April 25, 2017 E-Pearl of the Week: Hemiplegic migraine

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April 25, 2017

Hemiplegic migraine

Hemiplegic migraine, a rare form of migraine with aura, has a sporadic and a familial form with similar clinical phenotypes. Unusual migraine triggers include minor head trauma and cerebral or coronary angiogram1, 2. Thorough investigation to rule out other etiologies is required on first presentation. Medications which have shown positive effects in case reports include flunarizine, verapamil, sodium valproate, lamotrigine and acetazolamide. Familial hemiplegic migraine is diagnosed when at least one family member also has hemiplegic migraines1, 2 and is linked to genes CACNA1A, ATP1A2, and SCN1A. Using mouse models targeting these genes, research is ongoing to help further elucidate migraine pathophysiology2.


  1. Pelzer N, Stam AH, Haan J, Ferrari MD, Terwindt GM. Familial and sporadic hemiplegic migraine: diagnosis and treatment. Curr Treat Options Neurol 2013; 15: 13-27.
  2. Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol 2011; 10: 457-470.

Submitted by Ilena George, MD, Department of Neurology, Yale School of Medicine - New Haven, CT

Dr. George is a member of the Resident & Fellow Section of Neurology.

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