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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: Sep 2016. | This topic last updated: May 22, 2014.
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  1. Nesbitt AD, Goadsby PJ. Cluster headache. BMJ 2012; 344:e2407.
  2. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.
  3. Russell MB. Epidemiology and genetics of cluster headache. Lancet Neurol 2004; 3:279.
  4. Bahra A, May A, Goadsby PJ. Cluster headache: a prospective clinical study with diagnostic implications. Neurology 2002; 58:354.
  5. Sjaastad O, Bakketeig LS. Cluster headache prevalence. Vågå study of headache epidemiology. Cephalalgia 2003; 23:528.
  6. Fischera M, Marziniak M, Gralow I, Evers S. The incidence and prevalence of cluster headache: a meta-analysis of population-based studies. Cephalalgia 2008; 28:614.
  7. Ekbom K, Svensson DA, Träff H, Waldenlind E. Age at onset and sex ratio in cluster headache: observations over three decades. Cephalalgia 2002; 22:94.
  8. Manzoni GC. Gender ratio of cluster headache over the years: a possible role of changes in lifestyle. Cephalalgia 1998; 18:138.
  9. Lin KH, Wang PJ, Fuh JL, et al. Cluster headache in the Taiwanese -- a clinic-based study. Cephalalgia 2004; 24:631.
  10. Manzoni GC. Cluster headache and lifestyle: remarks on a population of 374 male patients. Cephalalgia 1999; 19:88.
  11. Sjaastad O, Shen JM, Stovner LJ, Elsås T. Cluster headache in identical twins. Headache 1993; 33:214.
  12. Russell MB, Andersson PG, Thomsen LL. Familial occurrence of cluster headache. J Neurol Neurosurg Psychiatry 1995; 58:341.
  13. El Amrani M, Ducros A, Boulan P, et al. Familial cluster headache: a series of 186 index patients. Headache 2002; 42:974.
  14. Leone M, Russell MB, Rigamonti A, et al. Increased familial risk of cluster headache. Neurology 2001; 56:1233.
  15. Russell MB, Andersson PG, Thomsen LL, Iselius L. Cluster headache is an autosomal dominantly inherited disorder in some families: a complex segregation analysis. J Med Genet 1995; 32:954.
  16. Lampl C. Childhood-onset cluster headache. Pediatr Neurol 2002; 27:138.
  17. Evers S, Frese A, Majewski A, et al. Age of onset in cluster headache: the clinical spectrum (three case reports). Cephalalgia 2002; 22:160.
  18. Rozen TD. Childhood exposure to second-hand tobacco smoke and the development of cluster headache. Headache 2005; 45:393.
  19. Black DF, Swanson JW, Stang PE. Decreasing incidence of cluster headache: a population-based study in Olmsted County, Minnesota. Headache 2005; 45:220.
  20. Manzoni GC, Terzano MG, Bono G, et al. Cluster headache--clinical findings in 180 patients. Cephalalgia 1983; 3:21.
  21. May A. Cluster headache: pathogenesis, diagnosis, and management. Lancet 2005; 366:843.
  22. Drummond PD. Dysfunction of the sympathetic nervous system in cluster headache. Cephalalgia 1988; 8:181.
  23. Drummond PD. Sweating and vascular responses in the face: normal regulation and dysfunction in migraine, cluster headache and harlequin syndrome. Clin Auton Res 1994; 4:273.
  24. Drummond PD. Mechanisms of autonomic disturbance in the face during and between attacks of cluster headache. Cephalalgia 2006; 26:633.
  25. Ekbom K. Evaluation of clinical criteria for cluster headache with special reference to the classification of the International Headache Society. Cephalalgia 1990; 10:195.
  26. Sjaastad, O (Ed). Cluster Headache Syndrome. W B Saunders Company Ltd, London 1992.
  27. Sandrini G, Friberg L, Jänig W, et al. Neurophysiological tests and neuroimaging procedures in non-acute headache: guidelines and recommendations. Eur J Neurol 2004; 11:217.
  28. Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 1997; 120 ( Pt 1):193.
  29. Goadsby PJ, Matharu MS, Boes CJ. SUNCT syndrome or trigeminal neuralgia with lacrimation. Cephalalgia 2001; 21:82.
  30. Favier I, van Vliet JA, Roon KI, et al. Trigeminal autonomic cephalgias due to structural lesions: a review of 31 cases. Arch Neurol 2007; 64:25.