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- Primary headache disorder
- Multiple attacks of unilateral, excruciating, sharp, searing, or piercing pain; typically localized in the periorbital area and temple accompanied by signs of ipsilateral parasympathetic autonomic features, along with restlessness and agitation
- Autonomic symptoms: parasympathetic hyperactivity signs (ipsilateral lacrimation, eye redness, nasal congestion) and sympathetic hypoactivity (ipsilateral ptosis and miosis)
- Patients often pace the floor during an acute attack, as lying down seems to exacerbate the pain.
- Symptoms usually remain on the same side during a single cluster attack.
- Individual attacks last 15 to 180 minutes if untreated and occur from once every other day to 8 times per day.
- Attacks usually occur in series (cluster periods) that are often seasonal, lasting for weeks or months; separated by remission periods usually lasting months or years
- About 10–15% of patients have chronic symptoms without remissions (i.e., chronic cluster headache [CH]).
1-year incidence: 2 to 10/100,000
- Lifetime prevalence: 124/100,000
- Predominant sex: male > female; 2.1:1 overall
- Women often develop earlier in life (20s).
- Mean age of onset: 30 years
- Episodic/chronic ratio: 6:1
Etiology and Pathophysiology
- Complex and incompletely understood
- Proposed mechanism include the following:
- Posterior hypothalamus activation may trigger an attack by activating trigeminal nociceptive pathways through perivascular activation and increased parasympathetic outflow, causing unilateral pain.
- Usually sporadic: autosomal dominant in 5% of cases; autosomal recessive or multifactorial in other families
- Evidence varies: first-degree relatives carry 5- to 8-fold; second degree, 1- to 3-fold increased relative risk of disease
- >50% with migraine and 18% with CH in family history
- Male gender
- Age (70% onset before age 30 years)
- Cigarette smoking or childhood exposure to cigarette smoke
- Family history of CH
- Head trauma
Commonly Associated Conditions
- Depression (24%)
- Increased risk of suicide secondary to the extreme nature of the pain
- Medication-overuse headache
- Asthma (9%)
- History of migraine, frequently in female patients
- Sleep apnea (30–80%)
- Increased prevalence of cardiac right-to-left shunt and patent foramen ovale (relationship unclear)