(See also Approach to the Patient With Headache Approach to the Patient With Headache Headache is pain in any part of the head, including the scalp, face (including the orbitotemporal area), and interior of the head. Headache is one of the most common reasons patients seek medical... read more .)
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 months, 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 and Signs of Cluster Headache
Symptoms of cluster headache are distinctive. Attacks, often multiple, frequently 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 minutes to 1 hour. 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 Horner Syndrome Horner syndrome is ptosis, miosis, and anhidrosis due to dysfunction of cervical sympathetic output. (See also Overview of the Autonomic Nervous System.) Horner syndrome results when the cervical... read more , are prominent and occur on the same side as the headache.
Diagnosis of Cluster 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 (SUNCT) Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare headache disorder characterized by extremely frequent attacks of unilateral head pain... read more (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing): Attacks are very brief (5 to 250 seconds) 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 Migraine Migraine is an episodic primary headache disorder. Symptoms typically last 4 to 72 hours and may be severe. Pain is often unilateral, throbbing, worse with exertion, and accompanied by symptoms... read more ), respond dramatically to indomethacin, but not to other nonsteroidal anti-inflammatory drugs (NSAIDs).
Treatment of Cluster Headache
For aborting attacks, parenteral triptans, dihydroergotamine, or 100% oxygen
For long-term prophylaxis, verapamil, lithium, topiramate, divalproex, or a combination or galcanezumab (a monoclonal antibody) for episodic cluster
Acute attacks of cluster headache can be aborted with either a parenteral triptan or dihydroergotamine (see table Drugs for Migraine and Cluster Headaches Drugs for Cluster Headaches ), zolmitriptan taken by nasal spray, and/or 100% oxygen given by nonrebreathing face mask. Also, evidence suggests that acute attacks of episodic cluster headache can be treated with a handheld device that delivers noninvasive vagus nerve stimulation Vagus nerve stimulation A seizure is an abnormal, unregulated electrical discharge that occurs within the brain’s cortical gray matter and transiently interrupts normal brain function. A seizure typically causes altered... read more ( 1 Treatment reference Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical... read more , 2 Treatment reference Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical... read more ).
All patients require preventive drugs because cluster headache is frequent, severe, and incapacitating. Prednisone (eg, 60 mg orally once a 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.
Noninvasive transcutaneous supraorbital nerve stimulation delivered using a device applied to the forehead can reduce the frequency of cluster headaches, as can noninvasive vagus nerve stimulation ( 3 Treatment reference Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical... read more ).
1. Silberstein SD, Mechtler LL, Kudrow DB, et al: Non-invasive vagus nerve stimulation for the acute treatment of cluster headache: Findings from the randomized, double-blind, sham-controlled ACT1 study. Headache 56 (8):1317–1332, 2016. doi: 10.1111/head.12896.
2. Miller S, Sinclair AJ, Davies B, Matharu M: Neurostimulation in the treatment of primary headaches. Pract Neurol 16 (5):362–375, 2016. doi: 10.1136/practneurol-2015-001298. Epub 2016 May 5.
3. Gaul C, Diener H, Solbach K, et al: EHMTI-0364. Non-invasive vagus nerve stimulation using Gammacore® for prevention and acute treatment of chronic cluster headache: Report from the randomized phase of the PREVA study. J Headache and Pain 15 (suppl 1):I7, 2014.
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 years.
Usually, patients experience ≥ 1 attack/day for 1 to 3 months, followed by remission for months to years.
Diagnose cluster headache based on clinical findings.
To prevent attacks, use prednisone or a greater occipital nerve block for short-term relief and verapamil, lithium, topiramate, and/or galcanezumab for long-term relief.
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