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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)

From: Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72

Case ID# Gene Pathology TDP-43 score
   DG TCX FCX Total
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