What is the management of hemiplegic migraines?

Management of Hemiplegic Migraines

Beta-blockers (such as propranolol), topiramate, or candesartan should be used as first-line preventive treatments for hemiplegic migraines, with acetaminophen and NSAIDs as first-line acute treatments. 1, 2

Understanding Hemiplegic Migraine

Hemiplegic migraine is a rare subtype of migraine with aura characterized by transient hemiparesis (one-sided weakness) during attacks. It can be classified as:

• Familial hemiplegic migraine (FHM): When at least one first-degree relative also has hemiplegic migraine
• Sporadic hemiplegic migraine (SHM): When there is no family history

The diagnosis is based on the International Classification of Headache Disorders criteria, with key features including fully reversible motor weakness and at least one other aura symptom that develops gradually and lasts 5-60 minutes 1, 3.

Acute Treatment

First-line options:

• Acetaminophen and NSAIDs (such as ibuprofen 400-600mg or naproxen sodium 500-550mg) 2, 3
  • Start early in the attack while pain is still mild
  • Often the safest first choice for hemiplegic migraine

Second-line options:

• Antiemetics if nausea/vomiting is present 2

Controversial options:

• Triptans: While traditionally contraindicated in hemiplegic migraine due to theoretical vascular concerns, recent evidence suggests they may be safe and effective for most hemiplegic migraine patients 4
  • Consider only when headaches are not relieved with common analgesics
  • Use with caution and after discussing risks with patients
  • Monitor closely for any prolonged neurological symptoms

Third-line options (if other treatments fail):

• Ditans (lasmiditan) or Gepants (ubrogepant, rimegepant) 2
  • These newer agents don't have vascular contraindications like triptans
  • May be particularly useful in hemiplegic migraine where vascular concerns exist

Preventive Treatment

Consider preventive treatment when:

• Attack frequency exceeds 2 attacks per month
• Attacks are severe and disabling
• Acute treatments are ineffective or contraindicated
• Medication overuse is a concern (using acute medications more than twice weekly) 1, 2

First-line preventive options:

• Beta-blockers (propranolol, metoprolol, atenolol, bisoprolol) 1, 2, 5
• Topiramate 1, 2
• Candesartan 1, 2

Second-line preventive options:

• Flunarizine 1, 3
• Amitriptyline 1, 2
• Sodium valproate (contraindicated in women of childbearing potential) 1, 2, 3

Third-line preventive options:

• CGRP monoclonal antibodies (erenumab, fremanezumab, galcanezumab, eptinezumab) 1, 2
• Lamotrigine (may be particularly effective for migraine with aura) 3
• Verapamil 3
• Acetazolamide 3

Non-Pharmacological Approaches

These can be used as adjuncts to medication or as standalone treatments when medications are contraindicated:

• Neuromodulatory devices 1
• Biobehavioural therapy including relaxation training and cognitive-behavioral therapy 1, 2
• Acupuncture 1, 2
• Regular aerobic exercise and strength training 2
• Regular sleep schedule and adequate hydration 2
• Identification and avoidance of triggers 2

Treatment Evaluation and Follow-up

• Use headache calendars to document frequency, intensity, and disability
• Evaluate treatment response after 2-3 months of therapy 2
• If treatment is successful, continue for 6-12 months before attempting to taper to find minimum effective dose 6
• If treatment fails, check for medication overuse and compliance before switching to another option 1

Special Considerations for Hemiplegic Migraine

• Avoid conventional cerebral angiography as it may trigger an attack 3
• Additional diagnostics (imaging, CSF analysis, EEG) may be needed to exclude other causes of focal neurological symptoms with headache 3
• Genetic testing can confirm but not rule out the diagnosis, as not all genetic causes are known 3
• Monitor closely for prolonged neurological symptoms, especially when trying new treatments

Medication Limitations to Prevent Overuse

• Limit triptans to no more than 9 days per month
• Limit NSAIDs to no more than 15 days per month
• Limit OTC medications to no more than 14 days per month 2

By following this approach, most patients with hemiplegic migraine can achieve significant improvement in their condition and quality of life.