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

By I. Mackenzie, M. Neumann, Eileen Bigio, Nigel J. Cairns, I. Alafuzoff, J. Kril, G. Kovacs, B. Ghetti, G. Halliday, I. Holm and 11 more. Acta Neuropathologica, 2009

985 citations recorded by the source corpus.

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.

Subfields: Neurology, Physiology

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Citations in the corpus

Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. (2011, methodology): “The classification was modified from existing FTLD criteria (Mackenzie et al., 2009, 2010) to accommodate cases with incomplete immunohistochemistry.”
National Institute on Aging-Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease: a practical approach (2011, neutral): “We refer the reader to recent consensus guidelines for the neuropathologic evaluation of FTLD and its subtypes [17, 34, 35], and issues important in the distinction of AD neuropathologic change from those of some forms of FTLD [58].”
ALS‐associated fused in sarcoma (FUS) mutations disrupt Transportin‐mediated nuclear import (2010, background): “Moreover, TDP-43-positive inclusions are also found in 490% of FTLDU patients (Arai et al, 2006; Neumann et al, 2006), now renamed as FTLD-TDP (Mackenzie et al, 2010).”
Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review (2010, background): “FTLD-tau includes patients with MAPT mutations, Pick’s disease, PSP, CBD, argyrophilic grain disease (AGD) and multiple system tauopathy with dementia (MSTD).(18) MAPT mutations are associated with different types of tau inclusions (Pick bodies, neurofibrillar tangles and pretangles) in the frontal ”
Clinicopathological Evaluation of Chronic Traumatic Encephalopathy in Players of American Football (2017, neutral): “Alzheimer disease 1 (4) 21 (25) 22 (20)”
Parkinson's disease and parkinsonism: neuropathology. (2012, background): “Frontotemporal lobar degenerations are clinically and pathologically heterogenous, and importantly fall into two major classes—TDP43 proteinopathies and tauopathies (Mackenzie et al. 2010).”
National Institute on Aging–Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease (2012, neutral): “For some tauopathies, such as tangle-predominant senile dementia (TPSD), chronic traumatic encephalopathy (CTE), or diffuse neurofibrillary tangles with calcification (DNTC), the distribution and density of tangles and the paucity of neocortical plaques must be carefully documented, since TPSD, CTE,”
Converging mechanisms in ALS and FTD: Disrupted RNA and protein homeostasis (2013, neutral): “Given that TDP-43 and FUS/TLS inclusion can also be found in glia (Mackenzie et al., 2010a), it is conceivable that glia contribute to disease pathogenesis.”