Headache, Cluster

Key Features

• 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

Clinical Findings

• 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

Treatment

• 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