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Symptoms of<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>can present in individuals in their 20s through to their 90s, but the mean age at onset is in the sixth decade. The most common presentation is with a change in personality and impaired social conduct (behavioural variant<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>). Less frequently patients present with language problems (primary progressive aphasia). Both of these groups of patients can develop motor features consistent with either motor neuron disease (usually the amyotrophic lateral sclerosis variant) or parkinsonism (most commonly a progressive supranuclear palsy or corticobasal syndrome). In about a third of cases<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>is familial, with mutations in the progranulin, microtubule\u2010associated protein tau and chromosome 9 open reading frame 72 genes being the major causes. Mutations in a number of other genes including<jats:styled-content style=\"fixed-case\">TANK<\/jats:styled-content>\u2010binding kinase 1 are rare causes of familial<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>. This review aims to clarify the often confusing terminology of<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>, and outline the various clinical features and diagnostic criteria of sporadic and familial<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>syndromes. It will also discuss the current major challenges in<jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>research and clinical practice, and potential areas for future research.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p><jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc13654-fig-0001-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text>This review clarifies the terminology of frontotemporal dementia (FTD) and summarizes the various clinical features and most recent diagnostic criteria of sporadic and familial FTD syndromes. It also discusses the current major challenges in FTD research and clinical practice, and highlights potential areas for future research.<\/jats:p><jats:p><jats:bold>This article is part of the<\/jats:bold>\u00a0<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/onlinelibrary.wiley.com\/journal\/10.1111\/(ISSN)1471-4159\/homepage\/special_issues.htm\">Frontotemporal Dementia special issue<\/jats:ext-link>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jnc.13654","type":"journal-article","created":{"date-parts":[[2016,5,4]],"date-time":"2016-05-04T15:10:53Z","timestamp":1462374653000},"page":"6-31","source":"Crossref","is-referenced-by-count":132,"title":["The clinical spectrum of sporadic and familial forms of frontotemporal dementia"],"prefix":"10.1111","volume":"138","author":[{"given":"Ione O. 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