Cluster Headache (Migrainous Neuralgia)

Cluster headache affects predominantly middle-aged men. Its cause is unclear but may relate to a vascular headache disorder or a disturbance of serotonergic mechanisms. Activation of cells in the ipsilateral hypothalamus has been shown to occur. 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's syndrome. Episodes often occur at night, awaken the patient, and last for less than 2 hours. Spontaneous remission then occurs, and the patient remains well for weeks or months before another bout of closely spaced attacks occurs. 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's 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 (7 L/min for 15 minutes) may be effective. Dihydroergotamine (1–2 mg intramuscularly or intravenously) is sometimes used. Butorphanol tartrate, a synthetic opioid agonist-antagonist administered by nasal spray, may also be helpful. The dose is 1 mg (one spray in one nostril), repeated after 60–90 minutes if necessary. Ergotamine tartrate is an effective prophylactic 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). Various prophylactic agents that have been found to be effective in individual patients are valproate, cyproheptadine, lithium carbonate (monitored by plasma lithium determination), prednisone (20–40 mg daily or on alternate days for 2 weeks, followed by gradual withdrawal), and verapamil (240–480 mg daily). Topiramate (25–200 mg daily) or valproate (750–1500 mg daily) may also be useful in some instances.