Mutations in the KCNA1 gene encoding the Kv1.1 voltage‐gated potassium (K+) channel have been linked to episodic ataxia type 1 (EA1), a rare neurological syndrome characterized by continuous myokymia and attacks of generalized ataxia that can be triggered by fever, abrupt movements, emotional stress, and fatigue. Here, KCNA1 is linked to episodic ataxia type 1.