Treatment of Hemiplegic Migraine
Critical Contraindication
Triptans and ergot-containing medications are absolutely contraindicated in hemiplegic migraine and should never be used. 1
Acute Treatment Approach
First-Line Acute Therapy
- NSAIDs are the primary acute treatment option, including acetaminophen (though less effective alone), ibuprofen, naproxen, or aspirin. 2, 3, 4
- Combination analgesics containing acetaminophen, aspirin, and caffeine may be effective for mild to moderate attacks. 2, 5
- Treatment should be initiated as early as possible during an attack for maximum efficacy. 2, 6
Adjunctive Acute Management
- For nausea and vomiting, use metoclopramide or prochlorperazine, which also provide synergistic analgesia and improve gastric motility. 2, 5
- Bed rest alone may be sufficient in some cases, particularly in younger patients. 2
Controversial Acute Options
- Triptans remain highly controversial in hemiplegic migraine. While FDA labeling explicitly contraindicates their use 1, some expert opinion suggests they can be prescribed when common analgesics fail and headaches are severe. 3
- This represents a significant divergence in the evidence, with regulatory guidance being more restrictive than some clinical practice patterns. 3
- If triptans are considered despite contraindication warnings, this decision carries substantial medicolegal risk and requires thorough informed consent.
Medications to Avoid
- Opioids and barbiturates should be avoided due to risk of medication overuse headache, dependency, and limited efficacy. 2, 5, 6
- Ergot alkaloids are contraindicated. 6, 1
Prophylactic Treatment
When to Initiate Prevention
- Consider prophylaxis when attack frequency exceeds 2 attacks per month or when severe attacks pose significant burden. 3
- Early prophylactic treatment may prevent permanent brain damage, as severe hemiplegic migraine attacks can cause cytotoxic edema and progressive brain atrophy. 7, 8
First-Line Prophylactic Options (No Strict Order)
- Flunarizine (5-10 mg oral once daily) - contraindicated in Parkinsonism and depression. 2, 3, 7
- Sodium valproate (600-1,500 mg oral daily) - absolutely contraindicated in women of childbearing potential. 2, 3, 7
- Lamotrigine - can be tried as prophylaxis. 3
- Verapamil - calcium channel blocker option. 3
- Acetazolamide - particularly useful in some cases. 3
Second-Line Prophylactic Options
- Topiramate (50-100 mg oral daily) - contraindicated in pregnancy, lactation, nephrolithiasis, and glaucoma. 2, 3
- Amitriptyline (10-100 mg oral at night) - contraindicated in age <6 years, heart failure, glaucoma, and with MAO inhibitors. 2, 3
- Candesartan and pizotifen have less evidence but can be considered. 3
Controversial Prophylactic Options
- Propranolol use is controversial in hemiplegic migraine, with insufficient evidence of adverse effects to definitively contraindicate beta-blockers, but caution is warranted. 3
Novel Prophylactic Therapies
- CGRP monoclonal antibodies (e.g., galcanezumab, erenumab, fremanezumab) show promise for patients who have failed other preventive medications. 2, 5, 9
- Case series data suggest galcanezumab reduced monthly headache days and days with motor weakness in hemiplegic migraine patients, with good tolerability. 9
- These agents may work by blocking peripheral CGRP pathways or secondarily inhibiting cortical spreading depression. 9
Special Diagnostic Considerations
Screening for Patent Foramen Ovale (PFO)
- Screen for PFO in patients with prolonged or intractable hemiplegic migraine, as PFO closure may significantly reduce attack frequency and prevent disabling sequelae. 8
Monitoring for Complications
- Severe attacks can cause permanent neurological deficits, including visual impairment, cortical necrosis, and progressive cerebellar atrophy. 7, 8, 4
- Persistent hyperperfusion during prolonged attacks may contribute to irreversible damage. 8
- Brain atrophy may halt after cessation of severe attacks with effective prophylaxis. 7
Critical Pitfalls to Avoid
- Never use conventional cerebral angiography in hemiplegic migraine, as this may provoke an attack. 3
- Monitor for medication overuse headache (≥15 days/month with NSAIDs or ≥10 days/month with triptans). 5, 6
- Distinguish hemiplegic migraine aura from TIA: aura symptoms spread gradually over ≥5 minutes and occur in succession, whereas TIA symptoms have sudden, simultaneous onset. 2
- Consider hospitalization for long-lasting auras to manage complications and provide supportive care. 4