Table 2.
Classification of frontotemporal lobar degenerations (adapted from [152])
| FTLD-Molecular abnormality | Genetic loci |
|---|---|
| FTLD-TDP | |
| Frontotemporal lobar degeneration with TDP-43 inclusions | |
| Subtype 1 (associated with SD) | None known |
| Subtype 2 (associated with MND) | Chromosome 9 |
| Subtype 3 (associated with FTDbv and PNFA) | GRN |
| Subtype 4 (associated with Paget's and IBM) | VCP |
| FTLD-TAU | |
| Frontotemporal lobar degeneration with tauopathy | MAPT |
| Pick's disease (3R tauopathy) | |
| Corticobasal degeneration (4R tauopathy) | |
| Progressive supranuclear palsy | |
| Multisystem tauopathy (4R tauopathy) | |
| FTLD-IF | |
| Frontotemporal lobar degeneration with intermediate filament inclusions | None known |
| FTLD-FUS | |
| Frontotemporal lobar degeneration with FUS inclusions | FUS |
| FTLD-UPS | |
| Frontotemporal lobar degeneration with inclusions composed of ubiquitin and other components of ubiquitin-proteasome system (e.g., P62-sequestosome) | CHMP2b |
| FTLD-AS | |
| Frontotemporal lobar degeneration with inclusions composed of α-synuclein (atypical Pick's disease) | SNCA? |
| FTLD-NI | |
| Frontotemporal lobar degeneration with no inclusions | None known |
FTLD-TDP Subtype 1 = Mackenzie Type 2; FTLD-TDP Subtype 2 = Mackenzie Type 3; FTLD-TDP Subtype 3 = Mackenzie Type 1; FTLD-TDP Subtype 4 = no Mackenzie type assigned.