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Table 1 Demographics and pathologic features of FTLD-TDP cases

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

  FTLD-TDP type A
(n = 8)
FTLD-TDP type B
(n = 8)
FTLD-TDP type C
(n = 8)
Demographics
 Female Sex % 1 (12.5%) 5 (62.5%) 4 (50.0%)
 Age at death 72 (60, 78) 63 (55, 79) 70 (65, 83)
Clinical diagnosis
 bvFTD 3 (37.5%) 3 (37.5%) 1 (12.5%)
 PPA 1 (12.5%) 0 7 (87.5%)
 FTD-MND/ALS 1 (12.5%) 5 (62.5%) 0
 Other 3 (37.5%) 0 0
Genetic findings
C9ORF72 1 (12.5%) 4 (50.0%) 0
GRN 4 (50.0%) 0 0
Co-pathologies
 Hippocampal sclerosis 8 (100%) 2 (25%) 1 (12.5%)
 Braak NFT stage 1.0 (0.0, 2.0) 2.0 (1.0, 3.0) 1.5 (0.0, 3.0)
 Thal Phases 0.5 (0.0, 3.0) 0.0 (0.0, 4.0) 0.5 (0.0, 2.0)
 Pathological results
ERC/CA1/Sub
 Phosphorylated TDP-43 (pTDP-43) 2.0 (1.0, 3.0) 2.5 (1.0, 3.0) 3.0 (1.0, 3.0)
 C-Terminal specie TDP-43 (cTDP-43) 1.5 (0.5, 3.0) 0.5 (0.0, 3.0) 3.0 (0.5, 3.0)
 Full length TDP-43 (nTDP-43) 1.0 (0.5, 2.0) 2.0 (0.0, 3.0) 2.0 (0.0, 3.0)
Dentate nucleus of hippocampus
 Phosphorylated TDP-43 (pTDP-43) 2.5 (1.0, 3.0) 3.0 (2.0, 4.0) 3.0 (2.0, 4.0)
 C-Terminal specie TDP-43 (cTDP-43) 2.0 (1.0, 3.0) 2.0 (1.0, 4.0) 3.0 (1.0, 4.0)
 Full length TDP-43 (nTDP-43) 1.0 (0.0, 1.0) 1.0 (0.0, 4.0) 1.0 (0.0, 3.0)
  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 proganulin, NFT neurofibrillary tangle