Incidence and Survival Rates of Frontotemporal Lobar Degeneration: Population-Based Registry Study.
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Nagel Gabriele, Peter Raphael Simon, Uzelac Zeljko, Wernecke Deborah, Niehaus Ludwig, Trottenberg Thomas, Jöbges Michael, Dettmers Christian, Bäzner Hansjörg, Börtlein Andreas, Althaus Katharina, Mayer-Freitag Kristina, Ratzka Peter, Naumann Markus, Lindner Alfred, Chatzikonstantinou Anastasios, Andres Frank, Arnold Guy, Blickhan Marko, Opherk Christian, Knier Benjamin, Ertl Michael, Metrikat Jens, Huber Roman, Thomas Christine, Kozian Ralf, Kimmig Hubert, Demuth Klaus, Hecht Martin, Foerch Christian, Kloetsch Christof, Reinhard Matthias, Bengel Dietmar, Neuhaus Oliver, Buttmann Mathias, Volkmann Jens, Pinkhardt Elmar, Lichy Christoph, Laske Christoph, Beattie James, Häckert Jan, Jesse Sarah, Brenner David, Weishaupt Jochen, Otto Markus, Uttner Ingo, Anderl-Straub Sarah, Lulé Dorothée E, Rothenbacher Dietrich, Rosenbohm Angela, Ludolph Albert Christian, ALS-FTLD Registry Swabia Study Group
Аннотация
METHODS: 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.
RESULTS: 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.
DISCUSSION: 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.