Table 1 Demographic and clinical characteristics of FTLD patients grouped according to the global CDR plus NACC FTLD

Number

563

138

130

175

120

Age, years

66.4 (60.5–72.4)

65.9 (59.0–71.5)

68.6 (62.3–74.2)

65.4 (59.8–71.2)

66.7 (61.4–73.8)

0.022

Sex male, n (%)

296 (52.6)

80 (58.0)

72 (55.4)

91 (52.0)

53 (44.2)

0.143

Education, years

8.0 (5.0–13.0)

8.0 (5.0–13.0)

8.0 (5.0–13.0)

8.0 (5.0–12.0)

8.0 (5.0–12.8)

0.195

Age at onset, years

63.0 (58.0–69.0)

63.0 (56.0–69.0)

65.0 (60.0–71.3)^c

62.0 (57.0–68.0)^b

63.5 (57.3–70.0)

0.017

Disease duration, years

2.3 (1.5–3.7)

1.9 (1.2–3.1)^cd

2.0 (1.3–3.1)^cd

2.6 (1.6–4.0)^ab

3.2 (1.9–4.9)^ab

< 0.001

Monogenic disease, n (%)

109 (19.4)

26 (18.8)

23 (17.7)

34 (19.4)

26 (21.7)

0.884

Serum NfL (pg/mL)*

42.6 (30.5–54.9)

26.9 (20.0–39.4)^bcd

40.8 (28.5–54.8)^a

44.1 (36.9–60.7)^a

52.7 (44.0–66.5)^ab

< 0.001

bvFTD/avPPA/svPPA, n

392/108/63

92/25/21

89/27/14

121/35/19

90/108/63

0.602

CDR plus NACC FTLD SB

5.5 (3.0–10.0)

2.5 (1.0–3.5)^bcd

4.0 (3.0–5.0)^acd

7.5 (6.0–9.5)^abd

13.0 (11.0–15.0)^abc

< 0.001

MMSE

22.2 (16.3–25.4)

28.0 (26.0–29.0)^bcd

25.0 (22.0–27.3)^acd

21.0 (17.0–25.0)^abd

12.5 (4.3–19.0)^abc

< 0.001

FBI

15.0 (8.0–26.0)

8.0 (3.0–11.0)^bcd

12.0 (7.0–18.0)^acd

20.0 (12.0–26.0)^abd

31.0 (24.3–36.8)^abc

< 0.001

BADL lost

0.0 (0.0–1.0)

0.0 (0.0–0.0)^cd

0.0 (0.0–0.0)^d

0.0 (0.0–1.0)^ad

3.0 (1.0–5.0)^abc

< 0.001

IADL lost

1.0 (0.0–3.0)

0.0 (0.0–0.0)^cd

0.0 (0.0–1.0)^cd

2.0 (1.0–3.0)^abd

5.0 (4.0–7.0)^abc

< 0.001

Short story

7.2 (4.9–10.0)

9.5 (7.7–13.0)^bcd

8.4 (6.0–11.0)^acd

6.0 (3.5–8.0)^ab

5.4 (3.9–7.6)^ab

< 0.001

Rey figure copy

24.0 (14.8–30.5

30.1 (26.0–32.6)^cd

27.8 (21.9–32.6)^cd

21.0 (14.5–29.0)^abd

12.6 (6.9–18.6)^abc

< 0.001

Rey figure recall

8.0 (4.5–24.0)

11.8 (8.8–16.5)^bcd

9.8 (6.5–13.3)^acd

6.3 (3.5–9.5)^ab

5.3 (3.2–7.3)^ab

< 0.001

Phonological fluency

17.0 (10.0–24.0)

23.6 (18.4–29.0)^bcd

19.5 (14.0–29.0)^acd

14.9 (9.0–21.0)^abd

9.4 (4.9–15.1)^abc

< 0.001

Semantic fluency

22.0 (13.5–31.0)

31.1 (24.0–37.0)^bcd

27.0 (20.0–33.0)^acd

19.0 (13.0–25.0)^abd

10.6 (4.3–18.0)^abc

<0.001

Digit span

4.5 (3.8–5.5)

5.0 (4.5–5.8)^cd

4.8 (4.3–5.5)^d

4.5 (3.8–5.3)^ad

3.6 (2.8–4.5)^abc

< 0.001

Token test

26.7 (21.2–29.8)

30.0 (27.9–32.3)^bcd

28.3 (26.0–30.2)^acd

24.0 (19.5–27.8)^abd

18.3 (12.8–24.4)^abc

< 0.001

Trail making test part A

92.0 (47.0–197.0)

50.0 (28.0–73.0)^bcd

61.5 (40.8–109.3)^acd

107.0 (66.0–272.0)^abd

249.5 (133.3–391.0)^abc

< 0.001

Trail making test part B

383.0 (198.0–420.0)

191.0 (104.0–358.8)^bcd

340.0 (130.8–412.0)^acd

403.0 (337.0–432.0)^ab

403.0 (377.3–430.8)^ab

< 0.001

Clock drawing

5.0 (3.0–8.0)

8.0 (6.0–9.0)^bcd

7.0 (5.0–9.0)^acd

5.0 (3.0–6.0)^abd

3.0 (0.0–4.0)^abc

< 0.001