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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