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Symptoms
- Typically 1-3 attacks within 24 hours, averaging 30-40 minutes each
- Extremely intense unilateral stabbing, burning facial and eye pain with simultaneous ipsilateral autonomic symptoms (Horner syndrome, tearing, and a running nose)
- Often occurring at night for a few hours (not in attacks)
Triggers
- Lack of sleep, alcohol, nitrates, light stimuli
Work-up
- Corneal sensitivity? (Innervation area of the ophthalmic nerve V1)
- Rule out angle closure
- Referral to a neurologist for further investigations and consider imaging (CT/MRI head)
Acute Therapy
- First Choice
- 100% oxygen 7-15L for 15-20 minutes
- Sumatriptan (6 mg s.c.) or Zolmitriptan nasal spray (5–10 mg)
- Second Choice
- Sumatriptan (20mg nasal)
- Intranasal application of Lidocaine 4–10%
Prophylaxis
- First Choice
- Verapamil (start at 3x 80mg per day, increase by 80mg weekly up to 480mg/day, rarely up to 960mg per day)
- Caution: Perform an EKG due to bradycardia and AV block
- Gradual tapering of prednisolone 100mg/day over approximately 2 weeks
- Verapamil (start at 3x 80mg per day, increase by 80mg weekly up to 480mg/day, rarely up to 960mg per day)
- Second Choice
- Lithium (plasma level: 0.6-1.2 mmol/l)
- Topiramate (start with 50mg/day, increase by 50mg weekly up to 100-200 mg/day)
- Others: Topical application of 0.5% Capsaicin ointment in the ipsilateral nostril
Sources
- AWMF Leitlinie Cluster Kopfschmerz und trigeminoautonome Kopfschmerzen 05/2015
- Lungenliga – Muster für Verordnung von kurzfristiger Sauerstofftherapie
- Schweizerische Kopfwehgesellschaft – Therapiempfehlungen
- The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
- Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)