Cluster headache: Epidemiology, clinical features, and diagnosis
- Arne May, MD
Arne May, MD
- Professor of Neurology, Department of Systems Neuroscience
- University Clinic Hamburg, Eppendorf (UKE)
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 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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- CLINICAL FEATURES
- Autonomic symptoms
- Circadian periodicity
- Diagnostic criteria
- Laboratory investigations
- DIFFERENTIAL DIAGNOSIS
- Paroxysmal hemicrania
- SUNCT syndrome
- Trigeminal neuralgia
- Primary stabbing headache
- Secondary cluster headache
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS