Excerpt from Cluster Headache

Synonyms, Key Words, and Related Terms: CH, Bing-Horton syndrome, histaminic cephalalgia, cluster migraine, paroxysmal nocturnal cephalalgia, red migraine, erythromelalgia of the head, sphenopalatine neuralgia, migrainous neuralgia, periorbital pain

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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.

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