Table 1.
Recommended nomenclature for frontotemporal lobar degenerations
| Current terminology | Recommended new terminology |
Major pathological subtypesa |
|---|---|---|
| tau-positive FTLD | FTLD-tau | PiD |
| CBD | ||
| PSP | ||
| AGD | ||
| MSTD | ||
| Unclassifiable | ||
| tau-negative FTLD | ||
| FTLD-U | ||
| TDP-43-positive | FTLD-TDP | Type 1-4b |
| Unclassifiable | ||
| TDP-43-negative | FTLD-UPS | aFTLD-U |
| FTD-3 | ||
| NIFID | FTLD-IF | |
| DLDH | FTLD-ni | |
| Other | ||
| BIBD | BIBD |
aFTLD-U atypical frontotemporal lobar degeneration with ubiquitinated inclusions, AGD argyrophilic grain disease, BIBD basophilic inclusion body disease, CBD corticobasal degeneration, DLDH dementia lacking distinctive histopathology, FTD-3 frontotemporal dementia linked to chromosome 3, FTLD frontotemporal lobar degeneration, FTLD-U FTLD with ubiquitinated inclusions, IF intermediate filament, MSTD multiple system tauopathy with dementia, ni no inclusions, NIFID neuronal intermediate filament inclusion disease, PiD Pick’s disease, PSP progressive supranuclear palsy, TDP TDP-43, UPS ubiquitin proteosome system
Indicates the characteristic pattern of pathology, not the clinical syndrome. Note that FTDP-17 is not listed as a pathological subtype because cases with different MAPT mutations do not have a consistent pattern of pathology. These cases would all be FTLD-tau, but further subtyping would vary