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Table 2 Demographics and pathologic features of FTLD-TDP type A and type B cases split by mutation

From: C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation

Mutation FTLD-TDP type A FTLD-TDP type B
GRN + (n = 4) GRN – (N = 4) C9+ (n = 4) C9 – (n = 4)
Demographics
 Female Sex % 1 (25%) 0 3 (75%) 2 (50%)
 Age at death 67 (60, 76) 74 (61, 78) 62 (55, 67) 68 (60, 79)
Clinical diagnosis
 bvFTD 2 (50%) 1 (25%) 2 (50%) 1 (25%)
 PPA 1 (25%) 0 0 0
 FTD-MND/ALS 0 1 (25%) 2 (50%) 3 (75%)
 Other 1 (25%) 2 (50%) 0 0
Co-pathologies
 Hippocampal sclerosis 4 (100%) 4 (100%) 2 (50%) 0
 Braak NFT stage 1 (0.0, 2.0) 1.0 (1.0, 1.5) 2.0 (2.0, 3.0) 1.5 (1.0, 3.0)
 Thal Phases 0.5 (0.0, 1.0) 1.0 (0.0, 3.0) 1.0 (0.0. 2.0) 0.0 (0.0, 4.0)
Pathological results
ERC/CA1/Sub
 Phosphorylated TDP-43 (pTDP-43) 2.5 (2.0–3.0) 1.5 (1.0, 3.0) 3.0 (1.0, 3.0) 2.0 (2.0, 3.0)
 C-Terminal specie TDP-43 (cTDP-43) 2.0 (1.0–3.0) 1.0 (0.5–2.0) 0.0 (0.0, 1.0) 1.0 (0.0, 3.0)
 Full length TDP-43 (nTDP-43) 1.0 (1.0, 1.0) 1.0 (0.5, 2.0) 1.5 (0.0, 2.0) 2.0 (1.0, 3.0)
Dentate nucleus of hippo
 Phosphorylated TDP-43 (pTDP-43) 2.5 (2.0, 3.0) 2.0 (1.0, 3.0) 3.0 (2.0, 3.0) 3.0 (2.0, 4.0)
 C-Terminal specie TDP-43 (cTDP-43) 2.0 (1.0, 3.0) 1.5 (1.0, 2.0) 2.0 (1.0, 3.0) 2.0 (1.0, 4.0)
 Full length TDP-43 (nTDP-43) 1.0 (0.0, 1.0) 0.75 (0.0, 1.0) 2.0 (0.0, 3.0) 1.0 (1.0, 4.0)
  1. Data shown as median (range) and percentages
  2. bvFTD Behavioral variant frontotemporal dementia, FTD-MND/ALS Frontotemporal dementia with motor neuron disease/amyotrophic lateral sclerosis, PPA Primary progressive aphasia, GRN progranulin, C9 C9ORF72, NFT neurofibrillary tangle