What is the first line of treatment for hemiplegic migraines?

First-Line Treatment for Hemiplegic Migraines

NSAIDs (such as acetylsalicylic acid, ibuprofen, or diclofenac potassium) are the first-line treatment for hemiplegic migraines. 1

Understanding Hemiplegic Migraine

Hemiplegic migraine is a rare subtype of migraine with aura characterized by temporary motor weakness (hemiparesis) during attacks, which may be accompanied by visual, sensory, or speech disturbances. It can occur in two forms:

• Familial hemiplegic migraine (FHM): With autosomal dominant inheritance
• Sporadic hemiplegic migraine (SHM): Without family history

The motor symptoms typically last less than 72 hours and are associated with other aura symptoms 2.

Treatment Algorithm for Hemiplegic Migraines

Acute Treatment

1. First-line: NSAIDs

   • Options include acetylsalicylic acid, ibuprofen, naproxen sodium, or diclofenac potassium 1
   • Acetaminophen alone is ineffective 1
   • The acetaminophen-aspirin-caffeine combination can also be effective 1

2. For nausea/vomiting

   • Use prokinetic antiemetics (domperidone or metoclopramide) as adjuncts 1
   • Consider non-oral routes of administration when nausea/vomiting are significant 1

3. Second-line options (controversial)

   • While triptans are traditionally contraindicated in hemiplegic migraine according to some guidelines 1, more recent evidence suggests they may be safe and effective for most hemiplegic migraine patients 3
   • Dihydroergotamine (DHE) intranasal may be considered in some cases 1

4. Rescue medications

   • When other treatments fail, opioids may be considered if risk of abuse has been addressed 1

Important Cautions

• Avoid oral ergot alkaloids, which are poorly effective and potentially toxic 1
• Avoid opioids and barbiturates as regular treatment due to questionable efficacy and risk of dependency 1
• Limit acute treatments to no more than twice weekly to prevent medication overuse headache 1

Preventive Treatment

Consider preventive treatment when:

• Two or more migraine attacks per month with disability for ≥3 days per month
• Use of rescue medication more than twice weekly
• Failure of or contraindications to acute treatments
• Presence of hemiplegic migraine 1

Preventive Options for Hemiplegic Migraine

First-line preventive agents:

• Sodium valproate/divalproex sodium (500-1,500 mg/day)
• Amitriptyline (30-150 mg/day)
• Flunarizine (where available)
• Lamotrigine
• Verapamil
• Acetazolamide 4

Second-line options:

• Topiramate
• Candesartan
• Pizotifen 4

Note on beta-blockers: While propranolol and timolol are first-line for typical migraine, their use in hemiplegic migraine is more controversial, though evidence of adverse effects is insufficient to absolutely contraindicate them 4.

Monitoring and Follow-up

• Evaluate treatment efficacy after 2-3 months for oral preventives 1
• Preventive medications must be given at therapeutic doses for at least 6-8 weeks before judging ineffectiveness 5
• Consider pausing preventive treatment after 6-12 months of successful control 1

Special Considerations

• Patients with hemiplegic migraine may require hospitalization for long-lasting auras 2
• Genetic testing can confirm but not rule out the diagnosis, as some genes remain unidentified 4
• Conventional cerebral angiography is contraindicated in hemiplegic migraine as it may provoke an attack 4

By following this treatment approach, the goal is to reduce the frequency, duration, and severity of hemiplegic migraine attacks while minimizing adverse effects and improving quality of life.