Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-53755
Otto, M; Ludolph, A C; Landwehrmeyer, B; Förstl, H; Diehl-Schmid, J; Neumann, M; Kretzschmar, H A; Schroeter, M; Kornhuber, J; Danek, A (2011). Konsortium zur Erforschung der frontotemporalen Lobärdegeneration. Der Nervenarzt, 82(8):1002-1005.
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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.
|Other titles:||German consortium for frontotemporal lobar degeneration|
|Item Type:||Journal Article, refereed, original work|
|Communities & Collections:||04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology|
|DDC:||570 Life sciences; biology|
610 Medicine & health
|Deposited On:||07 Jan 2012 16:08|
|Last Modified:||23 Feb 2014 13:31|
|Citations:||Web of Science®. Times Cited: 3|
Scopus®. Citation Count: 3
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