What are the guidelines for managing hemiplegic migraines in pediatric patients?

Management of Hemiplegic Migraine in Pediatric Patients

For pediatric hemiplegic migraine management, triptans should be avoided due to potential stroke risk, and first-line preventive options include amitriptyline, sodium valproate, cyproheptadine, or calcium channel antagonists with aspirin if not contraindicated. 1

Diagnosis and Clinical Features

• Hemiplegic migraine (HM) is characterized by transient hemiparesis during attacks, with two types:
  • Familial hemiplegic migraine (FHM)
  • Sporadic hemiplegic migraine (SHM)
• Genetic mutations in CACNA1A, ATP1A2, and SCN1A genes may be present 2
• Mean age of onset is around 10.5 years with attack duration varying from minutes to hours 3
• Associated symptoms often include sensory and basilar-type auras (65% and 63% respectively) 3

Acute Treatment

First-line options:

• Acetaminophen and NSAIDs (particularly ibuprofen) 2, 4
  • Ibuprofen has demonstrated effectiveness in pediatric migraine 4
  • Acetaminophen alone may be less effective but can be considered

Important cautions:

• Triptans are contraindicated in hemiplegic migraine due to potential vasoconstrictive effects and stroke risk 1, 2
• Conventional cerebral angiography is contraindicated as it may trigger an attack 2

Preventive Treatment

Consider preventive therapy when:

• Attack frequency exceeds 2 per month
• Attacks are severe or cause significant disability 2

Recommended preventive options:

1. Amitriptyline (30-150 mg/day) 1, 5
2. Sodium valproate (800-1500 mg/day) 1, 5, 2
3. Cyproheptadine 1
4. Calcium channel antagonists (e.g., verapamil, flunarizine) 1, 2
5. Acetazolamide 2

Second-line options:

• Topiramate 5, 2
• Lamotrigine 2

Controversial options:

• Beta-blockers (e.g., propranolol) - use with caution as they might worsen intracranial vasoconstriction 1, 2

Non-Pharmacological Approaches

• Headache diary to track frequency, severity, and medication effectiveness 1, 5
• Identify and avoid triggers (e.g., stress, fatigue, certain foods) 1
• Regular sleep schedule and stress management 5
• Relaxation training and biofeedback 5
• Cognitive-behavioral therapy 5

Special Considerations

• For female adolescents: avoid combined hormonal contraceptives with estrogens due to increased stroke risk 5
• Genetic testing may be considered but cannot rule out the diagnosis as some genes remain unidentified 2
• Additional diagnostics (imaging, EEG) are mainly to exclude other causes of focal neurological symptoms 2
• Patients with ATP1A2 mutations and prothrombotic conditions may have increased stroke risk 6

Follow-up and Monitoring

• Assess effectiveness of preventive treatment after 6-8 weeks 5
• Monitor for medication side effects, particularly with anticonvulsants
• Consider specialist referral if treatment fails or diagnosis is uncertain 5

Hemiplegic migraine in children often has a favorable clinical evolution despite potentially severe initial presentations 3. The management approach should focus on both effective symptom control and minimizing stroke risk, with careful selection of both acute and preventive medications.