What is the management and treatment of hemiplegia migraine?

Management and Treatment of Hemiplegic Migraine

Critical Diagnostic Distinction

Hemiplegic migraine requires a fundamentally different treatment approach than typical migraine, with triptans being controversial and potentially contraindicated, while acetaminophen, NSAIDs, and specific prophylactic agents form the cornerstone of management. 1

The diagnosis must be confirmed clinically based on ICHD-3 criteria: recurrent attacks with motor weakness (hemiparesis) lasting <72 hours, accompanied by visual, sensory, or speech aura symptoms that spread gradually over ≥5 minutes. 2, 3

Acute Treatment Algorithm

First-Line Acute Therapy

• Start with acetaminophen or NSAIDs (ibuprofen, naproxen, diclofenac) as the primary acute treatment options. 1
• These should be administered early in the attack when symptoms first develop. 1

Controversial Second-Line: Triptans

• Triptans remain controversial in hemiplegic migraine and should only be prescribed when headaches are not relieved sufficiently with common analgesics. 1
• The controversy stems from theoretical concerns about vasoconstriction in the setting of motor aura, though evidence of actual harm is insufficient to absolutely contraindicate them. 1
• If used, prescribe only after acetaminophen/NSAIDs have failed across multiple attacks. 1

Critical Contraindication

• Never perform conventional cerebral angiography in hemiplegic migraine patients, as this procedure may provoke a severe attack. 1

Treatment of Prolonged Aura

Currently, no effective acute treatment exists for the severe and often prolonged aura symptoms (motor weakness, sensory changes) that characterize hemiplegic migraine. 1

• Hospitalization may be necessary for long-lasting auras to monitor for complications and provide supportive care. 3
• Focus on controlling triggering factors during the aura phase. 3

Prophylactic Treatment Indications

Initiate prophylactic therapy when attack frequency exceeds 2 attacks per month, or when severe attacks pose significant burden requiring reduction of severity and frequency. 1

Prophylactic Medication Options (No Strict Hierarchy)

First-tier prophylactic agents with stronger evidence:

• Flunarizine (calcium channel blocker) 1, 4
• Sodium valproate 1, 4
• Lamotrigine 1, 4
• Verapamil (calcium channel blocker) 1, 4, 5
• Acetazolamide 1, 4, 5

Second-tier options with less evidence:

• Topiramate 1, 4
• Candesartan 1
• Pizotifen 1

Controversial agent:

• Propranolol and other beta-blockers are controversial in hemiplegic migraine, but evidence of adverse effects is insufficient to absolutely contraindicate them. 1
• Use with caution and only after other options have been exhausted. 1

Genetic Considerations Affecting Management

• Three genes (CACNA1A, ATP1A2, SCN1A) account for familial hemiplegic migraine, but genetic testing can confirm but not exclude the diagnosis. 1, 2
• Patients with identified mutations may present with more complex phenotypes including cerebellar ataxia, epilepsy, or intellectual disability, requiring additional management considerations. 4, 3
• The presence of extensive weakness, brainstem manifestations, or neurological comorbidities increases likelihood of finding a monogenic cause. 2, 4

Diagnostic Workup to Exclude Mimics

Perform neuroimaging (MRI preferred), CSF analysis, and EEG primarily to exclude other causes of focal neurological symptoms with headache, such as:

• Stroke or TIA (differentiated by sudden simultaneous onset vs. gradual spread over ≥5 minutes) 6, 3
• Epilepsy 2, 3
• Encephalitis 2
• Secondary headache syndromes 2

Common Pitfalls to Avoid

• Do not assume standard migraine treatment algorithms apply—hemiplegic migraine requires specialized consideration, particularly regarding triptan use. 1
• Do not overlook the need for prophylaxis—given the severity and potential for prolonged neurological deficits, prophylactic therapy should be strongly considered even at lower attack frequencies than typical migraine. 1
• Do not perform invasive vascular procedures without careful risk-benefit analysis, as conventional angiography is contraindicated. 1

Evidence Limitations

All treatment recommendations for hemiplegic migraine are based on empirical data, small unblinded studies, and expert opinion rather than randomized controlled trials, due to the rarity of this condition. 1, 2, 3 Treatment therefore involves a trial-and-error strategy guided by clinical response. 1