What is the best treatment for hemicrania continua?

Treatment of Hemicrania Continua

Indomethacin is the definitive first-line treatment for hemicrania continua, with complete response to this medication considered diagnostic for the condition. Doses typically range from 25-300 mg daily, with most patients responding to 100-150 mg per day 1, 2.

Diagnosis of Hemicrania Continua

Hemicrania continua is characterized by:

• Continuous, unilateral headache (though bilateral cases have been reported) 3
• Moderate baseline pain with superimposed exacerbations of more severe pain
• Autonomic features during exacerbations (ptosis, miosis, conjunctival injection, lacrimation, rhinorrhea)
• Complete response to indomethacin (considered pathognomonic)

Differential Diagnosis

Hemicrania continua must be differentiated from:

• Chronic migraine
• Chronic tension-type headache
• Sinus headache (which often lacks evidence of infection despite sinus region pain) 4
• Other trigeminal autonomic cephalalgias

Treatment Algorithm

First-Line Treatment

• Indomethacin: Start at 25 mg three times daily, titrate up to 50 mg three times daily as needed
  • Complete response typically occurs within 3 days of treatment 2
  • Long-term follow-up shows that 42% of patients can eventually decrease their dose by up to 60% while maintaining pain control 2
  • Gastrointestinal side effects can be managed with concurrent ranitidine or proton pump inhibitors

Alternative Treatments for Indomethacin-Intolerant Patients

If indomethacin is contraindicated or poorly tolerated, consider:

1. COX-2 Inhibitors:

   • Celecoxib has shown efficacy with better GI tolerability 5
   • Piroxicam is another option with demonstrated efficacy 5

2. Anticonvulsants:

   • Gabapentin (20% response rate) 1, 5
   • Topiramate (10% response rate) 1, 5

3. Tricyclic Antidepressants:

   • Amitriptyline (66.6% response rate in some case series) 1

4. Other options with limited evidence:

   • Melatonin
   • Beta-cyclodextrin

Monitoring and Follow-up

• Regular monitoring for indomethacin side effects, particularly gastrointestinal complications
• Periodic attempts to reduce dose to the minimum effective level
• Assessment for medication overuse headache if additional analgesics are being used

Special Considerations

• Posttraumatic hemicrania continua has been reported, with onset temporally linked to head trauma 6
• Some cases may present bilaterally, though this is rare 3
• Misdiagnosis is common, with patients often experiencing symptoms for years (average 12 years in one case series) before correct diagnosis 1

Pitfalls to Avoid

• Failure to consider hemicrania continua in the differential diagnosis of continuous headache
• Inadequate indomethacin trial (insufficient dose or duration)
• Not recognizing that autonomic features may be subtle or absent in some cases
• Overlooking the possibility of medication overuse headache in patients using multiple analgesics

Proper diagnosis and treatment of hemicrania continua can dramatically improve quality of life for affected patients, as complete pain relief is achievable in the majority of cases with appropriate therapy.