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000281785 1001_ $$0P:(DE-2719)2811732$$aHermann, Andreas$$b0$$eFirst author
000281785 245__ $$a'The DESCRIBE-ALS-FTD study: a prospective multicenter observational study of the ALS-FTD spectrum'.
000281785 260__ $$aAbingdon$$bTaylor Francis Group$$c2025
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000281785 520__ $$aBackground: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) exhibit significant clinical, genetic and neuropathological abnormalities, and are regarded as belonging to a common disease spectrum, referred to as the ALS-FTD spectrum disorders. Our understanding of the underlying mechanisms of these diseases has advanced significantly, including molecular neuropathology, genetics and molecular pathophysiology. The heterogeneity of these diseases poses significant challenges to translational research and drug development, particularly in sporadic cases. Consequently, there is an urgent need to improve patient stratification for the successful execution of future clinical trials. Methods/Results: We here describe the study design of the DESCRIBE-ALS/FTD study which aims to address this research gap by undertaking a systematic sampling of patients from the ALS FTD spectrum, encompassing all possible disease variants. The main objective of the study is to systematically document detailed cross-sectional phenotyping and the temporal progression of motor and neuropsychological abnormalities that occur in both ALS and FTD. Additionally, it seeks to systematically correlate these abnormalities with genetics and potentially predictive biomarkers including longitudinal biomaterial sampling, brain imaging and brain banking. Furthermore, first-degree relatives of patients with disease-causing gene variants undergo the same assessments to also sample presymptomatic risk gene carriers. Conclusion: With this prospective registry study we aim to generate datasets which will help researchers identifying different disease traits in people with sporadic and genetic ALS and FTD and to develop biomarkers to identify preclinical and prodromal disease stages.
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000281785 650_7 $$2Other$$aAmyotrophic lateral sclerosis
000281785 650_7 $$2Other$$abiomarkers
000281785 650_7 $$2Other$$adisease traits
000281785 650_7 $$2Other$$afrontotemporal dementia
000281785 650_7 $$2Other$$agenetics
000281785 650_7 $$2Other$$aneuropathology
000281785 650_2 $$2MeSH$$aHumans
000281785 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: genetics
000281785 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: diagnosis
000281785 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: physiopathology
000281785 650_2 $$2MeSH$$aFrontotemporal Dementia: genetics
000281785 650_2 $$2MeSH$$aFrontotemporal Dementia: diagnosis
000281785 650_2 $$2MeSH$$aProspective Studies
000281785 650_2 $$2MeSH$$aMale
000281785 650_2 $$2MeSH$$aFemale
000281785 650_2 $$2MeSH$$aMiddle Aged
000281785 650_2 $$2MeSH$$aAged
000281785 650_2 $$2MeSH$$aDisease Progression
000281785 650_2 $$2MeSH$$aCross-Sectional Studies
000281785 693__ $$0EXP:(DE-2719)DESCRIBE-FTD-20160101$$5EXP:(DE-2719)DESCRIBE-FTD-20160101$$eDZNE Clinical Registry Study on Frontotemporal Dementia (FTD)$$x0
000281785 7001_ $$0P:(DE-2719)2380559$$aPrudlo, Johannes$$b1$$udzne
000281785 7001_ $$0P:(DE-2719)9000419$$aKasper, Elisabeth$$b2$$udzne
000281785 7001_ $$0P:(DE-2719)2811275$$aSynofzik, Matthis$$b3$$udzne
000281785 7001_ $$0P:(DE-2719)2811024$$aPeters, Oliver$$b4$$udzne
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000281785 7001_ $$0P:(DE-2719)2811317$$aWiltfang, Jens$$b7$$udzne
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000281785 7001_ $$0P:(DE-2719)2812683$$aFlöel, Agnes$$b9$$udzne
000281785 7001_ $$0P:(DE-2719)2811351$$aBürger, Katharina$$b10$$udzne
000281785 7001_ $$0P:(DE-2719)2811373$$aHöglinger, Günter U$$b11$$udzne
000281785 7001_ $$0P:(DE-2719)2811659$$aLevin, Johannes$$b12$$udzne
000281785 7001_ $$0P:(DE-2719)2000005$$aDüzel, Emrah$$b13$$udzne
000281785 7001_ $$0P:(DE-2719)2000026$$aTeipel, Stefan$$b14$$udzne
000281785 7001_ $$0P:(DE-HGF)0$$aBeichert, Lukas$$b15
000281785 7001_ $$0P:(DE-2719)2810593$$aBrosseron, Frederic$$b16$$udzne
000281785 7001_ $$0P:(DE-2719)2000057$$aWagner, Michael$$b17$$udzne
000281785 7001_ $$0P:(DE-2719)2810481$$aFrommann, Ingo$$b18$$udzne
000281785 7001_ $$0P:(DE-2719)2812825$$aRamirez, Alfredo$$b19$$udzne
000281785 7001_ $$0P:(DE-2719)2812398$$aYakupov, Renat$$b20$$udzne
000281785 7001_ $$0P:(DE-2719)2811245$$aSchmid, Matthias$$b21$$udzne
000281785 7001_ $$0P:(DE-2719)2812561$$aLingor, Paul$$b22
000281785 7001_ $$0P:(DE-2719)2202037$$aHaass, Christian$$b23$$udzne
000281785 7001_ $$aDESCRIBE-ALS$$b24
000281785 7001_ $$aconsortium, DESCRIBE-FTD$$b25$$eCollaboration Author
000281785 7001_ $$0P:(DE-2719)2811324$$aSpottke, Annika$$b26$$udzne
000281785 7001_ $$0P:(DE-2719)2811849$$aGünther, René$$b27$$udzne
000281785 7001_ $$0P:(DE-2719)9001116$$aWeydt, Patrick$$b28$$udzne
000281785 7001_ $$0P:(DE-2719)2810592$$aNeumann, Manuela$$b29$$udzne
000281785 7001_ $$0P:(DE-2719)2812035$$aSchneider, Anja$$b30$$eLast author$$udzne
000281785 773__ $$0PERI:(DE-600)2705061-0$$a10.1080/21678421.2025.2509617$$gVol. 26, no. 7-8, p. 720 - 728$$n7-8$$p720 - 728$$tAmyotrophic lateral sclerosis & frontotemporal degeneration$$v26$$x2167-8421$$y2025
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