Home > Documents in Process > 'The DESCRIBE-ALS-FTD study: a prospective multicenter observational study of the ALS-FTD spectrum'. > print

001     281785
005     20251017161616.0
024 7 _ |a 10.1080/21678421.2025.2509617
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024 7 _ |a 2167-8421
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024 7 _ |a 2167-9223
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037 _ _ |a DZNE-2025-01178
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Hermann, Andreas
|0 P:(DE-2719)2811732
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|e First author
245 _ _ |a 'The DESCRIBE-ALS-FTD study: a prospective multicenter observational study of the ALS-FTD spectrum'.
260 _ _ |a Abingdon
|c 2025
|b Taylor Francis Group
336 7 _ |a article
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336 7 _ |a Journal Article
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336 7 _ |a ARTICLE
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520 _ _ |a Background: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) exhibit significant clinical, genetic and neuropathological abnormalities, and are regarded as belonging to a common disease spectrum, referred to as the ALS-FTD spectrum disorders. Our understanding of the underlying mechanisms of these diseases has advanced significantly, including molecular neuropathology, genetics and molecular pathophysiology. The heterogeneity of these diseases poses significant challenges to translational research and drug development, particularly in sporadic cases. Consequently, there is an urgent need to improve patient stratification for the successful execution of future clinical trials. Methods/Results: We here describe the study design of the DESCRIBE-ALS/FTD study which aims to address this research gap by undertaking a systematic sampling of patients from the ALS FTD spectrum, encompassing all possible disease variants. The main objective of the study is to systematically document detailed cross-sectional phenotyping and the temporal progression of motor and neuropsychological abnormalities that occur in both ALS and FTD. Additionally, it seeks to systematically correlate these abnormalities with genetics and potentially predictive biomarkers including longitudinal biomaterial sampling, brain imaging and brain banking. Furthermore, first-degree relatives of patients with disease-causing gene variants undergo the same assessments to also sample presymptomatic risk gene carriers. Conclusion: With this prospective registry study we aim to generate datasets which will help researchers identifying different disease traits in people with sporadic and genetic ALS and FTD and to develop biomarkers to identify preclinical and prodromal disease stages.
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588 _ _ |a Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
650 _ 7 |a Amyotrophic lateral sclerosis
|2 Other
650 _ 7 |a biomarkers
|2 Other
650 _ 7 |a disease traits
|2 Other
650 _ 7 |a frontotemporal dementia
|2 Other
650 _ 7 |a genetics
|2 Other
650 _ 7 |a neuropathology
|2 Other
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Amyotrophic Lateral Sclerosis: genetics
|2 MeSH
650 _ 2 |a Amyotrophic Lateral Sclerosis: diagnosis
|2 MeSH
650 _ 2 |a Amyotrophic Lateral Sclerosis: physiopathology
|2 MeSH
650 _ 2 |a Frontotemporal Dementia: genetics
|2 MeSH
650 _ 2 |a Frontotemporal Dementia: diagnosis
|2 MeSH
650 _ 2 |a Prospective Studies
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Aged
|2 MeSH
650 _ 2 |a Disease Progression
|2 MeSH
650 _ 2 |a Cross-Sectional Studies
|2 MeSH
693 _ _ |0 EXP:(DE-2719)DESCRIBE-FTD-20160101
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700 1 _ |a Prudlo, Johannes
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700 1 _ |a Kasper, Elisabeth
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700 1 _ |a Priller, Josef
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700 1 _ |a Dinter, Elisabeth
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700 1 _ |a Wiltfang, Jens
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700 1 _ |a Zerr, Inga
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700 1 _ |a Flöel, Agnes
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700 1 _ |a Bürger, Katharina
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700 1 _ |a Höglinger, Günter U
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700 1 _ |a Levin, Johannes
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700 1 _ |a Düzel, Emrah
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700 1 _ |a Teipel, Stefan
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700 1 _ |a Beichert, Lukas
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700 1 _ |a Brosseron, Frederic
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700 1 _ |a Wagner, Michael
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700 1 _ |a Frommann, Ingo
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700 1 _ |a Ramirez, Alfredo
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700 1 _ |a Yakupov, Renat
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700 1 _ |a Schmid, Matthias
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700 1 _ |a Lingor, Paul
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700 1 _ |a Haass, Christian
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700 1 _ |a DESCRIBE-ALS
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700 1 _ |a consortium, DESCRIBE-FTD
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700 1 _ |a Spottke, Annika
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700 1 _ |a Günther, René
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700 1 _ |a Weydt, Patrick
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700 1 _ |a Neumann, Manuela
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700 1 _ |a Schneider, Anja
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773 _ _ |a 10.1080/21678421.2025.2509617
|g Vol. 26, no. 7-8, p. 720 - 728
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|t Amyotrophic lateral sclerosis & frontotemporal degeneration
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