Episodic ataxias are a clinically and genetically heterogeneous group of neurological disorders that are characterized by attacks of incoordination and imbalance. The prevalence of epilepsy in episodic ataxias type 1 (EA1) patients is approximately ten times that in the general population. To support a suspected diagnosis, the patient should undergo electromyography for myokymia. It should be explained that EA1 is in principle a benign condition. Episodic ataxias type 2 (EA2) is by far the most common episodic ataxia but precise data about the prevalence are missing. Based on the pathophysiological understanding of EA2, two pharmacologic approaches were established. They focus on modulation of the pH level and membrane ion conductance. Today, so far acetazolamide is the drug of choice for the preventive treatment of EA2. Aminopyridines may prevent attacks in EA2 (and improve downbeat nystagmus) by increasing the release of GABA in the Purkinje cells.