intractable migraine
• 346.10 Common migraine without mention of intractable migraine
• 346.30 Hemiplegic migraine, without mention of intractable migraine without mention of status migrainosus

• 37796009 migraine (disorder)
• 4473006 migraine with aura (disorder)
• 59292006 hemiplegic migraine (disorder)

• Migraine is a chronic headache disorder that may present very differently between people or between episodes in the same person.
• Migraine should be differentiated from other primary headache disorders and also from other CNS etiologies of headache and neurological symptoms.

Acknowledgment
I would like to credit Roger K. Cady, MD for his previous work on this chapter.

Tracy Madsen, MD

1. The International Classification of Headache Disorders. 2nd ed. Cephalalgia 2004;24:1–160.
2. Vasconcelos LP, Stancioli FG, Leal JC, et al. Ophthalmoplegic migraine: A case with recurrent palsy of the abducens nerve. Headache 2008;48(6):961–964.  [PMID:18549374]
3. Busch V, Gaul C. Exercise in migraine therapy—is there any evidence for efficacy? Headache 2008;48:890–899.  [PMID:18572431]
4. The Sumatriptan and Naratriptan Pregnancy Registry: Data from GlaxoSmithKline.
5. Hale TW. Medications and Mother's Milk. 11th ed. Amarillo, TX: Pharmasoft Pub.; 2004.
6. Matchar DB, Young WB, Rosenberg JH, et al. Evidence-based guidelines for migraine headache in the primary care setting: Pharmacological management of acute attacks. AAN Headache Guidelines. US Headache Consortium 2000:1–58.
7. Brandes JL, Kudrow D, Cady R. Eletriptan in migraine. Cephalalgia 2005;25:735.  [PMID:16109056]
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9. Sahal-Srivastava S, Desai P, Zheng L. Analysis of headache management in a busy emergency room in the United States. Headache 2008;48:931–938.  [PMID:18549371]
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11. Ramadan NM, Silberstein SD, Freitag FG, et al. Evidence-based guidelines for migraine headache in the primary care setting: Pharmacological management for prevention of migraine. AAN. US Headache Consortium 2000:1–55.
12. Pittler MH, Ernst E. Feverfew for preventing migraine. Cochrane Pain, Palliative and Supportive Care Group Cochrane Database of Systematic Reviews 2006:4.