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Additionally, to correlate NfL levels to clinical and neuroimaging parameters.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this multicenter case\u2013control study, we investigated\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      NfL in 174 subjects (48 controls, 40 presymptomatic carriers and 86 patients with\n                      <jats:italic>microtubule\u2010associated protein tau<\/jats:italic>\n                      (\n                      <jats:italic>\n                        <jats:styled-content style=\"fixed-case\">MAPT<\/jats:styled-content>\n                      <\/jats:italic>\n                      ),\n                      <jats:italic>progranulin<\/jats:italic>\n                      (\n                      <jats:italic>\n                        <jats:styled-content style=\"fixed-case\">GRN<\/jats:styled-content>\n                      <\/jats:italic>\n                      ), and\n                      <jats:italic>chromosome 9 open reading frame 72<\/jats:italic>\n                      (\n                      <jats:italic>C9orf72<\/jats:italic>\n                      ) mutations), and serum NfL in 118 subjects (39 controls, 44 presymptomatic carriers, 35 patients). In 55 subjects both\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      and serum was determined. In two subjects\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      was available before and after symptom onset (converters). Additionally, NfL levels were correlated with clinical parameters, survival, and regional brain atrophy.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      NfL levels in patients (median 6762 pg\/mL, interquartile range 3186\u20139309 pg\/mL) were strongly elevated compared with presymptomatic carriers (804 pg\/mL, 627\u20131173 pg\/mL,\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.001), resulting in a good diagnostic performance to discriminate both groups. Serum NfL correlated highly with\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      NfL (\n                      <jats:italic>r<\/jats:italic>\n                      <jats:sub>\n                        <jats:italic>s<\/jats:italic>\n                      <\/jats:sub>\n                      = 0.87,\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.001) and was similarly elevated in patients. Longitudinal samples in the converters showed a three\u2010 to fourfold increase in\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      NfL after disease onset. Additionally, NfL levels in patients correlated with disease severity, brain atrophy, annualized brain atrophy rate and survival.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>\n                      NfL in both serum and\n                      <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>\n                      has the potential to serve as a biomarker for clinical disease onset and has a prognostic value in genetic\n                      <jats:styled-content style=\"fixed-case\">FTD<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  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