Hemiplegic Migraine Diagnosis and Management

Diagnosis

The International Headache Society recommends diagnosing hemiplegic migraine clinically based on ICHD-3 criteria requiring at least two attacks with fully reversible motor weakness as the aura symptom, lasting 5-72 hours, accompanied by or followed by headache within 60 minutes 1
The diagnosis requires motor aura to spread gradually over ≥5 minutes 1
Headache accompanies or follows aura within 60 minutes 1
The diagnosis should not be better explained by another diagnosis 2

Patients with hemiplegic migraine often experience duration and frequency of attacks 3
Pain characteristics, such as location, quality, severity, and aggravating factors, are important to document 1
Accompanying symptoms, including photophobia, phonophobia, nausea, and vomiting, should be noted 2
Specific aura features, such as gradual spread, succession of symptoms, and unilateral presentation, are crucial for diagnosis 2

The American Academy of Neurology recommends administering NSAIDs early in the headache phase for maximum effectiveness 3
Prokinetic antiemetics, such as domperidone or metoclopramide, can be used for nausea and vomiting 3
Ergot alkaloids are poorly effective and potentially toxic, and should be avoided 3
Opioids and barbiturates have questionable efficacy and risk of dependency, and should be avoided 3

The American Headache Society suggests assessing efficacy of preventive therapy after 2-3 months 3
Consider pausing preventive therapy after 6-12 months of successful treatment 3

Seizures during attacks require urgent attention 3
Altered consciousness during attacks requires urgent attention 1
Medication overuse headache can occur with frequent use of acute medications, and patients should be educated on this risk 4

The American Academy of Neurology recommends performing neuroimaging, such as MRI, to exclude other causes of focal neurological symptoms with headache, such as stroke or TIA, which can be differentiated by sudden simultaneous onset vs. gradual spread over ≥5 minutes 5
The International Headache Society suggests that EEG and CSF analysis should also be performed to rule out conditions like epilepsy and encephalitis, which can present with similar symptoms 5

In patients with hemiplegic migraine who develop a new isolated speech disturbance, urgent MRI of the brain with diffusion‑weighted imaging should be performed to rule out acute ischemic stroke or TIA because the symptom represents a change from the typical gradual aura pattern. 6
Hemiplegic migraine aura typically evolves gradually over at least 5 minutes and progresses sequentially, whereas transient ischemic attack symptoms appear suddenly and simultaneously; this distinction guides the need for immediate imaging. 6
Migraine with aura, particularly hemiplegic migraine, is associated with an increased risk of cerebrovascular events, necessitating exclusion of stroke when new focal neurological signs emerge. 6

Early administration of non‑steroidal anti‑inflammatory drugs (e.g., ibuprofen 400–800 mg or naproxen sodium 275–550 mg) at the onset of the headache phase provides maximal acute relief in hemiplegic migraine attacks. 7
Adding a prokinetic antiemetic such as metoclopramide or domperidone can improve nausea and gastric emptying, enhancing the effectiveness of acute migraine therapy. 7
Opioids and barbiturates should be avoided in hemiplegic migraine because they have limited efficacy, carry a risk of dependence, and may precipitate medication‑overuse headache. 7

Preventive therapy should be considered when a patient experiences two or more hemiplegic migraine attacks per month or when attacks cause substantial functional impairment. 8
First‑line preventive agents for hemiplegic migraine include flunarizine (5–10 mg daily) and sodium valproate (600–1500 mg daily), the latter being contraindicated in individuals of child‑bearing potential. 9
The effectiveness of a preventive regimen should be reassessed after 2–3 months of therapy to determine whether adjustments are needed. 9

Patients should be educated that using acute migraine medications on more than 10 days per month for triptans or more than 15 days per month for NSAIDs can lead to medication‑overuse headache, and they should track medication use with a headache diary. 9