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000285348 037__ $$aDZNE-2026-00214
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000285348 1001_ $$00000-0001-6185-8535$$aNagel, Gabriele$$b0
000285348 245__ $$aIncidence and Survival Rates of Frontotemporal Lobar Degeneration: Population-Based Registry Study.
000285348 260__ $$aPhiladelphia, Pa.$$bWolters Kluwer$$c2026
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000285348 520__ $$aFrontotemporal 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.
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000285348 650_2 $$2MeSH$$aHumans
000285348 650_2 $$2MeSH$$aMale
000285348 650_2 $$2MeSH$$aFemale
000285348 650_2 $$2MeSH$$aRegistries
000285348 650_2 $$2MeSH$$aIncidence
000285348 650_2 $$2MeSH$$aAged
000285348 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: epidemiology
000285348 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: mortality
000285348 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: diagnosis
000285348 650_2 $$2MeSH$$aMiddle Aged
000285348 650_2 $$2MeSH$$aGermany: epidemiology
000285348 650_2 $$2MeSH$$aSurvival Rate
000285348 650_2 $$2MeSH$$aAged, 80 and over
000285348 7001_ $$0P:(DE-2719)9002267$$aPeter, Raphael$$b1
000285348 7001_ $$aUzelac, Zeljko$$b2
000285348 7001_ $$aWernecke, Deborah$$b3
000285348 7001_ $$aNiehaus, Ludwig$$b4
000285348 7001_ $$aTrottenberg, Thomas$$b5
000285348 7001_ $$aJöbges, Michael$$b6
000285348 7001_ $$aDettmers, Christian$$b7
000285348 7001_ $$aBäzner, Hansjörg$$b8
000285348 7001_ $$aBörtlein, Andreas$$b9
000285348 7001_ $$aAlthaus, Katharina$$b10
000285348 7001_ $$aMayer-Freitag, Kristina$$b11
000285348 7001_ $$aRatzka, Peter$$b12
000285348 7001_ $$aNaumann, Markus$$b13
000285348 7001_ $$aLindner, Alfred$$b14
000285348 7001_ $$aChatzikonstantinou, Anastasios$$b15
000285348 7001_ $$aAndres, Frank$$b16
000285348 7001_ $$aArnold, Guy$$b17
000285348 7001_ $$aBlickhan, Marko$$b18
000285348 7001_ $$aOpherk, Christian$$b19
000285348 7001_ $$00000-0003-4187-9472$$aKnier, Benjamin$$b20
000285348 7001_ $$aErtl, Michael$$b21
000285348 7001_ $$aMetrikat, Jens$$b22
000285348 7001_ $$aHuber, Roman$$b23
000285348 7001_ $$aThomas, Christine$$b24
000285348 7001_ $$aKozian, Ralf$$b25
000285348 7001_ $$aKimmig, Hubert$$b26
000285348 7001_ $$aDemuth, Klaus$$b27
000285348 7001_ $$aHecht, Martin$$b28
000285348 7001_ $$00000-0002-3770-7857$$aFoerch, Christian$$b29
000285348 7001_ $$aKloetsch, Christof$$b30
000285348 7001_ $$aReinhard, Matthias$$b31
000285348 7001_ $$aBengel, Dietmar$$b32
000285348 7001_ $$aNeuhaus, Oliver$$b33
000285348 7001_ $$00000-0001-8309-7685$$aButtmann, Mathias$$b34
000285348 7001_ $$00000-0002-9570-593X$$aVolkmann, Jens$$b35
000285348 7001_ $$aPinkhardt, Elmar$$b36
000285348 7001_ $$aLichy, Christoph$$b37
000285348 7001_ $$0P:(DE-2719)2000055$$aLaske, Christoph$$b38$$udzne
000285348 7001_ $$aBeattie, James$$b39
000285348 7001_ $$00000-0002-4319-5034$$aHäckert, Jan$$b40
000285348 7001_ $$0P:(DE-2719)9001441$$aJesse, Sarah$$b41$$udzne
000285348 7001_ $$0P:(DE-2719)9002137$$aBrenner, David$$b42$$udzne
000285348 7001_ $$0P:(DE-2719)9000455$$aWeishaupt, Jochen$$b43
000285348 7001_ $$00000-0003-4273-4267$$aOtto, Markus$$b44
000285348 7001_ $$aUttner, Ingo$$b45
000285348 7001_ $$00000-0002-4850-3091$$aAnderl-Straub, Sarah$$b46
000285348 7001_ $$0P:(DE-2719)9002172$$aLule, Dorothee$$b47$$udzne
000285348 7001_ $$0P:(DE-2719)9002270$$aRothenbacher, Dietrich$$b48
000285348 7001_ $$0P:(DE-2719)9002269$$aRosenbohm, Angela$$b49
000285348 7001_ $$0P:(DE-2719)2812633$$aLudolph, Albert Christian$$b50$$eLast author
000285348 7001_ $$aGroup, ALS-FTLD Registry Swabia Study$$b51$$eCollaboration Author
000285348 773__ $$0PERI:(DE-600)1491874-2$$a10.1212/WNL.0000000000214482$$gVol. 106, no. 6, p. e214482$$n6$$pe214482$$tNeurology$$v106$$x0028-3878$$y2026
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