Konsortium zur Erforschung der frontotemporalen Lobärdegeneration

Translated title of the contribution: German consortium for frontotemporal lobar degeneration

M. Otto, A. C. Ludolph, B. Landwehrmeyer, H. Förstl, J. Diehl-Schmid, M. Neumann, H. A. Kretzschmar, M. Schroeter, J. Kornhuber, A. Danek

Research output: Contribution to journalArticlepeer-review

29 Scopus citations

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. Neuropathological-ly FTLDs are now defined based on the molecular composition of these protein accumulations. A major distinction of tau-associat-ed (FTLD-tau) and TDP43-associated (FTLD-TDP43) and to a lesser extend FUS-associ-ated (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.

Translated title of the contributionGerman consortium for frontotemporal lobar degeneration
Original languageGerman
Pages (from-to)1002-1005
Number of pages4
JournalNervenarzt
Volume82
Issue number8
DOIs
StatePublished - Aug 2011
Externally publishedYes

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