Cluster Headache: Rapid Evidence Review

 

Am Fam Physician. 2022 Jan ;105(1):24-32.

  Patient information: See related handout on cluster headaches, written by the authors of this article.

Author disclosure: No relevant financial affiliations.

Cluster headache, the most common form of trigeminal autonomic cephalgia, is a rare primary headache disorder that affects less than 1% of the population. The mean age of onset is 30 years, and it is two to three times more common in males. Cluster headache consists of attacks of severe unilateral pain located in the orbital, supraorbital, and/or temporal region that occur from every other day up to eight times per day and last from 15 to 180 minutes. The pain is associated with ipsilateral autonomic symptoms (most commonly lacrimation, conjunctival injection, nasal congestion or rhinorrhea, ptosis, edema of the eyelid, sweating of the forehead or face, and miosis) and a sense of agitation or restlessness. Attacks occur in clusters, called bouts, and are episodic or chronic. Common triggers include alcohol, nitroglycerin, food containing nitrates, and strong odors. Abortive treatments include triptans and oxygen; transitional treatments include steroids and suboccipital steroid injections; and prophylactic treatments include verapamil, lithium, melatonin, and topiramate. Newer treatments for cluster headache include galcanezumab, neurostimulation, and somatostatin receptor agonists.

Cluster headache is the most common form of trigeminal autonomic cephalgia, and it affects less than 1% of the population. It is characterized by attacks of severe unilateral pain located in the orbital, supraorbital, and/or temporal region. This article provides a review of patient-oriented evidence to guide the diagnosis and management of cluster headache.

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SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComments

A sense of restlessness is one key feature of cluster headache and should be elicited in the history.1,3

C

Consensus statement

First-line abortive therapies for cluster headache include 100% supplemental oxygen, subcutaneous sumatriptan (Imitrex), sumatriptan nasal spray, and intranasal zolmitriptan (Zomig).16,1922,2426

A

Good quality meta-analyses and randomized controlled trials

Suboccipital steroid injection may be considered for transitional therapy to provide time for prophylactic therapy to take full effect.14,32,33

A

Good quality randomized controlled trials

First-line prophylactic therapy for cluster headache is verapamil.12,34,37

A

Good quality randomized controlled trials


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComments

A sense of restlessness is one key feature of cluster headache and should be elicited in the history.1,3

C

Consensus statement

First-line abortive therapies for cluster headache include 100% supplemental oxygen, subcutaneous sumatriptan (Imitrex), sumatriptan nasal spray, and intranasal zolmitriptan (Zomig).16,1922,2426

A

Good quality meta-analyses and randomized controlled trials

Suboccipital steroid injection may be considered for transitional therapy to provide time for prophylactic therapy to take full effect.14,32,33

A

Good quality randomized controlled trials

First-line prophylactic therapy for cluster headache is verapamil.12,34,37

A

Good quality randomized controlled trials


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

Epidemiology and Risk Factors

  • Typical age of onset is 20 to 40 years 1,2; it is two to three times more common in men than women.3

  • Lifetime prevalence is 124 per 100,000.4

  • Having a first-degree relative with cluster headache increases the patient's risk five to 18 times, whereas having a second-degree relative with cluster headache increases the risk one to three times.5

Diagnosis

  • Cluster headache is a clinical diagnosis based on the criteria described in Table 1.2

  • Cluster headache patterns are episodic or chronic. Headache episodes that last seven days to one year with remission periods lasting three months or longer are episodic. When episodes last for one year or longer with remission periods of less than three months, they are categorized as chronic.1

  • A suggested approach to the evaluation of acute headache is summarized in Figure 1.1,2,6

The Authors

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OMOJO ODIHI MALU, MD, MSc, is a staff physician at DiLorenzo TRICARE Health Clinic at the Pentagon, Washington, D.C....

JONATHAN BAILEY, MD, is the director for Medical Services at DiLorenzo TRICARE Health Clinic at the Pentagon.

MATTHEW KENDALL HAWKS, MD, FAAFP, is an associate professor in the Department of Family Medicine at the Uniformed Services University of the Health Sciences, Bethesda, Md.

Address correspondence to Omojo Odihi Malu, MD, MSc, 9300 DeWitt Loop, Fort Belvoir, VA 22060 (email: oodihi@gmail.com). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

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