Table 2.

Harmonized classification for FTLD-TDP pathology (Adapted from Mackenzie et al., 2011)74.

Subtypes	Common phenotype	Pathological findings
A	bvFTD and PNFA	Numerous short DN and crescentic or oval NCI concentrated in neocortical layer 2. Moderate number of lentiform NII are common but inconsistent in this subtype.
B	bvFTD and MND with FTD	Moderate numbers of NCI, throughout all cortical layers with few DN.
C	SD and bvFTD	Predominance of elongated DN in upper cortical layers, with few NCI.
D	Familial IBMPFD	Numerous short DN and frequent lentiform NII.

bvFTD: behavioral variant frontotemporal dementia; PNFA: Progressive non-fluent aphasia; GRN: progranulin gene; MND: motor neuron disease; SD: semantic dementia; IBMPFD: inclusion body myopathy with Paget's disease of bone and frontotemporal dementia; VCP: valosin-containing protein gene.