Headache, Cluster

Affects mainly middle-aged men

May relate to activation of cells in the ipsilateral hypothalamus, triggering the trigeminal autonomic vascular system

There is often no family history of headache or migraine

Severe unilateral periorbital pain occurring daily for several weeks

• Often accompanied by ipsilateral nasal congestion, rhinorrhea, redness of the eye, lacrimation, or Horner syndrome

Episodes often occur at night and last for between 15 minutes and 3 hours

During attacks, patients are often restless and agitated

Precipitants of an attack

• Alcohol

• Stress

• Glare

• Specific foods

Spontaneous remission occurs, and the patient remains well for weeks or months before another bout occurs

Bouts may last 4–8 weeks and may occur up to several times per year

Occasionally, remission does not occur; this variant is chronic cluster headache

Sumatriptan, 6 mg subcutaneously or 20-mg/spray intranasally, or inhalation of 100% oxygen (12–15 L/min for 15 min) may be effective

Dihydroergotamine (0.5–1 mg intramuscularly or intravenously) is sometimes used

Viscous lidocaine (1 mg of 4–6% solution) intranasally is sometimes effective

For prophylaxis, give ergotamine tartrate as rectal suppositories (0.5–1.0 mg at night or twice daily), orally (2 mg once daily), or by subcutaneous injection (0.25 mg three times daily for 5 days per week)

Other potentially helpful prophylactic agents include

• Lithium carbonate (start at 300 mg daily, titrating according to serum levels and treatment response up to a typical total daily dosage of 900–1200 mg divided 3–4 times)

• Verapamil (240–960 mg daily orally)

• Topiramate (100–400 mg daily orally)

• Ergotamine tartrate

  • 0.5–1 mg rectally at night or twice daily

  • 2 mg orally daily

  • 0.25 mg subcutaneously three times daily for 5 days per week

• Civamide (zucapsaicin 50 mg solution for intranasal administration) (not FDA approved for use in the United States)

Hemicrania continua completely resolves with indomethacin

Electrical stimulation

• Stimulation of the vagus nerve at headache onset successfully aborts pain in 30–50% of attacks

• Twice daily prophylactic stimulation reduces attack number in chronic cluster headache

• Approved in the United States

In Europe, sphenopalatine ganglion stimulation is approved for treatment of cluster headache based on efficacy in one randomized sham-controlled study

Stimulation of the occipital nerve may be helpful