Headache, Cluster

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Basics

Description

  • A primary headache disorder characterized by 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 because 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 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 [cCH]).

Epidemiology

Incidence
1-year incidence: 2 to 10/100,000

Prevalence
  • 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
  • Possible mechanisms include the following:
    • Posterior hypothalamus activation may trigger an attack by activating trigeminal nociceptive pathways through perivascular activation and increased parasympathetic outflow
    • Alterations in the descending pain modulation network and disordered pain modulation during cluster periods

Genetics
  • 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 and second degree 1- to 3-fold increased relative risk of disease.
  • >50% with migraine and 18% with CH in family history

Risk Factors

  • Male gender
  • Age: 70% onset before age 30 years
  • Cigarette smoking or childhood exposure to cigarette smoke
  • Family history of CH
  • Personal history of 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%)

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Basics

Description

  • A primary headache disorder characterized by 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 because 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 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 [cCH]).

Epidemiology

Incidence
1-year incidence: 2 to 10/100,000

Prevalence
  • 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
  • Possible mechanisms include the following:
    • Posterior hypothalamus activation may trigger an attack by activating trigeminal nociceptive pathways through perivascular activation and increased parasympathetic outflow
    • Alterations in the descending pain modulation network and disordered pain modulation during cluster periods

Genetics
  • 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 and second degree 1- to 3-fold increased relative risk of disease.
  • >50% with migraine and 18% with CH in family history

Risk Factors

  • Male gender
  • Age: 70% onset before age 30 years
  • Cigarette smoking or childhood exposure to cigarette smoke
  • Family history of CH
  • Personal history of 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%)

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