Headache, Cluster

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Basics

Description

  • A primary headache disorder characterized by multiple attacks of severe, unilateral sharp, searing, or piercing pain typically localized to the periorbital and/or temporal areas
  • Accompanied by signs of ipsilateral parasympathetic autonomic activation as well as restlessness or agitation
  • Autonomic symptoms: signs of parasympathetic hyperactivity (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.
  • Individual attacks occur from once every other day up to 8 times per day and can last 15 to 180 minutes per day if untreated.
  • Attacks usually occur in series (cluster periods) that are often seasonal, lasting for weeks or months, and are separated by remission periods usually lasting months to years.
  • About 10–15% of patients have chronic symptoms without remissions (i.e., chronic cluster headache [cCH]).

Epidemiology

Incidence
Incidence: 2 to 10/100,000

Prevalence
Prevalence: 53/100,000

  • Gender: male > female; 4.3:1 overall
  • Women often develop CH 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:
    • Activation of the trigeminovascular system, which leads to the release of vasodilatory peptides including substance P, neurokinin, and calcitonin gene-related peptide (CGRP)
    • Posterior hypothalamus activation may trigger an attack by activating trigeminal nociceptive pathways through 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; otherwise, autosomal recessive or multifactorial
  • Evidence varies: First-degree relatives are 18 times more likely, and second-degree relatives are 1 to 3 times more likely to be affected by cluster headaches.
  • >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 severe, unilateral sharp, searing, or piercing pain typically localized to the periorbital and/or temporal areas
  • Accompanied by signs of ipsilateral parasympathetic autonomic activation as well as restlessness or agitation
  • Autonomic symptoms: signs of parasympathetic hyperactivity (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.
  • Individual attacks occur from once every other day up to 8 times per day and can last 15 to 180 minutes per day if untreated.
  • Attacks usually occur in series (cluster periods) that are often seasonal, lasting for weeks or months, and are separated by remission periods usually lasting months to years.
  • About 10–15% of patients have chronic symptoms without remissions (i.e., chronic cluster headache [cCH]).

Epidemiology

Incidence
Incidence: 2 to 10/100,000

Prevalence
Prevalence: 53/100,000

  • Gender: male > female; 4.3:1 overall
  • Women often develop CH 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:
    • Activation of the trigeminovascular system, which leads to the release of vasodilatory peptides including substance P, neurokinin, and calcitonin gene-related peptide (CGRP)
    • Posterior hypothalamus activation may trigger an attack by activating trigeminal nociceptive pathways through 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; otherwise, autosomal recessive or multifactorial
  • Evidence varies: First-degree relatives are 18 times more likely, and second-degree relatives are 1 to 3 times more likely to be affected by cluster headaches.
  • >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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