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Open Access 01-01-2010 | Consensus Paper

Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update

Authors: Ian R. A. Mackenzie, Manuela Neumann, Eileen H. Bigio, Nigel J. Cairns, Irina Alafuzoff, Jillian Kril, Gabor G. Kovacs, Bernardino Ghetti, Glenda Halliday, Ida E. Holm, Paul G. Ince, Wouter Kamphorst, Tamas Revesz, Annemieke J. M. Rozemuller, Samir Kumar-Singh, Haruhiko Akiyama, Atik Baborie, Salvatore Spina, Dennis W. Dickson, John Q. Trojanowski, David M. A. Mann

Published in: Acta Neuropathologica | Issue 1/2010

Excerpt

One year ago, in this journal, we published a recommended nomenclature for the neuropathologic subtypes of frontotemporal lobar degeneration (FTLD) [7]. A major impetus behind this was to resolve the confusion that had arisen around the use of the term “FTLD with ubiquitinated inclusions” (FTLD-U), following the discovery that the molecular pathology of these cases was heterogeneous, with most, but not all, being characterized by pathological TDP-43 [6, 11]. In addition, a system of nosology was introduced that grouped the FTLD subtypes into broad categories, based on the molecular defect that is most characteristic, according to current evidence. This system provided a concise and consistent terminology that has now been widely adopted in the literature. Another anticipated advantage was the ability to readily accommodate new discoveries. At the time, we did not anticipate how quickly this attribute would be put to use. …

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Title: Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update
Authors: Ian R. A. Mackenzie
Manuela Neumann
Eileen H. Bigio
Nigel J. Cairns
Irina Alafuzoff
Jillian Kril
Gabor G. Kovacs
Bernardino Ghetti
Glenda Halliday
Ida E. Holm
Paul G. Ince
Wouter Kamphorst
Tamas Revesz
Annemieke J. M. Rozemuller
Samir Kumar-Singh
Haruhiko Akiyama
Atik Baborie
Salvatore Spina
Dennis W. Dickson
John Q. Trojanowski
David M. A. Mann
Publication date: 01-01-2010
Publisher: Springer-Verlag
Published in: Acta Neuropathologica / Issue 1/2010
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI: https://doi.org/10.1007/s00401-009-0612-2

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Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update

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