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

From: Prodromal frontotemporal dementia: clinical features and predictors of progression

Variable All Global CDR plus NACC FTLD
0.5 1 2 3 p value
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
  1. NfL Neurofilament light chain, CDR Plus NACC FTLD SB Clinical dementia rating plus National Alzheimer’s Coordinating Center behaviour and language domains FTLD sum-of-boxes, MMSE Mini mental state examination, FBI Frontal Behavioural Inventory, BADL Basic activities of daily living, IADL Instrumental activities of daily living. Results are expressed as median (interquartile range), unless otherwise specified
  2. *Results for serum NfL are reported for a subgroup of patients; asignificant difference vs CDR plus NACC FTLD of 0.5; bsignificant difference vs CDR plus NACC FTLD of 1; csignificant difference vs CDR plus NACC FTLD of 2; dsignificant difference vs CDR plus NACC FTLD of 3