Hemiplegic migraine
Alerts and Notices
Synopsis
To diagnose HM, patients must have episodic, unilateral, reversible motor weakness as a migraine aura, as well as at least one other type of aura. Between attacks, patients typically have a normal neurologic examination.
HM is a rare condition occurring with a prevalence of 0.01%. HM is more prevalent in women than men (3:1), with an age of onset in the teenage years. HM has been associated with mutations in CACNA1A, ATP1A2, and SCN1A genes, which can be sporadic or familial. Inheritance in familial HM is autosomal dominant, although penetrance is incomplete. Patients with HM, cerebellar findings, and developmental delay should be tested for CACNA1A gene mutations. Patients with HM and associated epilepsy should be tested for ATP1A2 gene mutations. Many patients with HM do not demonstrate any of these known gene mutations.
Codes
ICD10CM:G43.409 – Hemiplegic migraine, not intractable, without status migrainosus
SNOMEDCT:
59292006 – Hemiplegic migraine
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Migraine with aura
- Intracerebral hemorrhage
- Cerebral infarction
- Transient ischemic attack
- Arteriovenous malformations
- Cerebral amyloid angiopathy
- Subarachnoid hemorrhage
- Cavernous sinus thrombosis
- Postictal Todd paralysis
- Stroke-like migraine attacks after radiation therapy (SMART) syndrome
- Reversible cerebral vasoconstriction syndrome
- Pseudotumor cerebri
- Infectious meningitis or encephalitis
- Neurosarcoidosis
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Therapy
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References
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Last Reviewed:01/18/2022
Last Updated:02/27/2022
Last Updated:02/27/2022