Cluster Headache

Background

• Cluster headache is an uncommon primary headache disorder characterized by attacks of severe unilateral pain with ipsilateral autonomic symptoms.
• The attacks last 15-180 minutes and they occur in "bouts" or "clusters" of frequencies 0.5-8 headaches/day that typically last 1 week to 1 year.
• The typical age of onset is between ages 10 and 39 years, and it is more common in men.
• Risk factors include a family history of cluster headache and a history of tobacco use.

Evaluation

• The diagnosis of a cluster headache is made by the presence of characteristic clinical features.
• Patients usually present with an excruciating unilateral headache characterized by a sharp, piercing, throbbing, or burning periorbital pain with:
  • rapid onset and progression to excruciating pain over a few minutes
  • attacks usually lasting 15-180 minutes
  • pain usually at maximum intensity for duration of attack and then ending abruptly
• The headaches are often accompanied by ipsilateral cranial autonomic signs and/or restlessness or agitation.
• A cluster headache needs to be distinguished from a migraine headache, trigeminal neuralgia, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic features (SUNA) syndrome.
• Consider magnetic resonance imaging of the brain and carotid arteries to rule out secondary causes of a cluster headache.

Management

• First-line treatments to abort a cluster headache during an acute attack include:
  • sumatriptan 6 mg subcutaneous injection (Strong recommendation)
  • zolmitriptan 5-10 mg nasal spray (Strong recommendation)
  • 100% oxygen at 6-12 L/minute via high-flow mask with patient in sitting position (Strong recommendation)
• Also consider prophylactic therapies depending on the individual patient and the effect of the headache attacks; these include:
  • verapamil 360 mg/day orally in divided doses (Weak recommendation) (generally considered preventative drug of choice); requires electrocardiogram before and after each increase in dosage and on a regular basis
  • melatonin 10 mg orally at bedtime (Weak recommendation)
  • civamide 0.025% nasal spray 100 mcL in each nostril (for patients with episodic cluster headache) (Weak recommendation) (not available in United States)
  • lithium 900 mg orally daily in divided doses (Weak recommendation)
  • suboccipital corticosteroid injection (Strong recommendation)
• Other interventions with some evidence for efficacy include noninvasive vagus nerve stimulation and invasive neuromodulatory treatments.