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This study aimed to identify factors that predict survival and clinical progression in bv\n                      <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Consecutive patients with clinically probable bv\n                      <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>\n                      were prospectively followed up over an 8\u2010year period. Baseline neuropsychological variables, presence of a known pathogenic frontotemporal dementia gene mutation and a systematic visual magnetic resonance imaging assessment at baseline were examined as candidate predictors using multivariate modelling.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      After screening 121 cases, the study cohort consisted of 75 patients with probable bv\n                      <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>\n                      , with a mean age of 60.8\u00a0\u00b1\u00a08.5\u00a0years, followed up for a mean duration of 7.2\u00a0\u00b1\u00a03.5\u00a0years from symptom onset. Median survival time from disease onset was 10.8\u00a0years and median survival, prior to transition to nursing home, was 8.9\u00a0years. A total of 25 of the 75 patients died during the study follow\u2010up period. Survival without dependence was predicted by shorter disease duration at presentation (hazard ratio, 0.49,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.001), greater atrophy in the anterior cingulate cortex (hazard ratio, 1.75,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.047), older age (hazard ratio, 1.07,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.026) and a higher burden of behavioural symptoms (hazard ratio, 1.04,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.015).\n                    <\/jats:p>\n                    <jats:p>\n                      In terms of disease progression, presence of a known pathogenic frontotemporal dementia mutation (\n                      <jats:italic>\u03b2\u00a0<\/jats:italic>\n                      =\u00a00.46,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0&lt;\u00a00.001) was the strongest predictor of progression. Deficits in letter fluency (\n                      <jats:italic>\u03b2\u00a0<\/jats:italic>\n                      =\u00a0\u22120.43,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.017) and greater atrophy in the motor cortex (\n                      <jats:italic>\u03b2\u00a0<\/jats:italic>\n                      =\u00a00.51,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.03) were also associated with faster progression.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      This study provides novel clinical predictors of survival and progression in bv\n                      <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>\n                      . Our findings are likely to have an impact on prognostication and care planning in this difficult disease.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.13887","type":"journal-article","created":{"date-parts":[[2018,12,19]],"date-time":"2018-12-19T01:43:45Z","timestamp":1545183825000},"page":"774-779","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Predictors of survival and progression in behavioural variant frontotemporal dementia"],"prefix":"10.1111","volume":"26","author":[{"given":"S.","family":"Agarwal","sequence":"first","affiliation":[{"name":"Neurology Unit Addenbrooke's Hospital Cambridge UK"},{"name":"Central Clinical School and Brain and Mind Centre The University of Sydney Sydney New South Wales"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. M.","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Central Clinical School and Brain and Mind Centre The University of Sydney Sydney New South Wales"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"D'Mello","sequence":"additional","affiliation":[{"name":"School of Psychology and Brain and Mind Centre The University of Sydney Sydney New South Wales"},{"name":"ARC Centre of Excellence in Cognition and its Disorders Sydney New South Wales"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Foxe","sequence":"additional","affiliation":[{"name":"School of Psychology and Brain and Mind Centre The University of Sydney Sydney New South Wales"},{"name":"ARC Centre of Excellence in Cognition and its Disorders Sydney New South Wales"},{"name":"Neuroscience Research Australia (NeuRA) University of New South Wales Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Kaizik","sequence":"additional","affiliation":[{"name":"Central Clinical School and Brain and Mind Centre The University of Sydney Sydney New South Wales"},{"name":"Neuroscience Research Australia (NeuRA) University of New South Wales Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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