What are the diagnostic criteria and treatment options for cluster headaches?

Cluster Headache Diagnosis and Treatment

Cluster headache is diagnosed by the presence of severe unilateral pain lasting 15-180 minutes with ipsilateral autonomic symptoms, occurring in clusters with a frequency of 1-8 attacks daily. 1

Diagnostic Criteria for Cluster Headache

According to the International Headache Society classification, cluster headache diagnosis requires:

• Five attacks with a frequency of one to eight attacks on any given day 1
• Severe unilateral, supraorbital, or temporal pain lasting 15 to 180 minutes (untreated) 1, 2
• At least one of the following features on the same side as the pain:
  • Lacrimation 1, 2
  • Nasal congestion or rhinorrhea 1, 2
  • Forehead and/or facial sweating 1
  • Ptosis 1, 2
  • Miosis 1
  • Eyelid edema 1

Clinical Presentation and Associated Features

• Most patients (98.8%) present with cranial autonomic features 3
• Many patients (67.9%) report restlessness or agitation during attacks, unlike migraine where patients prefer to lie still 3, 2
• Some patients (27.8%) may experience nausea and vomiting 3
• Photophobia or phonophobia may be present in 61.2% of patients 3
• About 23% may report a migrainous aura preceding attacks 3
• Attacks frequently occur at night, awakening patients from sleep 4
• Distinct circadian and circannual periodicity is characteristic 4

Types of Cluster Headache

• Episodic cluster headache: Occurs in clusters lasting weeks to months with periods of remission 2, 5
• Chronic cluster headache: Attacks occur for more than 1 year without remissions 2, 5

Common Triggers

• Alcohol (particularly red wine in 70% of patients) 3, 2
• Nitroglycerin and foods containing nitrates 2
• Strong odors 2

Treatment Options

Acute Attack Treatment

• First-line options:

  • 100% oxygen at 12 L/min via non-rebreathable mask 6, 5
  • Subcutaneous sumatriptan 6 mg (shown to provide pain relief in 49-74% of patients within 15 minutes) 7, 6, 5

• Second-line options:

  • Intranasal sumatriptan or zolmitriptan 6

Prophylactic Treatment

• First-line:

  • Verapamil (typically 360-720 mg/day, with ECG monitoring for PR interval prolongation) 6, 5

• Transitional prophylaxis (at the start of a cluster):

  • Corticosteroids 6, 5
  • Suboccipital steroid injections (greater occipital nerve blockade) 6, 5

• Additional prophylactic options:

  • Lithium 6, 5
  • Melatonin 2, 6
  • Topiramate 2, 6, 5
  • Valproic acid 6, 5
  • Gabapentin 6

Newer Treatment Options

• Galcanezumab (monoclonal antibody to calcitonin gene-related peptide) - shown effective for episodic cluster headache 2, 6
• Neurostimulation (occipital nerve stimulation or deep brain stimulation) for drug-resistant chronic cluster headache 2, 5

Clinical Pitfalls and Considerations

• Diagnostic delay: Average of 7 years from symptom onset to diagnosis 5
• Misdiagnosis: Often misdiagnosed as migraine or sinusitis 5
• Safety considerations: Sumatriptan should not be given to patients with coronary artery disease or uncontrolled risk factors for CAD 7
• Medication dosing: Verapamil doses used for cluster headache are often higher than those used in cardiology, requiring ECG monitoring 5
• Treatment adequacy: A significant number of cluster headache patients do not receive adequate treatment despite availability of effective options 3

Distinguishing from Other Headache Types

• Migraine: Usually lasts 4-72 hours (vs. 15-180 minutes for cluster), often has nausea/vomiting and photo/phonophobia, and patients prefer to lie still rather than pace 1, 8
• Tension headache: Typically bilateral with pressing/tightening quality, mild to moderate intensity, and lacks autonomic features 8