Introduction
Background
Cluster headache (CH) is an idiopathic syndrome consisting of recurrent brief attacks of sudden, severe, unilateral periorbital pain.
Pathophysiology
The pathophysiology of CH is not entirely understood. Its typical periodicity has been attributed to hypothalamic (particularly suprachiasmatic nuclei) hormonal influences. More recently, functional neuroimaging with positron emission tomography (PET) and anatomical imaging with voxel-based morphometry have identified the posterior hypothalamic grey matter as the key area for the basic defect in CH. Hypothalamic dysfunction has recently been confirmed by abnormal metabolism based on the N -acetylaspartate neuronal marker in magnetic resonance spectroscopy.
CH pain is thought to be generated at the level of the pericarotid/cavernous sinus complex. This region receives sympathetic and parasympathetic input from the brain stem, possibly mediating occurrence of autonomic phenomena during an attack. The exact roles of immunologic and vasoregulatory factors, as well as the influence of hypoxemia and hypocapnia, in CH are still controversial.
Frequency
United States
The exact prevalence is unknown. Kudrow estimated 0.4% in men and 0.08% in women.
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 more prevalent in the black population.
Sex
CH is more common in men than in women; the male-to-female ratio is 5:1.
Age
CH affects middle-aged persons.
Clinical
History
- Attacks of 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. As opposed to migraine, CH is not preceded by aura, affording patients little or no warning.
- Pain generally is described as excruciating, penetrating, and not throbbing.
- It may radiate to other areas of the face and neck but is typically periorbital.
- It may be triggered by stress, relaxation, extreme temperatures, glare, allergic rhinitis, and sexual activity.
- CH rarely is triggered by ingestion of specific foods, although tobacco or alcohol products may precipitate an attack.
- An attack of CH is a dramatic event during which the patient may be extremely restless. In desperation, CH patients may rock, sit, pace, or bang themselves against a hard surface.
- Classification of CH: The International Headache Society (IHS) classifies CH by duration as episodic or chronic.
- Episodic CH occurs in periods (clusters) lasting from 7 days to 1 year; clusters are separated by pain-free intervals lasting at least 2 weeks. Typically, a cluster lasts 2 weeks to 3 months.
- 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.
- Chronic CH is notoriously difficult to treat and resistant to standard prophylactic agents.
- Symptomatic clusterlike headache should be suspected if the presentation is atypical. Atypical features may include the following:
- Absence of a periodic pattern
- Residual headache between exacerbations
- Incomplete or minimal response to standard therapy
- Presence of lateralizing findings on exam (except for those of CH-related Horner syndrome)
Physical
The association of prominent autonomic phenomena is a hallmark of 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.
- Persons affected by CHs often are tall and rugged-looking.
Causes
Cases of CH affecting multiple members with an autosomal dominant pattern within a single family have been reported, suggesting that a genetic predisposition may exist in those families. Complex segregation analysis, however, has consistently resulted in a sporadic model of inheritance.
More on Cluster Headache |
 Overview: Cluster Headache |
| Differential Diagnoses & Workup: Cluster Headache |
| Treatment & Medication: Cluster Headache |
| Follow-up: Cluster Headache |
| References |
| Next Page » |
References
Ekbom K, Nappi G. Diagnosis, differential diagnosis, and prognosis of cluster headache. In: The Headaches. 1993;585-589. [Medline].
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].
Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurol. Aug 2002;1(4):251-7. [Medline].
Kudrow L. Cluster headache: diagnosis and management. Headache. Apr 1979;19(3):142-50. [Medline].
Lance JW. Headache: Mechanism and Management. 5th ed. Boston, Mass: Butterworth-Heinemann; 1993.
Leone M, Franzini A, Broggi G, Bussone G. Hypothalamic stimulation for intractable cluster headache. Neurology. 2006;67(1):150-2. [Medline].
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].
Mathew NT. Cluster Headache. Neurology. 1992;42 (suppl 2):22-31. [Medline].
May A, Leone M. Update on cluster headache. Curr Opin Neurol. Jun 2003;16(3):333-40. [Medline].
Mendizabal JE, Umana E, Zweifler RM. Cluster headache: Horton''s cephalalgia revisited. South Med J. Jul 1998;91(7):606-17. [Medline].
Peterlin BL, Levin M, Cohen JA, Ward TN. Secondary cluster headache: a presentation of cerebral thrombosis. Cephalalgia. 2006;26(8):1022-4. [Medline].
Further Reading
Keywords
CH, Bing-Horton syndrome, histaminic cephalalgia, cluster migraine, paroxysmal nocturnal cephalalgia, red migraine, erythromelalgia of the head, sphenopalatine neuralgia, migrainous neuralgia, periorbital pain
