Cluster Headache

Cluster Headache

Cluster headache affects predominantly middle-aged men. The pathophysiology is unclear but 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. Episodes of severe unilateral periorbital pain occur daily for several weeks and are often accompanied by one or more of the following: ipsilateral nasal congestion, rhinorrhea, lacrimation, redness of the eye, and Horner syndrome. During attacks, patients are often restless and agitated. Episodes often occur at night, awaken the patient, and last for between 15 minutes and 3 hours. Spontaneous remission then occurs, and the patient remains well for weeks or months before another bout of closely spaced attacks occurs. Bouts may last for 4 to 8 weeks and may occur up to several times per year. During a bout, many patients report that alcohol triggers an attack; others report that stress, glare, or ingestion of specific foods occasionally precipitates attacks. In occasional patients, typical attacks of pain and associated symptoms recur at intervals without remission. This variant has been referred to as chronic cluster headache.

Examination reveals no abnormality apart from Horner syndrome that either occurs transiently during an attack or, in longstanding cases, remains as a residual deficit between attacks.

Treatment of an individual attack with oral drugs is generally unsatisfactory, but subcutaneous (6 mg dose) or intranasal (20-mg/spray) sumatriptan or inhalation of 100% oxygen (12–15 L/min for 15 minutes via a non-rebreather mask) may be effective. Zolmitriptan (5- and 10-mg nasal spray) is also effective. Dihydroergotamine (0.5–1 mg intramuscularly or intravenously) is sometimes used. Viscous lidocaine (1 mg of 4–6% solution) intranasally is sometimes effective.

Various prophylactic agents that have been found to be effective in individual patients are cyproheptadine, lithium carbonate (monitored by plasma lithium determination), verapamil (240–960 mg daily), topiramate (100–400 mg daily), valproate (750–1500 mg daily), and methysergide (2–12 mg daily). As there is often a delay before these medications are effective, transitional therapy is often used. Ergotamine tartrate is effective and can be given as rectal suppositories (0.5–1 mg at night or twice daily), by mouth (2 mg daily), or by subcutaneous injection (0.25 mg three times daily for 5 days per week). Other options include prednisone (60 mg daily for 5 days followed by gradual withdrawal), dihydroergotamine (9.25 mg intravenously over several days or 0.5 mg intramuscularly twice daily), or greater occipital nerve injection with lidocaine and corticosteroid.

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