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Cluster headache: Epidemiology, clinical features, and diagnosis

Arne May, MD
Section Editor
Jerry W Swanson, MD, MHPE
Deputy Editor
John F Dashe, MD, PhD


Cluster headache belongs to a group of idiopathic headache entities, the trigeminal autonomic cephalalgias (TACs), all of which involve unilateral, often severe headache attacks and typical accompanying autonomic symptoms [1,2]. Cluster headache is the most prominent of these entities. The concept of the TACs is useful for clinicians seeking a pathophysiologic understanding of the primary neurovascular headaches and a rational therapeutic approach to treating or preventing these headaches. (See "Pathophysiology of the trigeminal autonomic cephalalgias".)

This review focuses on the epidemiology, clinical features, and diagnosis of cluster headache. Treatment and prognosis is discussed separately. (See "Cluster headache: Treatment and prognosis".)


The pathogenesis of cluster headache is complex and remains incompletely understood. The most widely accepted theory is that primary cluster headache is characterized by hypothalamic activation with secondary activation of the trigeminal-autonomic reflex, probably via a trigeminal-hypothalamic pathway (figure 1). Another theory holds that neurogenic inflammation of the walls of the cavernous sinus obliterates venous outflow and thus injures the traversing sympathetic fibers of the intracranial internal carotid artery and its branches. (See "Pathophysiology of the trigeminal autonomic cephalalgias".)


The prevalence of cluster headache is <1 percent and mostly affects men [3-6]. In a meta-analysis of 16 population-based epidemiologic studies, the following observations were reported [6]:

The lifetime prevalence of cluster headache for adults of all ages was 124 per 100,000 (95% CI 101-154), or approximately 0.1 percent

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Literature review current through: Nov 2017. | This topic last updated: Jul 18, 2017.
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