• Clinical science

Cluster headache

Abstract

Cluster headache (CH) is a type of primary headache that mostly affects adult men. Patients present with recurrent, fifteen minute up to three hour attacks of agonizing, strictly unilateral headaches in the periorbital and forehead region (areas innervated by the trigeminal nerve). These attacks are associated with ipsilateral symptoms of increased cranial autonomic activity, e.g., lacrimation, conjunctival injection, rhinorrhea, or partial Horner syndrome. Cluster headaches tend to occur in episodic patterns (“cluster bouts”) followed by months of remission, but are considered chronic if remission between bouts lasts less than one month. Diagnosis is based on the patient's history, in particular on the exact description and timing of the headaches. Acute episodes are treated with 100% oxygen or triptans, while verapamil is used for preventative treatment.

Epidemiology

  • Sex: > (3:1)
  • Peak incidence: 20–40 years

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Not entirely understood
  • Risk factor: tobacco use
  • Possible triggers: alcohol, histamine, seasonal fluctuations

References:[1][2][3]

Clinical features

  • Headache characteristics
    • Agonizing pain
    • Strictly unilateral, periorbital, and/or temporal
    • Short, recurring attacks; that usually occur in a cyclical pattern (“clusters”)
      • May become chronic (less common), with interruptions of less than one month between cluster bouts
      • Attacks often wake patients up during sleep.
  • Ipsilateral autonomic symptoms
  • Restlessness and agitation

While patients with migraine headaches tend to rest motionlessly in a quiet, dark room, individuals with cluster headache pace around restlessly in excruciating pain!References:[1][2][4][5][6][7]

Diagnostics

  • Based on patient history and physical examination
  • Rule out any suspected underlying disease
    • Neuroimaging
    • Doppler ultrasound

References:[2]

Differential diagnoses

  • See learning card on “Headache for more information regarding differential diagnoses.
  • Paroxysmal hemicrania
    • Epidemiology
      • Rare; the exact prevalence is unknown
      • Reversed gender distribution: >
    • Clinical features
      • Symptoms are very similar to those seen in cluster headache; severe unilateral periorbital headaches that occur in attacks are associated with symptoms of autonomic dysfunction
      • Attacks are generally more frequent (usually ≥ 5 per day), but shorter (2–45 min) and may occur at any time of the day.
    • Therapy: no established treatment for acute attacks
    • Prevention: absolute response to indomethacin

References:[1][8][9]

The differential diagnoses listed here are not exhaustive.

Treatment

Medical therapy

  • Acute
    • Oxygen therapy with FiO2 100%
    • First-line: triptans (e.g., sumatriptan) or zolmitriptan
    • Pain relievers (i.e. NSAIDs) are generally not recommended because their onset of action is too slow.
  • Prevention

Interventional therapy

Interventional procedures (e.g., ablative injections, deep brain stimulation) may be considered in patients with cluster headache who do not respond to medical therapy.

  • Bilateral stimulation of the greater occipital nerve
  • Deep brain stimulation of the posterior inferior hypothalamus
  • Case studies have found positive effects in patients treated with:
    • Glycerol/local anesthetic injection into the trigeminal cistern or the trigeminal ganglion
    • Vascular decompression

References:[2][10][11]