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Based on the constellation of symptoms, <jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> has been recently classified into a nonfluent, semantic, or logopenic variant. Nonfluent variant <jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> is characterized by dysfluent and effortful speech, often combined with agrammatism. Also, some patients have initially predominant apraxia of speech. The neuroimaging findings in nfv<jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> are in most cases progressive atrophy within the left inferior, opercular, and insular regions. Pathology is a tauopathy (<jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>\u2010T), most often Pick's disease or <jats:styled-content style=\"fixed-case\">CBD<\/jats:styled-content>. Semantic variant <jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content>, on the other hand is characterized by fluent, but circumlocutory speech, then severe anomia and word\u2010finding difficulties, all being associated with a progressive loss of lexical\u2010semantic knowledge. As the disease progresses, the semantic impairment typically becomes multimodal. The clinical picture of sv<jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> is often associated with atrophy of the anterior regions of the temporal lobes, usually more prominent on the left side. The majority of these patients have <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043 pathology. The third, most recently described form of <jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> is the logopenic variant characterized by decreased spontaneous speech output with frequent word\u2010finding pauses, phonologic parahpasias, and repetition deficits. It resembles aphasia in Alzheimer's disease. Imaging abnormalities in lv<jats:styled-content style=\"fixed-case\">PPA<\/jats:styled-content> have been predominantly found in the left temporo\u2010parietal junction area, and the pathological changes have been often those of <jats:styled-content style=\"fixed-case\">AD<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/sjop.12105","type":"journal-article","created":{"date-parts":[[2014,4,10]],"date-time":"2014-04-10T07:07:36Z","timestamp":1397113656000},"page":"191-201","source":"Crossref","is-referenced-by-count":16,"title":["Primary progressive aphasia"],"prefix":"10.1111","volume":"55","author":[{"given":"Andrew","family":"Kertesz","sequence":"first","affiliation":[{"name":"Department of Clinical Neurological Sciences Western University London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micha\u0142","family":"Harciarek","sequence":"additional","affiliation":[{"name":"Division of Clinical Psychology and Neuropsychology Institute of Psychology University of Gda\u0144sk Gda\u0144sk Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,4,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl285"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awm213"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0093-934X(82)90006-2"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bandl.2009.12.001"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroling.2008.12.001"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.51.9.1201"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0093-934X(92)90079-T"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2008.11.008"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2010.06.007"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1355617706060255"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1355617707070269"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(12)70320-4"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.69.2.178"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0028-3932(00)00034-8"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1080\/02643299408251987"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1037\/a0020287"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1355617799566034"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cortex.2007.07.005"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1996.tb34426.x"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0028-3932(01)00035-5"},{"key":"e_1_2_7_22_1","volume-title":"Etude clinique de la maladie Pick","author":"Caron M.","year":"1934"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1992.00530310118022"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp037"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.92"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000280574.17166.26"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/113.2.397"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.wad.0000155069.23937.d2"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1355617700000163"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bandl.2004.11.007"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0010-9452(08)70667-1"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/0028-3932(94)00066-2"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9568.2004.03710.x"},{"key":"e_1_2_7_34_1","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1017\/CBO9781316135457.008","volume-title":"Frontotemporal dementia syndromes","author":"Davies R. 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