{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:15:49Z","timestamp":1787015749132,"version":"build-2736575974"},"reference-count":38,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2015,5,2]],"date-time":"2015-05-02T00:00:00Z","timestamp":1430524800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"La Fundaci\u00f3 Marat\u00f3 TV3 2013"},{"name":"Fundaci\u00f3n Tatiana Perez de Guzm\u00e1n el Bueno 2013"},{"name":"Spanish Ministry of Health","award":["FIS PI11\/00234"],"award-info":[{"award-number":["FIS PI11\/00234"]}]},{"name":"Spanish Ministerio de Econom\u00eda y Competividad programa","award":["PTA 2011"],"award-info":[{"award-number":["PTA 2011"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>\n                      The aims of this study were to assess the sensitivity of the\n                      <jats:styled-content style=\"fixed-case\">I<\/jats:styled-content>\n                      nternational\n                      <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>\n                      ehavioural\n                      <jats:styled-content style=\"fixed-case\">V<\/jats:styled-content>\n                      ariant\n                      <jats:styled-content style=\"fixed-case\">FTD C<\/jats:styled-content>\n                      riteria\n                      <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>\n                      onsortium (\n                      <jats:styled-content style=\"fixed-case\">FTDC<\/jats:styled-content>\n                      ) revised criteria of behavioural variant frontotemporal dementia (\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      ) in a pathological cohort and to determine their predictive values in a clinical context suggestive of\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      . In addition, the study aimed to assess the influence of the age at onset and underlying pathology in the clinicopathological correlations.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Retrospective, blinded review of the clinical and neuropathological data from the\n                      <jats:styled-content style=\"fixed-case\">N<\/jats:styled-content>\n                      eurological\n                      <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>\n                      issue\n                      <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>\n                      ank of the\n                      <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>\n                      iobank\n                      <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>\n                      ospital\n                      <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>\n                      linic\u2013\n                      <jats:styled-content style=\"fixed-case\">IDIBAPS<\/jats:styled-content>\n                      ,\n                      <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>\n                      arcelona (\n                      <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>\n                      pain) was conducted, assessing the fulfilment of the diagnostic criteria on a case\u2010by\u2010case basis. Two separate nonexclusive cohorts were selected: Cohort 1 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200958) subjects with pathological diagnosis of frontotemporal lobar degeneration (\n                      <jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>\n                      ) and Cohort 2 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200966) subjects with the\n                      <jats:italic>premortem<\/jats:italic>\n                      diagnosis of\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The\n                      <jats:styled-content style=\"fixed-case\">FTDC<\/jats:styled-content>\n                      criteria reached a sensitivity of 93% for possible and 80% for probable\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      . Early\u2010onset cases displayed significantly more disinhibition, loss of empathy and compulsive behaviour with respect to late\u2010onset\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      , leading to a slightly higher sensitivity of the diagnostic criteria (97%\n                      <jats:italic>vs.<\/jats:italic>\n                      91%). There were no differences in the diagnostic performance between tau\u2010positive and tau\u2010negative cases. In subjects clinically diagnosed as\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      , a \u2018possible\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      \u2019 diagnosis reached a positive predictive value for\n                      <jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>\n                      pathology of 90%, irrespective of underlying proteinopathy. False\u2010positive clinical diagnoses were mainly\n                      <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\n                      lzheimer's disease. These cases were significantly older, had less family history of dementia and had a predominantly apathetic clinical picture.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      The revised\n                      <jats:styled-content style=\"fixed-case\">bvFTD<\/jats:styled-content>\n                      criteria present good sensitivity and positive predictive value in both early\u2010 and late\u2010onset cases and regardless of the underlying\n                      <jats:styled-content style=\"fixed-case\">FTLD<\/jats:styled-content>\n                      pathology.\n                    <\/jats:p>\n                  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