Published in:
01-01-2009 | Editorial
Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations
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
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Issue 1/2009
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Excerpt
The neuropathology associated with the clinical entities frontotemporal dementia (FTD, behavioral variant FTD), progressive non-fluent aphasia (PNFA) and semantic dementia (SD), is heterogeneous with the common feature being a relatively selective degeneration of the frontal and temporal lobes (frontotemporal lobar degeneration, FTLD). As in other neurodegenerative conditions, most pathological subtypes of FTLD are characterized by specific kinds of intracellular protein inclusions. In the past few decades, the biochemical composition of many of these inclusion bodies has been determined. There is a growing trend to classify FTLD based on the presumed molecular defect, in the belief that this most closely reflects the underlying pathogenic process and because many of the eponymous and descriptively named syndromes of the past are now known to have imperfect clinicopathological correlation. …
