Cluster Headache 

  • Author: Ragasri Kumar, DO; Chief Editor: Howard A Crystal, MD   more...
 
Updated: Jun 22, 2011
 

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]

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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]

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Epidemiology

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.

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Proceed to Clinical Presentation
 
 
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.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

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: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

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: Nothing to disclose.

References

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  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].

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  13. 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].

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  17. Fontaine D, Lazorthes Y, Mertens P, Blond S, Géraud G, Fabre N. Safety and efficacy of deep brain stimulation in refractory cluster headache: a randomized placebo-controlled double-blind trial followed by a 1-year open extension. J Headache Pain. Feb 2010;11(1):23-31. [Medline].

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  19. 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].

  20. Calhoun AH, Peterlin BL. Treatment of cluster headache in pregnancy and lactation. Curr Pain Headache Rep. Apr 2010;14(2):164-73. [Medline].

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

  22. 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.

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

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

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

 
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