ATP1A2 encodes the catalytic alpha-2 subunit of the Na+/K+-ATPase pump, which catalyzes ATP hydrolysis coupled with sodium-potassium ion exchange across the plasma membrane 1. This ion exchange establishes the electrochemical gradient essential for cellular signaling and active transport of nutrients. The pump functions in neurons and glial cells, where it maintains optimal intracellular ion homeostasis 2. Mutations in ATP1A2 cause familial hemiplegic migraine (FHM2), characterized by migraine attacks with transient motor weakness 23. These mutations increase neurotransmitter and potassium ion levels at synaptic clefts, facilitating cortical spreading depolarization underlying migraine aura 2. Heterozygous ATP1A2 mutations also cause alternating hemiplegia of childhood and developmental epileptic encephalopathy, with severe mutations producing polymicrogyria and early lethality 1. In vitro studies demonstrate that disease-associated mutations cause severe loss of Na+/K+-ATPase pump function 1. Beyond neurological disease, ATP1A2 participates in brown adipose tissue thermogenesis regulation 4. Clinical management of ATP1A2-related hemiplegic migraine includes antiepileptic drugs (lamotrigine, valproate, topiramate), calcium channel blockers (flunarizine, verapamil), and acetazolamide 5. Diagnosis requires genetic testing combined with clinical evaluation, as penetrance varies among mutation carriers.