What is the initial treatment approach for a patient presenting with hemiplegic migraine?

Initial Treatment Approach for Hemiplegic Migraine

For acute treatment of hemiplegic migraine, NSAIDs (aspirin, ibuprofen, or naproxen sodium) should be used as first-line therapy, while triptans and ergot-containing medications are absolutely contraindicated due to the risk of prolonged vasospasm in this specific migraine subtype. 1, 2

Acute Treatment Strategy

First-Line: NSAIDs

• Administer NSAIDs early in the headache phase for maximum effectiveness, using aspirin (800-1000 mg), ibuprofen, or naproxen sodium 3, 4
• Acetaminophen alone is ineffective and should not be relied upon as monotherapy 1
• These agents address the headache pain but do not treat the motor aura symptoms 5

Contraindicated Medications

• Triptans are absolutely contraindicated in hemiplegic migraine due to concerns about prolonged vasospasm and potential for stroke-like complications 1, 2
• Ergotamine-containing medications (DHE, ergotamine) are similarly contraindicated 1, 2
• This contraindication applies even if the patient has previously used these medications for other migraine types 2

Adjunctive Symptomatic Treatment

• Use prokinetic antiemetics (domperidone or metoclopramide 10-20 mg) for nausea and vomiting 1, 3
• Consider nonoral routes of administration when nausea or vomiting are prominent early symptoms 1

Rescue Medication Considerations

• Opioids may be considered as rescue medication when NSAIDs fail and the risk of abuse has been addressed, though they have questionable efficacy and dependency risk 1, 3
• Butalbital-containing compounds can serve as home rescue medication but carry risk of medication overuse headache 1
• Limit acute treatment use to no more than twice weekly to prevent medication overuse headache 1

Preventive Therapy Indication

Hemiplegic migraine is an explicit indication for preventive therapy regardless of attack frequency, as it represents an "uncommon migraine condition" with potential for severe neurological complications 1, 6

First-Line Preventive Options

• Flunarizine (5-10 mg daily) is particularly effective for hemiplegic migraine, with efficacy comparable to propranolol and topiramate 6, 5
• Sodium valproate (800-1500 mg/day) or divalproex sodium (500-1500 mg/day) may be particularly effective in patients with prolonged or atypical migraine aura, though strictly contraindicated in women of childbearing potential 1, 6
• Verapamil (calcium channel blocker) can be considered as an alternative 5
• Lamotrigine may reduce aura frequency and severity 5

Alternative Preventive Agents

• Acetazolamide has shown efficacy in case reports for familial hemiplegic migraine, though used off-label 5, 7
• Topiramate can be tried, though evidence is less robust in this specific population 5

Controversial Agents

• Propranolol use in hemiplegic migraine is controversial, with insufficient evidence of adverse effects to definitively contraindicate beta-blockers, but caution is warranted 5
• The theoretical concern relates to potential prolongation of neurological symptoms 5

Implementation Strategy

Preventive Therapy Initiation

• Start with low doses and titrate slowly until clinical benefits are achieved or side effects limit further increases 1
• Allow an adequate trial period of 2-3 months before determining efficacy 1, 6
• After 6-12 months of successful treatment, consider tapering or discontinuing to assess ongoing need 1

Monitoring Requirements

• Track attack frequency, severity, duration, degree of disability, treatment response, and adverse effects using headache diaries 1, 4
• Monitor for seizures or altered consciousness during attacks, which require urgent attention 3
• Educate patients about medication overuse headache risk with frequent use of acute medications (≥10 days/month for NSAIDs) 3, 4

Critical Pitfalls to Avoid

• Never prescribe triptans or ergot derivatives for hemiplegic migraine, even if the patient requests them based on prior use for other migraine types 1, 2
• Do not perform conventional cerebral angiography during evaluation, as this may provoke an attack 5
• Avoid inadequate duration of preventive trial (less than 2-3 months) before declaring treatment failure 1, 6
• Do not overlook the need for neuroimaging (MRI) to exclude stroke, TIA, or other structural causes of focal neurological symptoms 3

Differential Diagnosis Considerations

• Perform neuroimaging (MRI) to exclude stroke or TIA, which present with sudden simultaneous onset versus the gradual spread over ≥5 minutes characteristic of hemiplegic migraine aura 1, 3
• Consider EEG and CSF analysis to rule out epilepsy and encephalitis when diagnosis is uncertain 3