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Most cases are now known to be caused by pathogenic variants in the sodium channel gene <jats:italic><jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>1A<\/jats:italic>, but several other genes have also been implicated. This review examines current understanding of the role of non\u2010<jats:italic><jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>1A<\/jats:italic> genes in <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>, and what is known about phenotypic similarities and differences. We discuss whether these are best thought of as minority causes of <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>, or as similar but distinct conditions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Based on a review of literature, a list of genes linked to DS was compiled and PubMed was searched for reports of <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>\u2010like phenotypes arising from variants in each. Online Mendelian Inheritance in Man (<jats:styled-content style=\"fixed-case\">OMIM<\/jats:styled-content>) was used to identify further reports relevant to each gene.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Genes that have been reported to cause <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>\u2010like phenotypes include <jats:italic><jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>2A, <jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>8A, <jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>9A, <jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>1B, <jats:styled-content style=\"fixed-case\">PCDH<\/jats:styled-content>19, <jats:styled-content style=\"fixed-case\">GABRA<\/jats:styled-content>1, <jats:styled-content style=\"fixed-case\">GABRG<\/jats:styled-content>2, <jats:styled-content style=\"fixed-case\">STXBP<\/jats:styled-content>1, <jats:styled-content style=\"fixed-case\">HCN<\/jats:styled-content>1, <jats:styled-content style=\"fixed-case\">CHD<\/jats:styled-content>2<\/jats:italic>, and <jats:italic><jats:styled-content style=\"fixed-case\">KCNA<\/jats:styled-content>2<\/jats:italic>. Many of these genes, however, appear to be associated with their own, different, clinical picture. Other candidate genes for <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content> have been reported, but there is currently an insufficient body of literature to support their causative role.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Although most cases of <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content> arise from <jats:italic><jats:styled-content style=\"fixed-case\">SCN<\/jats:styled-content>1A<\/jats:italic> variants, numerous other genes cause encephalopathies that are clinically similar. Increasingly, a tendency is noted to define newly described epileptic disorders primarily in genetic terms, with clinical features being linked to genotypes. As genetic diagnosis becomes more readily available, its potential to guide pathophysiologic understanding and therapeutic strategy cannot be ignored. Clinical assessment remains essential; the challenge now is to develop a gene\u2010based taxonomy that complements traditional syndromic classifications, allowing elements of both to inform new approaches to treatment.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.13889","type":"journal-article","created":{"date-parts":[[2017,9,7]],"date-time":"2017-09-07T18:41:14Z","timestamp":1504809674000},"page":"1807-1816","source":"Crossref","is-referenced-by-count":173,"title":["Dravet syndrome and its mimics: Beyond <i><scp>SCN<\/scp>1A<\/i>"],"prefix":"10.1111","volume":"58","author":[{"given":"Dora","family":"Steel","sequence":"first","affiliation":[{"name":"The Paediatric Neurosciences Research Group Royal Hospital for Children Glasgow United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph D.","family":"Symonds","sequence":"additional","affiliation":[{"name":"The Paediatric Neurosciences Research Group Royal Hospital for Children Glasgow United 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