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Ataxia episodes are usually of short duration and often triggered by specific stimuli. There are currently seven classified subtypes of <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content>. <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> types 1 and 2 have the highest prevalence and are therefore the clinically most relevant. Between attacks, <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 1 is associated with myokymia. In <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 2, often an interictal downbeat nystagmus with other cerebellar ocular dysfunctions is present; patients with <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 2 may display slowly progessive ataxia and vermian atrophy. <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 1 and 2 are both channelopathies, affecting the potassium channel gene, <jats:italic><jats:styled-content style=\"fixed-case\">KCNA<\/jats:styled-content>1<\/jats:italic>, in <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 1 and the <jats:styled-content style=\"fixed-case\">PQ<\/jats:styled-content> calcium channel\u2010encoding gene, <jats:italic><jats:styled-content style=\"fixed-case\">CACNA<\/jats:styled-content>1A<\/jats:italic>, in <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 2. The types <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 3 to 7 are very rare and have to be further elucidated. Here, we review the historical, clinical, and genetic aspects of autosomal\u2010dominant <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content>s and their current treatment, focusing on <jats:styled-content style=\"fixed-case\">EA<\/jats:styled-content> 1 and 2.<\/jats:p>","DOI":"10.1002\/mdc3.12075","type":"journal-article","created":{"date-parts":[[2014,7,28]],"date-time":"2014-07-28T06:45:25Z","timestamp":1406529925000},"page":"285-290","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["The Clinical Spectrum of Autosomal\u2010Dominant Episodic Ataxias"],"prefix":"10.1002","volume":"1","author":[{"given":"Stefan","family":"Kipfer","sequence":"first","affiliation":[{"name":"Department of Neurology Kantonsspital Olten Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Strupp","sequence":"additional","affiliation":[{"name":"Department of Neurology and German 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