Introduction

Cluster headaches are known to be one of the most painful conditions that patients can have the misfortune to suffer. The name relates to the pattern of the headaches - they typically occur in clusters lasting several weeks, with the clusters themselves typically once a year.

Epidemiology

  • Incidence: 10.00 cases per 100,000 person-years
  • Peak incidence: 50-60 years
  • Sex ratio: more common in males 3:1
Condition Relative
incidence
Migraine500.00
Trigeminal neuralgia1.50
Cluster headache1
Paroxysmal hemicrania0.02
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Aetiology

Risk factors include:
  • Male gender (3:1)
  • Smoking

Alcohol may trigger an attack and there also appears to be a relation to nocturnal sleep.

Clinical features

Features
  • Pain
    • Intense sharp, stabbing pain around one eye (recurrent attacks 'always' affect same side)
    • Typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
  • Clusters typically last 4-12 weeks
  • Patient is restless and agitated during an attack
  • Often accompanied by redness, nasal stuffiness, lacrimation, lid swelling
  • Miosis and ptosis in a minority

Investigations

  • most patients will have neuroimaging - underlying brain lesions are sometimes found even if the clinical symptoms are typical for cluster headache
  • MRI with gadolinium contrast is the investigation of choice

Diagnosis

From the International Headache Society we present the third edition of the International Classification of Headache Disorders:

A. At least five attacks fulfilling criteria B-D

B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes (when untreated)

C. Either or both of the following:
  • 1. at least one of the following symptoms or signs, ipsilateral to the headache:
    • conjunctival injection and/or lacrimation
    • nasal congestion and/or rhinorrhoea
    • eyelid oedema
    • forehead and facial sweating
    • miosis and/or ptosis
  • 2. a sense of restlessness or agitation

D. Occurring with a frequency between one every other day and 8 per day

E. Not better accounted for by another ICHD-3 diagnosis.

Management

Management
  • Acute:
    • 100% oxygen (80% response rate within 15 minutes), subcutaneous triptan (75% response rate within 15 minutes)
  • Prophylaxis:
    • Verapamil is the drug of choice
    • There is also some evidence to support a tapering dose of prednisolone
  • NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches with respect to neuroimaging

Some neurologists use the term trigeminal autonomic cephalgia to group a number of conditions including cluster headache, paroxysmal hemicrania and short-lived unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). It is recommended such patients are referred for specialist assessment as specific treatment may be required, for example it is known paroxysmal hemicrania responds very well to indomethacin.