Table 7: Neuropathologic subtypes.

HistopathologySubtypesClinicalChromosome

TauoapthyPiDbvFTD, PNFA17 (MAPT)
PSPPSP
CBDCBD, PNFA
AGDbvFTD, MND
MST

TDP-43Type 1bvFTD
Type 2SD, MND9 (IFT74)
Type 3bvFTD, SD, PNFA17 (PGRN)
Type 4bvFTD, myopathy, Paget’s9 (VCP)

FTLD-UPSFTD 3bvFTD3 (CHMP2B)

BIBDFTD-MND

FTLD-IF

FTLD-ni

PiD: Pick’s disease, PSP: progressive supranuclear palsy, CBD: cortico-basal-ganglionic degeneration, AGD: argyrophilic grain disease, MND: motor neuron disease, PNFA: progressive nonfluent aphasia, SD: semantic disease/variant, MST: sporadic multisystem tauopathy, BIBD: basophilic inclusion body disease, FTLD IF: FTLD with intermediate filament inclusions, FTLD ni: FTLD with no inclusions, FUS: fused in sarcoma, NIF: neuronal intermediate filaments, VCP: valosin containing protein, CMBP 2B: charged multivesicular body protein 2B, MAPT: microtubule associated protein tau, TDP-43: TAR DNA binding protein 43, IFT74: intraflagellar transport protein 74.
Modified from Josephs [25].