What is the treatment for paroxysmal hemicrania?

Treatment for Paroxysmal Hemicrania

Indomethacin is the definitive first-line treatment for paroxysmal hemicrania, with complete response to this medication being so characteristic that it serves as a diagnostic criterion for the condition. 1, 2

First-Line Treatment

• Indomethacin should be initiated at the lowest effective dose (typically starting at 25 mg three times daily) and titrated up as needed to achieve complete pain relief 3
• Most patients achieve complete relief with doses ranging from 75-150 mg/day, with an average effective dose of 84 mg/day 3
• Long-term treatment shows good safety and tolerability profile, with approximately 42% of patients experiencing a decrease of up to 60% in the required dose over time 3

Side Effects and Management

• Gastrointestinal side effects are most common with indomethacin (occurring in approximately 23% of patients) and can often be managed with concurrent ranitidine or other gastroprotective agents 3
• For patients with significant gastrointestinal concerns, consider:
  • Adding a proton pump inhibitor for gastroprotection 2
  • Using the lowest effective dose to minimize side effects 3

Second-Line Treatment Options

For patients who cannot tolerate indomethacin or have contraindications to its use, consider:

• Verapamil - shown to be one of the most effective second-line options 4, 5
• NSAIDs:
  • Acetylsalicylic acid (aspirin) - demonstrated partial efficacy 4
  • Celecoxib - may be better tolerated than indomethacin in some patients 5
  • Piroxicam - shown to be effective in some cases 5

Ineffective Treatments

• Carbamazepine has not shown significant efficacy in paroxysmal hemicrania 4
• Oxygen therapy, which is effective for cluster headache, does not show significant benefit for paroxysmal hemicrania 4

Emerging Treatments

• Noninvasive vagus nerve stimulation has recently shown promise as both an indomethacin-sparing strategy and, in some cases, as a primary treatment option 2

Diagnostic Considerations

• Complete response to indomethacin is considered pathognomonic for paroxysmal hemicrania 1
• An indomethacin trial (either oral or intramuscular 100-200 mg) is recommended for any patient with lateralized discrete attacks of head pain with associated cranial autonomic symptoms 1
• Brain MRI with pituitary views is recommended when paroxysmal hemicrania is diagnosed to rule out secondary causes 2

Clinical Pearls and Pitfalls

• Paroxysmal hemicrania attacks typically last around 17 minutes on average (much shorter than cluster headache) 1
• The pain is typically orbital and temporal (77% of patients), but can occur in various locations including retro-orbital, frontal, and occipital regions 1
• Associated autonomic features include lacrimation (87%), conjunctival injection (68%), rhinorrhea (58%), and nasal congestion (54%) 1
• Patients may be agitated or restless during attacks (80%) and some may become aggressive (26%) 1