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This article will review currently available therapies for FTD, its related disorders and their clinical evidence. It will also discuss recent advancements in FTD pathophysiology, treatment development, biomarker advancement and their relation to recently completed or\u00a0currently ongoing clinical trials as well as future implications.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>\n<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\/jnc13640-fig-0001-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text>\nFrontotemporal dementia (FTD) is a type of dementia with distinct clinical syndromes. Current treatments involve off\u2010label use of medications for symptomatic management and cannot modify disease course. Advancements in FTD pathophysiology, genetics, and biomarkers have led to development of small molecules targeting the underlying pathology in hopes of achieving a disease\u2010modifying effect. This article will review current therapies for FTD, discuss advancements in FTD pathophysiology, therapy development, biomarker advancement, their relation to recent clinical trials and future implications.<\/jats:p><jats:p><jats:bold>This article is part of the<\/jats:bold> <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>.\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/jnc.13640","type":"journal-article","created":{"date-parts":[[2016,6,16]],"date-time":"2016-06-16T05:50:52Z","timestamp":1466056252000},"page":"211-221","source":"Crossref","is-referenced-by-count":138,"title":["Therapy and clinical trials in frontotemporal dementia: past, present, and future"],"prefix":"10.1111","volume":"138","author":[{"given":"Richard M.","family":"Tsai","sequence":"first","affiliation":[{"name":"Assistant Adjunct Professor of Neurology University of California San Francisco Department of Neurology San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam L.","family":"Boxer","sequence":"additional","affiliation":[{"name":"Associate Professor of Neurology University of California San Francisco Department of Neurology San Francisco California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,6,15]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0062402"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-014-1683-y"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00158a027"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2015.06.008"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1176\/appi.neuropsych.12060158"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31827f0fd1"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2013.02.004"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2361-07.2007"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(15)00461-4"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10211"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2011.07337.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/WAD.0b013e318197852f"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2009.204081"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(12)70320-4"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jalz.2012.03.003"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(14)70088-2"},{"key":"e_1_2_8_18_1","first-page":"5040","article-title":"Microtubule\u2010stabilizing agents as potential therapeutics for neurodegenerative disease","volume":"5","author":"Brunden K. 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