What is the treatment for hemicrania continua?

Treatment of Hemicrania Continua

Indomethacin is the definitive treatment for hemicrania continua, with doses ranging from 25 to 300 mg per day providing absolute and complete resolution of symptoms—this response is so characteristic that it serves as a diagnostic criterion for the condition. 1, 2, 3, 4

First-Line Treatment: Indomethacin

• Indomethacin remains the gold standard and only proven effective treatment for hemicrania continua, demonstrating rapid near-complete or complete resolution of both headache and autonomic symptoms. 1, 2
• The typical dosing range is 25 to 300 mg per day, with treatment initiated as early as possible once the diagnosis is suspected. 2, 3
• An absolute response to therapeutic doses of indomethacin is required for diagnosis—this means complete or near-complete resolution, not just improvement. 4
• Patients should be instructed to report changes in headache and autonomic symptoms within several days of initiating treatment. 2

Alternative Options for Indomethacin-Intolerant Patients

For patients who cannot tolerate long-term indomethacin therapy due to gastrointestinal or other side effects, the following alternatives have demonstrated efficacy:

Acute Treatment Alternatives

• Piroxicam has shown good results for acute treatment and represents the best alternative NSAID to indomethacin. 1
• Celecoxib has demonstrated effectiveness for acute symptom control. 1

Prolonged/Preventive Treatment Alternatives

• Celecoxib, topiramate, and gabapentin are effective options for prolonged treatment when indomethacin cannot be used. 1
• Valproic acid has been reported effective in at least one case of hemicrania continua evolving from cluster headache where indomethacin was not tolerated. 5

Critical Clinical Considerations

• Recognizing the neuro-ophthalmologic symptoms of hemicrania continua (ptosis, miosis, conjunctival injection, lacrimation, rhinorrhea) is critical for prompt initiation of treatment, as these autonomic features may be the presenting complaint. 2
• The most prevalent accompanying symptoms are lacrimation, conjunctival injection, and restlessness or agitation. 4
• Unlike cluster headache, the autonomic features in hemicrania continua tend to be less pronounced. 3
• Hemicrania continua can arise de novo or be triggered by head trauma, so post-traumatic cases should not be dismissed. 3

Treatment Initiation Strategy

1. Start indomethacin at 25 mg and titrate upward based on response, up to 300 mg per day if needed. 1, 3
2. Assess response within days—the response should be absolute (complete or near-complete resolution), not partial. 2, 4
3. If gastrointestinal intolerance develops, consider switching to piroxicam first, then celecoxib, topiramate, or gabapentin for long-term management. 1
4. Do not diagnose hemicrania continua if there is only partial response to indomethacin—absolute response is required. 4

Common Pitfalls to Avoid

• Failing to recognize that hemicrania continua requires an absolute response to indomethacin, not just improvement—partial responders likely have a different diagnosis. 4
• Missing the diagnosis when patients present primarily with neuro-ophthalmologic symptoms rather than emphasizing the headache component. 2
• Discontinuing indomethacin prematurely before achieving adequate therapeutic dosing (up to 300 mg/day may be required). 1, 3
• Not considering hemicrania continua in post-traumatic headache cases, as trauma can trigger the condition. 3