KCNT1 encodes a sodium-activated potassium channel that acts as an important mediator of neuronal membrane excitability 1. The channel regulates delayed outward potassium currents and neuronal bursting in sensory neurons, contributing to synaptic development and plasticity. KCNT1 mutations predominantly cause gain-of-function effects that disrupt potassium channel function, altering neuronal excitability and impairing network stability 2. These variants are associated with a spectrum of severe early-onset epileptic disorders, with KCNT1 mutations identified in approximately 27% of epilepsy of infancy with migrating focal seizures (EIMFS) cases, making it the most frequently implicated gene in this condition 3. Additional phenotypes include developmental and epileptic encephalopathies and autosomal dominant sleep-related hypermotor epilepsy, characterized by drug-resistant seizures and neurodevelopmental impairment 4. Only missense mutations and one in-frame deletion have been documented, with sleep-related hypermotor epilepsy-associated mutations clustering around the RCK2 domain 4. Current clinical management relies on alternative therapies, with ketogenic diet benefiting 62.5% of EIMFS patients and cannabidiol showing promise in 50% of cases, while conventional anti-seizure medications prove rarely beneficial 5. Emerging precision therapies include gene therapy, small-molecule modulators, and quinidine 2, representing a shift toward mechanism-based treatment strategies 6.