Pathogenic CACNA1A mutations can result in paroxysmal attacks of encephalopathy, hemiplegia and cerebral edema. We report two patients with CACNA1A-associated encephalopathy, hemiplegia and contralateral hemispheric cerebral edema treated successfully with intravenous magnesium sulfate and dexamethasone. One patient met the clinical criteria for familial hemiplegic migraine. There is a paucity of guidance in the literature on how to manage these patients. Despite some discrepancies in the treatment protocols in our two cases, they indicate that magnesium and dexamethasone could be part of the treatment algorithm for these patients. Further research to delineate appropriate dosing and duration of therapy is needed.

Stroke is relatively rare in children but has become increasingly recognized clinically. Hemiplegic migraine (HM) is a rare subtype of migraine, with attacks typically beginning in childhood or adolescence. Attacks are characterized by migraine headaches and motor weakness, which develop over several minutes. HM may therefore mimic acute stroke; however, symptoms last less than an hour and resolve spontaneously, often without sequela.1–4 Distinction between these entities is important due to their different urgency and management. Neuroimaging is indispensible in working up patients presenting to the Emergency Department with stroke-like symptoms and can be used to distinguish between infarction and HM.