Table 2.
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.