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)

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