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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 (not available in the United States)

  • Hemicrania continua completely resolves with indomethacin

  • Stimulation of the occipital nerve may be helpful

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