Table 2 Summated scores for DPR pathology ratings in Cortical, Medial Temporal, Motor, Subcortical and Cerebellar regions, as well as total score across all 5 regions, for patients with FTD (cases #1-8), FTD + MND (cases#9-14) and MND (cases#41-47) associated with expansion in C9ORF72

1

FTLD-TDP A

16

12

3

3

5

39

2

FTLD-TDP A

12

14

3

1

5.5

35.5

3

FTLD-TDP A

15

18

3

5

7.5

48.5

4

FTLD-TDP A

13

12

2

2.5

3.5

33

5

FTLD-TDP A

12

11

1

3

3

30

6

FTLD-TDP A

11

9

1.5

3

2

26.5

7

FTLD-TDP A

12

9.5

1

5.5

3.5

31.5

8

FTLD-TDP A

22

23

2

4

5.5

55.5

9

FTLD-TDP B

20

15.5

2

1

4.5

43

10

FTLD-TDP B

12

11

2

2

4

31

11

FTLD-TDP B

11

10.5

2

1.5

4

29

12

FTLD-TDP B

18

16

3.5

4.5

5

47

13

FTLD-TDP B

11

11

2

3.5

2

29.5

14

FTLD-TDP B

16

15

3

4.5

6.5

45

41

MND

16

13

3

6

5.5

43.5

42

MND

15

13

3

3

1

35

43

MND

14

18

3

4

3

42

44

MND

12

18

2.5

5

3

40.5

45

MND

13

12

3

1.5

4.5

34

46

MND

11

19

2

4.5

3

39.5

47

MND

23

23

3

5

3.5

57.5