* This is the Professional Version. *
(See also Approach to the Patient With Headache.)
Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical. Acute treatment is with parenteral triptans, dihydroergotamine, or oxygen. Prevention is with verapamil, lithium, topiramate, divalproex, or a combination.
Cluster headache affects primarily men, typically beginning at age 20 to 40; prevalence in the US is 0.4%. Usually, cluster headache is episodic; for 1 to 3 mo, patients experience ≥ 1 attack/day, followed by remission for months to years. Some patients have cluster headaches without remission.
Pathophysiology is unknown, but the periodicity suggests hypothalamic dysfunction.
Alcohol intake triggers cluster headache during the attack period but not during remission.
Symptoms of cluster headache are distinctive. Attacks often occur at the same time each day, often awakening patients from sleep. When attacks occur, pain is always unilateral and occurs on the same side of the head in an orbitotemporal distribution. It is excruciating, peaking within minutes; it usually subsides spontaneously within 30 min to 1 h. Patients are agitated, restlessly pacing the floor, unlike migraine patients who prefer to lie quietly in a darkened room. The restlessness can be so severe that it leads to bizarre behavior (eg, banging the head on a wall).
Autonomic features, including nasal congestion, rhinorrhea, lacrimation, facial flushing, and Horner syndrome, are prominent and occur on the same side as the headache.
Diagnosis of cluster headache is based on the distinctive symptom pattern and exclusion of intracranial abnormalities.
Other unilateral primary headache syndromes with autonomic symptoms, which are sometimes grouped together with cluster headache as trigeminal autonomic cephalgias, should be excluded:
SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing): Attacks are very brief (5 to 250 sec) and occur at high frequency (up to 200 attacks/day).
Chronic paroxysmal hemicrania: Attacks are more frequent (> 5/day) and much briefer (usually just minutes) than in cluster headache.
Hemicrania continua: Moderately severe continuous unilateral head pain occurs with superimposed brief episodes of more intense pain.
Chronic paroxysmal hemicrania and hemicrania continua, unlike SUNCT and cluster headache (and migraine), respond dramatically to indomethacin, but not to other NSAIDs.
Acute attacks of cluster headache can be aborted with either a parenteral triptan or dihydroergotamine (see Table: Drugs for Migraine and Cluster Headaches*) and/or 100% oxygen given by nonrebreathing face mask.
All patients require preventive drugs because cluster headache is frequent, severe, and incapacitating. Prednisone (eg, 60 mg po once/day) or a greater occipital nerve block (with a local anesthetic and a corticosteroid) can provide prompt temporary prevention while preventive drugs with slower onset of action (eg, verapamil, lithium, topiramate, divalproex) are initiated.
Drugs for Migraine and Cluster Headaches*
Typically, cluster headache causes excruciating unilateral periorbital or temporal pain, with ipsilateral ptosis, lacrimation, rhinorrhea, and/or nasal congestion, in men aged 20 to 40 yr.
Usually, patients experience ≥ 1 attack/day for 1 to 3 mo, followed by remission for months to years.
Diagnose cluster headache based on clinical findings.
To abort attacks, give a parenteral triptan or dihydroergotamine (see Table: Drugs for Migraine and Cluster Headaches*) and/or 100% oxygen by a nonrebreathing face mask.
To prevent attacks, use prednisone or a greater occipital nerve block for short-term relief and verapamil, lithium, topiramate, and/or divalproex for long-term relief.
* This is the Professional Version. *