eMedicine Specialties > Neurology > Headache and Pain

Cluster Headache

Author: Ragasri Kumar, DO, Resident Physician, Department of Neurology, Loyola University Medical Center
Coauthor(s): Rima M Dafer, MD, MPH, FAHA, Associate Professor, Department of Neurology and Neurological Surgery, Loyola University, Chicago Stritch School of Medicine
Contributor Information and Disclosures

Updated: Sep 4, 2009

Introduction

Background

Cluster headache (CH) is a primary neurovascular primary headache disorder characterized by severe strictly unilateral, typically retro-orbital or periorbital, short-lasting headaches accompanied by prominent cranial facial parasympathetic autonomic features.1

Pathophysiology

The underlying pathophysiology of CH is not completely understood.2,3 The periodicity of the attacks in CH suggests the involvement of a biological clock within the hypothalamus, with central disinhibition of the nociceptive and autonomic pathways specifically the trigeminal nociceptive pathways. The posterior hypothalamic grey matter has been identified as the key area for the basic defect in CH by neuroimaging with positron emission tomography (PET) and anatomical imaging with voxel-based morphometry.1 Furthermore, altered habituation patterns and changes were observed within the trigeminal-facial neuronal circuitry secondary to central sensitization, in addition to dysfunction of the serotonergic raphe nuclei-hypothalamic pathways. More recently, functional hypothalamic dysfunction has recently been confirmed by abnormal metabolism based on the N-acetylaspartate neuronal marker in magnetic resonance spectroscopy.4

Frequency

United States

The exact prevalence is unknown. Kudrow estimated 0.4% in men and 0.08% in women.5

International

In an extensive study of 100,000 inhabitants of the republic of San Marino, the prevalence was 0.07%.

Race

CH has been suggested to be slightly more prevalent in the African American population.

Sex

CH is more common in men, with a male-to-female ratio of 3:1.

Age

Although it is more common in the third decade of life, CH has been reported in patients as young as 1 year and as old as 79 years.

Clinical

History

  • Attacks of cluster headache (CH) are typically short in duration (5-180 min) and occur with a frequency from once every other day to 8 times a day, particularly during sleep or early morning hours, usually corresponding with onset of REM sleep.3 As opposed to migraine, CH is not preceded by aura and is not usually associated with accompanying symptoms such as nausea, vomiting, photophobia, or osmophobia. Typically, a cluster lasts 2 weeks to 3 months.
  • Pain is described as excruciating, stabbing, sharp, and lancinating, rather than throbbing. The pain is unilateral, in the periorbital, retroorbital, or temporal regions. Sometimes, the pain radiates to the cheek, jaw, occipital, and nuchal regions. CH may rarely switch sides.
  • Pain is accompanied by cranial parasympathetic symptoms including ipsilateral lacrimation, conjunctival injection, rhinorrhea, eyelid edema, ptosis, and miosis.3
  • Alcoholic products and tobacco may precipitate an attack.
  • Some triggers include hot weather, watching television, nitroglycerin, stress, relaxation, extreme temperatures, glare, allergic rhinitis, and sexual activity.
  • During an attack of CH, patients may become agitated and extremely restless. Patients do not like to lie down to rest; instead, they are restless and prefer to pace or move around. In desperation, patients may rock, sit, pace, bang themselves against a hard surface, scream in pain, or crawl on the floor.
  • Classification of CH: The International Headache Society (IHS) classifies CH by duration as episodic or chronic.6
    • Episodic CH occurs in periods lasting from 7 days to 1 year; cluster attacks are separated by pain-free intervals lasting at least 2 weeks.
    • Chronic CH is defined as that occurring for more than 1 year without remission or with remissions lasting less than 2 weeks. It is subdivided into chronic CH from onset and chronic CH evolving from episodic.
  • Structural lesions have been described with CH and should be suspected if the presentation is atypical. Atypical features may include the following:
    • Absence of a periodic pattern
    • Residual headache between exacerbations
    • Bilaterality
    • Incomplete or minimal response to standard therapy
    • Presence of lateralizing findings on examination (other than Horner syndrome) 
  • Patients with CH have increased risk of suicide attempts, alcohol use, cigarette smoking, and peptic ulcer disease.

Physical

  • The association of prominent autonomic phenomena is a hallmark of cluster headache (CH). Such signs include ipsilateral nasal congestion and rhinorrhea, lacrimation, conjunctival hyperemia, facial diaphoresis, palpebral edema, and complete or partial Horner syndrome (which may persist between attacks). Tachycardia is a frequent finding.
  • A distinctive CH face is described as follows: leonine facial appearance, multifurrowed and thickened skin with prominent folds, a broad chin, vertical forehead creases, and nasal telangiectasias.

Causes

  • The exact cause of cluster headache (CH) is unknown.
  • The disorder is sporadic, although rare cases of an autosomal dominant pattern within a single family have been reported.

More on Cluster Headache

Overview: Cluster Headache
Differential Diagnoses & Workup: Cluster Headache
Treatment & Medication: Cluster Headache
Follow-up: Cluster Headache
References
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References

  1. Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol. Aug 2002;1(4):251-7. [Medline].

  2. Holle D, Obermann M, Katsarava Z. The electrophysiology of cluster headache. Curr Pain Headache Rep. Apr 2009;13(2):155-9. [Medline].

  3. Mendizabal JE, Umana E, Zweifler RM. Cluster headache: Horton's cephalalgia revisited. South Med J. Jul 1998;91(7):606-17. [Medline].

  4. Lodi R, Pierangeli G, Tonon C, et al. Study of hypothalamic metabolism in cluster headache by proton MR spectroscopy. Neurology. 2006;66(8):1624-6. [Medline].

  5. Kudrow L. Cluster headache: diagnosis and management. Headache. Apr 1979;19(3):142-50. [Medline].

  6. Mathew NT. Cluster Headache. Neurology. 1992;42 (suppl 2):22-31. [Medline].

  7. Peterlin BL, Levin M, Cohen JA, Ward TN. Secondary cluster headache: a presentation of cerebral thrombosis. Cephalalgia. 2006;26(8):1022-4. [Medline].

  8. Rozen TD. Trigeminal autonomic cephalalgias. Neurol Clin. May 2009;27(2):537-56. [Medline].

  9. Sewell RA. Response of cluster headache to kudzu. Headache. Jan 2009;49(1):98-105. [Medline].

  10. Ailani J, Young WB. The role of nerve blocks and botulinum toxin injections in the management of cluster headaches. Curr Pain Headache Rep. Apr 2009;13(2):164-7. [Medline].

  11. Franzini A, Ferroli P, Leone M, Broggi G. Stimulation of the posterior hypothalamus for treatment of chronic intractable cluster headaches: first reported series. Neurosurgery. May 2003;52(5):1095-9; discussion 1099-101. [Medline].

  12. Leone M, Proietti Cecchini A, Franzini A, et al. Lessons from 8 years' experience of hypothalamic stimulation in cluster headache. Cephalalgia. Jul 2008;28(7):787-97; discussion 798. [Medline].

  13. Leone M, Franzini A, Broggi G, Bussone G. Hypothalamic stimulation for intractable cluster headache. Neurology. 2006;67(1):150-2. [Medline].

  14. May A, Leone M. Update on cluster headache. Curr Opin Neurol. Jun 2003;16(3):333-40. [Medline].

  15. Leone M. Deep brain stimulation in headache. Lancet Neurol. Oct 2006;5(10):873-7. [Medline].

  16. Narouze S, Kapural L, Casanova J, Mekhail N. Sphenopalatine ganglion radiofrequency ablation for the management of chronic cluster headache. Headache. Apr 2009;49(4):571-7. [Medline].

  17. Giraud P, Chauvet S. Cluster headache during pregnancy: case report and literature review. Headache. Jan 2009;49(1):136-9. [Medline].

  18. Boes CJ, Capobianco DJ, Cutrer FM, Dodick DW, Eross EJ, Swanson JW. Headache and Other Crainofacial Pain. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J. Neurology in Clinical Practice: The Neurological Disorders. Vol 2. 4th ed. Philadelphia, Pa: Butterworth Heinemann; 2004:2090-1.

  19. Ekbom K, Nappi G. Diagnosis, differential diagnosis, and prognosis of cluster headache. In: The Headaches. 1993:585-9.

  20. Lance JW. Headache: Mechanism and Management. 5th ed. Boston, Mass: Butterworth-Heinemann; 1993.

  21. Rozen TD. Trigeminal Autonomic Cephalgias. Neurology Continium. December 2006;12:170-193.

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Further Reading

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Keywords

cluster headache, CH, Bing-Horton syndrome, histaminic cephalalgia, cluster migraine, paroxysmal nocturnal cephalalgia, red migraine, erythromelalgia of the head, sphenopalatine neuralgia, migrainous neuralgia, periorbital pain, Horton’s headache

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Contributor Information and Disclosures

Author

Ragasri Kumar, DO, Resident Physician, Department of Neurology, Loyola University Medical Center
Disclosure: Nothing to disclose.

Coauthor(s)

Rima M Dafer, MD, MPH, FAHA, Associate Professor, Department of Neurology and Neurological Surgery, Loyola University, Chicago Stritch School of Medicine
Rima M Dafer, MD, MPH, FAHA is a member of the following medical societies: American Academy of Neurology, American Headache Society, and American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Joseph Carcione Jr, DO, MBA, Consultant in Neurology and Medical Acupuncture, Medical Management and Organizational Consulting, Central Westchester Neuromuscular Care, PC; Medical Director, Oxford Health Plans
Joseph Carcione Jr, DO, MBA is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

James H Halsey, MD, Professor, Department of Neurology, University of Alabama Medical Center
James H Halsey, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neuroimaging, Medical Association of the State of Alabama, New York Academy of Sciences, Pan American Medical Association, Sigma Xi, Society for Neuroscience, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Howard A Crystal, MD, Professor, Departments of Neurology and Pathology, State University of New York Downstate; Consulting Staff, Department of Neurology, University Hospital and Kings County Hospital Center
Howard A Crystal, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association
Disclosure: Medivations Honoraria Consulting

 
 
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