title: [German consortium for frontotemporal lobar degeneration].
abstract: Frontotemporal lobar degeneration (FTLD) is an umbrella term for an aetiologically diverse group of neurodegenerative disorders with prominent lobar cortical atrophy. First this disease group was restricted to Pick's disease or Pick's complex. Several updates of the clinical classification systems were performed and discussed. Currently we summarize the following diseases under the FTLD spectrum: frontotemporal dementia (FTD) as a behavioural variant, primary non-fluent aphasia (PNFA) and semantic dementia as language variants, amyotrophic lateral sclerosis with FTD (ALS-FTD), corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP).From the pathophysiological aspect major progress was made. Neuropathologically FTLDs are now defined based on the molecular composition of these protein accumulations. A major distinction of tau-associated (FTLD-tau) and TDP43-associated (FTLD-TDP43) and to a lesser extend FUS-associated (FTLD-FUS) has been made. Additional risk genes were described. However from the therapeutic perspective even symptomatic therapy is under discussion. A major aim of our consortium is to develop parameters allowing an early diagnosis and follow-up, thus providing effective and objective parameters for therapeutic strategies.
authors.affiliation: Klinik für Neurologie, Universität Ulm, Oberer Eselsberg 35, 89081 Ulm, Deutschland. markus.otto@uni-ulm.de
authors.forename: M
authors.initials: M
authors.lastname: Otto
authors.forename: A C
authors.initials: AC
authors.lastname: Ludolph
authors.forename: B
authors.initials: B
authors.lastname: Landwehrmeyer
authors.forename: H
authors.initials: H
authors.lastname: Förstl
authors.forename: J
authors.initials: J
authors.lastname: Diehl-Schmid
authors.forename: M
authors.initials: M
authors.lastname: Neumann
authors.forename: H A
authors.initials: HA
authors.lastname: Kretzschmar
authors.forename: M
authors.initials: M
authors.lastname: Schroeter
authors.forename: J
authors.initials: J
authors.lastname: Kornhuber
authors.forename: A
authors.initials: A
authors.lastname: Danek
mesh_terms: D001284:Atrophy; D003430:Cross-Sectional Studies; D004268:DNA-Binding Proteins / Q000235:genetics; D018450:Disease Progression; D042241:Early Diagnosis; D005625:Frontal Lobe / Q000473:pathology; D057174:Frontotemporal Lobar Degeneration / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics; D005819:Genetic Markers / Q000235:genetics; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005820:Genetic Testing; D006801:Humans; D011379:Prognosis; D012307:Risk Factors; D057177:TDP-43 Proteinopathies / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D013702:Temporal Lobe / Q000473:pathology; D016875:tau Proteins / Q000235:genetics
chemical_list: D004268:DNA-Binding Proteins; D005819:Genetic Markers; C054369:MAPT protein, human; D016875:tau Proteins