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

Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update.
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DOI:
10.1007/s00401-009-0612-2
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发表时间:
2010-01
影响因子:
12.7
通讯作者:
Mann DM
Mann DM
中科院分区:
医学1区
文献类型:
--
作者:
Mackenzie IR;Neumann M;Bigio EH;Cairns NJ;Alafuzoff I;Kril J;Kovacs GG;Ghetti B;Halliday G;Holm IE;Ince PG;Kamphorst W;Revesz T;Rozemuller AJ;Kumar-Singh S;Akiyama H;Baborie A;Spina S;Dickson DW;Trojanowski JQ;Mann DM

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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. Although most FTLDs are characterized by cellular inclusion bodies composed of either tau (FTLD-tau) or TDP-43 (FTLD-TDP), approximately 10–15% of cases
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