Toward this aim, we (i) reviewed all available clinical reports on treatment response and functional consequences of all KCNA1 variants causing EA1, (ii) examined the potential effects on neuronal excitability of all variants using a single compartment conductance-based model and set out to assess two SCBs (carbamazepine and riluzole) regarding their potential to restore the identified underlying pathophysiological mechanisms of KCNA1 variant channels, and (iii) provide a comprehensive review of the literature considering all types of episodic ataxia. The gene discussed is KCNA1; the disease is episodic ataxia type 1.