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@ARTICLE{Nagel:285348,
      author       = {Nagel, Gabriele and Peter, Raphael and Uzelac, Zeljko and
                      Wernecke, Deborah and Niehaus, Ludwig and Trottenberg,
                      Thomas and Jöbges, Michael and Dettmers, Christian and
                      Bäzner, Hansjörg and Börtlein, Andreas and Althaus,
                      Katharina and Mayer-Freitag, Kristina and Ratzka, Peter and
                      Naumann, Markus and Lindner, Alfred and Chatzikonstantinou,
                      Anastasios and Andres, Frank and Arnold, Guy and Blickhan,
                      Marko and Opherk, Christian and Knier, Benjamin and Ertl,
                      Michael and Metrikat, Jens and Huber, Roman and Thomas,
                      Christine and Kozian, Ralf and Kimmig, Hubert and Demuth,
                      Klaus and Hecht, Martin and Foerch, Christian and Kloetsch,
                      Christof and Reinhard, Matthias and Bengel, Dietmar and
                      Neuhaus, Oliver and Buttmann, Mathias and Volkmann, Jens and
                      Pinkhardt, Elmar and Lichy, Christoph and Laske, Christoph
                      and Beattie, James and Häckert, Jan and Jesse, Sarah and
                      Brenner, David and Weishaupt, Jochen and Otto, Markus and
                      Uttner, Ingo and Anderl-Straub, Sarah and Lule, Dorothee and
                      Rothenbacher, Dietrich and Rosenbohm, Angela and Ludolph,
                      Albert Christian},
      collaboration = {Group, ALS-FTLD Registry Swabia Study},
      title        = {{I}ncidence and {S}urvival {R}ates of {F}rontotemporal
                      {L}obar {D}egeneration: {P}opulation-{B}ased {R}egistry
                      {S}tudy.},
      journal      = {Neurology},
      volume       = {106},
      number       = {6},
      issn         = {0028-3878},
      address      = {Philadelphia, Pa.},
      publisher    = {Wolters Kluwer},
      reportid     = {DZNE-2026-00214},
      pages        = {e214482},
      year         = {2026},
      abstract     = {Frontotemporal lobar degeneration (FTLD) can present as a
                      behavioral or language variant (bvFTLD or a primary
                      progressive aphasia [PPA], or as a syndrome with
                      parkinsonism, such as corticobasal syndrome [CBS] or
                      progressive supranuclear palsy [PSP]). The incidence of FTLD
                      varies in epidemiologic studies, reaching 3 per 100,000
                      person-years. Only few data exist regarding survival times.
                      We evaluated incidence and survival rates in a
                      population-based registry with high coverage in Southern
                      Germany.The epidemiologic ALS-FTLD registry Swabia covers a
                      population of 8.4 million inhabitants in south-west Germany.
                      Raw and age-standardized incidence rates, as well as
                      incidence rate ratios (IRR) with $95\%$ CIs were calculated.
                      Median survival time was estimated for different FTLD
                      variants using the Kaplan-Meier method.Between 2015 and
                      2022, 515 patients with FTLD (mean age at diagnosis 68.0 ±
                      9.5 years, $59.8\%$ men) were registered. The median
                      diagnostic delay was 24.8 months. The most common variant
                      was bvFTLD (n = 185, $35.9\%;$ $66.5\%$ men), followed by
                      PPA (n = 147, $28.5\%;$ $51.0\%$ men), PSP (n = 133,
                      $25.8\%;$ $62.9\%$ men), and CBS (n = 22, $4.3\%;$ $50\%$
                      men). The overall FTLD incidence was 0.77 $(95\%$ CI
                      0.71-0.84), and the age-standardized incidence was 0.76
                      $(95\%$ CI 0.69-0.82) per 100.000 person-years. The
                      age-standardized incidence was higher in men than in women,
                      with an IRR of 1.73 $(95\%$ CI 1.44-2.00). In men, incidence
                      increased from the age 50 years, primarily due to bvFTD,
                      whereas in women this rise was primarily due to PSP. The
                      median survival (N = 392) from diagnosis was 53.6 months
                      $(95\%$ CI 50.9-62.0) overall, 73.1 months $(95\%$ CI
                      63.6-82.8) for patients with PPA, 42.8 months $(95\%$ CI
                      35.1-64.3) for patients with bvFTD, and 49.5 months $(95\%$
                      CI 39.2-53.7) for patients with PPS/CBS.We observed a raw
                      incidence rate of 0.77, thus considerably lower than in most
                      previous reports. Incidence was substantially higher in men
                      than in women. The prognosis from the time of diagnosis
                      depended highly on the specific FTLD subtype. Our data are
                      based on the large sample size and high capture rate of a
                      central European population-based registry.},
      keywords     = {Humans / Male / Female / Registries / Incidence / Aged /
                      Frontotemporal Lobar Degeneration: epidemiology /
                      Frontotemporal Lobar Degeneration: mortality /
                      Frontotemporal Lobar Degeneration: diagnosis / Middle Aged /
                      Germany: epidemiology / Survival Rate / Aged, 80 and over},
      cin          = {Clinical Study Center (Ulm)},
      ddc          = {610},
      cid          = {I:(DE-2719)5000077},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41730149},
      doi          = {10.1212/WNL.0000000000214482},
      url          = {https://pub.dzne.de/record/285348},
}