Table 3 Summated scores for TDP-43 pathology ratings in Frontal and Temporal cortex, and Dentate Gyrus, as well as total score across all 3 regions, for patients with FTD (cases #1-8) or FTD + MND (cases#9-13) associated with C9ORF72 expansions, and for patients with FTLD due to GRN mutation (case#15-26) or not associated with any known mutation (cases#27-40)

1

C9ORF72

FTLD-TDP A

1

4

3

8

2

C9ORF72

FTLD-TDP A

2

4

3

8

3

C9ORF72

FTLD-TDP A

2

1

3

6

4

C9ORF72

FTLD-TDP A

1

3

3

7

5

C9ORF72

FTLD-TDP A

1

1

1

3

6

C9ORF72

FTLD-TDP A

2

2

2

6

7

C9ORF72

FTLD-TDP A

1

1

2

4

8

C9ORF72

FTLD-TDP A

2

2

3

7

9

C9ORF72

FTLD-TDP B

3

2

2

7

10

C9ORF72

FTLD-TDP B

4

4

4

12

11

C9ORF72

FTLD-TDP B

4

3

3

10

12

C9ORF72

FTLD-TDP B

1

2

2

5

13

C9ORF72

FTLD-TDP B

na

3

3

na

15

GRN

FTLD-TDP A

na

4

4

na

16

GRN

FTLD-TDP A

na

2

2

na

17

GRN

FTLD-TDP A

4

3

3

10

18

GRN

FTLD-TDP A

2

2

2

6

19

GRN

FTLD-TDP A

3

3

3

9

20

GRN

FTLD-TDP A

1

4

4

9

21

GRN

FTLD-TDP A

2

4

4

10

22

GRN

FTLD-TDP A

4

2

1

7

23

GRN

FTLD-TDP A

2

1

3

6

24

GRN

FTLD-TDP A

2

0

1

3

25

GRN

FTLD-TDP A

3

4

4

11

26

GRN

FTLD-TDP A

na

3

4

na

27

No mutation

FTLD-TDP A

2

3

1

6

28

No mutation

FTLD-TDP A

3

4

4

11

29

No mutation

FTLD-TDP A

2

2

2

6

30

No mutation

FTLD-TDP A

1

1

1

3

31

No mutation

FTLD-TDP B

4

4

4

12

32

No mutation

FTLD-TDP B

0

3

4

7

33

No mutation

FTLD-TDP B

3

0

1

4

34

No mutation

FTLD-TDP B

2

1

1

4

35

No mutation

FTLD-TDP B

3

2

4

9

36

No mutation

FTLD-TDP B

4

1

1

6

37

No mutation

FTLD-TDP B

2

2

2

6

38

No mutation

FTLD-TDP B

1

0

1

2

39

No mutation

FTLD-TDP B

1

2

3

6

40

No mutation

FTLD-TDP B

3

3

3

9