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, and an absolute response to indomethacin is required for diagnosis. 1, 2, 3

First-Line Treatment: Indomethacin

• Start indomethacin at 25 mg three times daily and titrate upward until complete headache resolution is achieved, typically requiring 75-225 mg daily in divided doses. 1, 2

• The response to indomethacin is considered a diagnostic criterion—patients must demonstrate an absolute response to therapeutic doses for the diagnosis to be confirmed. 3

• Most patients cannot be fully tapered off indomethacin without headache recurrence, necessitating long-term therapy. 4

Alternative Pharmacologic Options

For Acute Treatment

• Piroxicam and celecoxib have shown good results for acute management when indomethacin cannot be used. 1

For Chronic Preventive Treatment (When Indomethacin is Contraindicated or Not Tolerated)

• Celecoxib, topiramate, and gabapentin are the best alternative options for prolonged treatment. 1

• Melatonin (3-30 mg daily) achieves complete pain freedom in less than 20% of patients, but when added to indomethacin, approximately 45% of patients can reduce their indomethacin dose by 50-75%. 4

• Lamotrigine may be considered based on its efficacy in related trigeminal autonomic cephalalgias, though evidence specific to hemicrania continua is limited. 1

Non-Pharmacologic Interventions

Occipital Nerve Stimulation

• For patients who cannot tolerate indomethacin or have contraindications, occipital nerve stimulation (ONS) is a safe and effective treatment option. 5

• At median 13.5-month follow-up, 5 of 6 patients reported sufficient benefit (80-95% improvement in 4 patients) to recommend the device. 5

• The benefit of ONS is delayed by days to weeks after initiation, and headaches similarly do not recur immediately when the device is switched off. 5

• The bion device (a miniaturized second-generation ONS device) is implanted unilaterally ipsilateral to the headache and provides continuous stimulation. 5

Treatment Algorithm

1. Initiate indomethacin 25 mg TID, titrate every 3-5 days until complete headache resolution (maximum 300 mg/day). 1, 2

2. If indomethacin provides complete relief but causes intolerable side effects, add melatonin 3-30 mg nightly and attempt to reduce indomethacin dose by 50-75%. 4

3. If indomethacin is contraindicated or not tolerated, trial celecoxib, topiramate, or gabapentin as monotherapy. 1

4. If pharmacologic options fail or are contraindicated, refer for occipital nerve stimulation evaluation. 5

Critical Pitfalls to Avoid

• Do not diagnose hemicrania continua without confirming absolute response to therapeutic doses of indomethacin—this is a diagnostic requirement, not just a treatment preference. 3

• Do not assume other NSAIDs will work if indomethacin fails—the response is specific to indomethacin (though piroxicam and celecoxib show some efficacy). 1

• Do not expect immediate benefit from occipital nerve stimulation—onset of benefit is delayed by days to weeks, which differs from the rapid response seen with indomethacin. 5

• Do not rely solely on melatonin as monotherapy—only 18% achieve pain freedom, making it inadequate as a standalone treatment for most patients. 4