What is the treatment for continuous hemicrania?

Treatment of Hemicrania Continua

Indomethacin is the definitive first-line treatment for hemicrania continua, with an initial dose of 75-150 mg daily divided into 2-3 doses, as this medication provides complete and sustained symptom relief and serves as a diagnostic criterion for the condition. 1, 2, 3

Diagnostic Confirmation Through Treatment Response

• Absolute response to indomethacin is required for diagnosis of hemicrania continua, distinguishing it from other chronic unilateral headache disorders 1, 4, 2
• Relief of symptoms typically occurs within 3 days of initiating indomethacin treatment 3
• The mean effective dose in clinical series is approximately 137 mg daily (range 25-225 mg), though most patients respond to 75-150 mg daily 2, 3

Indomethacin Dosing Strategy

• Start with 25 mg three times daily and titrate upward every 3 days until complete headache resolution is achieved 3
• Once pain-free, maintain the effective dose for several weeks before attempting dose reduction 3
• Approximately 42% of patients can reduce their maintenance dose by up to 60% over time while remaining symptom-free 3
• Administer with food and consider prophylactic gastroprotection with ranitidine or proton pump inhibitors 3

Management of Indomethacin Intolerance

When indomethacin is not tolerated (occurs in approximately 17-50% of patients), alternative approaches include: 4

• Nerve blocks as primary alternative therapy: Greater occipital nerve blocks, supraorbital nerve blocks, or trochlear corticosteroid injections provide complete response in 54% and partial response in 39% of patients who cannot tolerate indomethacin 4
• Alternative NSAIDs such as piroxicam-beta-cyclodextrin may provide relief in select cases, though evidence is limited to case reports 5
• These alternatives should be considered early rather than abandoning treatment, as hemicrania continua is highly treatable when properly managed 4

Safety Profile and Monitoring

• Long-term indomethacin treatment (average 3.8 years) demonstrates good safety and tolerability 3
• Adverse events occur in approximately 23% of patients, predominantly gastrointestinal symptoms that respond to ranitidine 3
• No major side-effects have been observed with prolonged use at therapeutic doses 3
• Monitor for gastric discomfort and provide gastroprotection as needed 5, 3

Clinical Pitfalls to Avoid

• Do not delay indomethacin trial: All cases of chronic unilateral daily headaches should receive an indomethacin trial early in treatment, as hemicrania continua is not rare (2% of headache clinic patients) and is highly treatable 2, 4
• Do not confuse baseline continuous headache (mild-moderate intensity) with exacerbations (severe intensity with autonomic symptoms), as 70% of patients meet migraine criteria during exacerbations 2
• Do not abandon treatment if initial indomethacin dose fails—titrate adequately before considering the diagnosis incorrect 3
• Consider posttraumatic hemicrania continua in patients with temporal relationship between head trauma and headache onset 6