What is the treatment for hemicrania continua?

Treatment of Hemicrania Continua

Indomethacin is the definitive treatment for hemicrania continua, with therapeutic doses of 100-150 mg daily providing complete or near-complete resolution of symptoms in all cases. 1, 2, 3

Diagnostic Confirmation Through Treatment Response

• Absolute response to indomethacin is required for diagnosis of hemicrania continua, making it both a diagnostic criterion and the primary treatment 1, 3
• The response is typically rapid, with near-complete or complete resolution of headache and autonomic symptoms occurring within days of initiating therapy 4
• Secondary headache causes must be ruled out before confirming the diagnosis and starting treatment 1

Standard Indomethacin Regimen

• Start with indomethacin 100-150 mg daily as the standard therapeutic dose 2
• Doses may range from 25-300 mg per day depending on individual response, though most patients respond to the 100-150 mg range 2, 5
• Patients should be instructed to report changes in headache and autonomic symptoms after several days of treatment 4

Critical Problem: Long-Term Indomethacin Risks

The major clinical challenge is that indomethacin carries significant risks with both short-term and long-term use, making it a poor choice for continuous therapy despite its effectiveness. 2

• Most patients cannot discontinue indomethacin without headache recurrence, creating a therapeutic dilemma 6
• The risks of chronic NSAID use (gastrointestinal bleeding, cardiovascular events, renal toxicity) necessitate alternative approaches for long-term management 2

Alternative Medical Therapies

When indomethacin is contraindicated or poorly tolerated, consider these alternatives in order of evidence strength:

• Amitriptyline: Effective in 66.6% of cases as prophylactic treatment 2
• Gabapentin: Effective in 20% of cases 2
• Topiramate: Effective in 10% of cases 2
• Other reported alternatives include COX-2 inhibitors, piroxicam, beta-cyclodextrin, and melatonin, though evidence is limited to case reports 2

Important caveat: These alternatives are substantially less effective than indomethacin and should only be used when indomethacin cannot be tolerated 2

Interventional Procedures for Indomethacin-Dependent Patients

For patients who cannot discontinue indomethacin without recurrence, radiofrequency ablation procedures provide headache relief as complete as indomethacin with long-term efficacy. 6

Procedural Algorithm:

1. Perform diagnostic blockade based on clinical examination and presence of cranial autonomic symptoms 6:

   • Atlanto-axial joint block
   • C2 dorsal root ganglion block
   • Sphenopalatine ganglion block

2. If diagnostic block is positive, proceed to radiofrequency ablation of the corresponding target 6:

   • C2 ventral ramus
   • C2 dorsal root ganglion
   • Sphenopalatine ganglion

3. Long-term follow-up shows patients remain essentially headache-free without indomethacin, though some may need repeat procedures 6

Clinical Presentation Features to Recognize

• Continuous unilateral headache with ipsilateral cranial autonomic symptoms (lacrimation, conjunctival injection, nasal congestion, ptosis) 1, 3
• Restlessness or agitation during exacerbations 3
• Pain is continuous with superimposed exacerbations lasting minutes to days 5
• May present with neuro-ophthalmologic symptoms requiring prompt recognition for early treatment initiation 4

Common Diagnostic Pitfall

Average time to correct diagnosis is 12 years, as patients often receive incorrect diagnoses before being properly evaluated 2. Consider hemicrania continua in any patient with continuous unilateral headache, normal neurological examination, and normal imaging, regardless of age of onset 2.