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Table 2 Clinical features at baseline and follow-up by pathology group and subtype

From: Frontotemporal lobar degeneration proteinopathies have disparate microscopic patterns of white and grey matter pathology

  FTLD-Tau CBD MAPT PiD PSP FTLD-TDP Type A Type B Type C Type E
Tot. clinical data (N cases) 36 11 5 11 9 53 19 16 13 5
Onset to first visit, yearsa 3 (2–4) 1 (1–4) 4 (4–6) 3 (3–4) 3 (2–3) 2 (1–4) 2 (1–3) 2.5 (2–4) 3 (2–5) 2 (0–2)
N visitsa 5 (2–10) 7 (3–11) 2 (1–2) 8 (4–10) 4 (2–10) 3 (2–5) 5 (2–9) 2 (1–3) 2 (2–5) 3 (2–4)
BASELINE(0–3 years)b           
 Available data, N (%) 24/36 (66.7) 8/11 (72.7) 1/5 (20) 8/11 (72.7) 7/9 (77.8) 37/53 (69.8) 15/19 (78.9) 10/16 (62.5) 7/13 (53.8) 5/5 (100)
Behavioral features, N (%)
 Apathy/Inertia 13/24 (54.2) 3/8 (37.5) 1/1 (100) 5/8 (62.5) 4/7 (57.1) 29/37 (78.4) 14/15 (93.3) 9/10 (90) 2/7 (28.6) 4/5 (80)
 Disinhibition 19/24 (79.2) 5/8 (62.5) 1/1 (100) 8/8 (100) 5/7 (71.4) 29/37 (78.4) 12/15 (80) 8/10 (80) 4/7 (57.1) 5/5 (100)
 Empathy 12/24 (50) 4/8 (50) 0/1 (0) 5/8 (62.5) 3/7 (42.9) 16/37 (43.2) 6/15 (40) 3/10 (30) 2/7 (28.6) 5/5 (100)
 Hyperoral behavior 13/24 (54.2) 2/8 (25) 1/1 (100) 7/8 (87.5) 3/7 (42.9) 19/37 (51.4) 8/15 (53.3) 5/10 (50) 2/7 (28.6) 4/5 (80)
 Ritualistic behavior 14/24 (58.3) 5/8 (62.5) 0/1 (0) 7/8 (87.5) 2/7 (28.6) 21/37 (56.8) 7/15 (46.7) 8/10 (80) 3/7 (42.9) 3/5 (60)
Language features, N (%)
 Word-finding diff 16/24 (66.7) 8/8 (100) 0/1 (0) 3/8 (37.5) 5/7 (71.4) 27/37 (73) 11/15 (73.3) 8/10 (80) 6/7 (85.7) 2/5 (40)
 Agrammatism 8/24 (33.3) 6/8 (75) 0/1 (0) 1/8 (12.5) 1/7 (14.3) 9/37 (24.3) 4/15 (26.7) 3/10 (30) 1/7 (14.3) 1/5 (20)
 Imp gramm compr 7/24 (29.2) 6/8 (75.0) 0/1 (0) 0/8 (0) 1/7 (14.3) 11/37 (29.7) 6/15 (40) 3/10 (30) 2/7 (28.6) 0/5 (0)
 Imp naming 11/24 (45.8) 6/8 (75) 0/1 (0) 3/8 (37.5) 2/7 (28.6) 19/37 (51.4) 10/15 (66.7) 4/10 (40) 5/7 (71.4) 0/5 (0)
 Nonfluent speech 15/24 (62.5) 7/8 (87.5) 1/1 (100) 2/8 (25) 5/7 (71.4) 14/37 (37.8) 9/15 9 (60) 3/10 (30) 1/7 (14.3) 1/5 (20)
 Imp repetition 4/24 (16.7) 3/8 (37.5) 0/1 (0) 1/8 (12.5) 0/7 (0) 8/37 (21.6) 4/15 (26.7) 3/10 (30) 0/7 (0) 1/5 (20)
 Imp single-word compr 3/24 (12.5) 2/8 (25) 0/1 (0) 1/8 (12.5) 0/7 (0) 10/37 (27) 4/15 (26.7) 1/10 (10) 4/7 (57.1) 1/5 (20)
Other features, N (%)
 MND signs 0/24 (0) 0/8 (0) 0/1 (0) 0/8 (0) 0/7 (0) 6/37 (16.2) 1/15 (6.7) 4/10 (40) 0/7 (0) 1/5 (20)
 Parkinsonism 7/24 (29.2) 2/8 (25) 0/1 (0) 1/8 (12.5) 4/7 (57.1) 18/37 (48.6) 11/15 (73.3) 2/10 (20) 1/7 (14.3) 4/5 (80)
FOLLOW-UP (> 3 years)c
 Available data, N (%) 27/36 (75.0) 8/11 (72.7) 4/5 (80) 9/11 (81.8) 6/9 (66.7) 31/53 (58.5) 11/19 (57.9) 8/16 (50) 11/13 (84.6) 1/5 (20)
Behavioral features, N (%)
 Apathy/Inertia 24/27 (88.9) 6/8 (75) 3/4 (75) 9/9 (100) 6/6 (100) 27/31 (87.1) 10/11 (90.9) 8/8 (100) 8/11 (72.7) 1/1 (100)
 Disinhibition 24/27 (88.9) 7/8 (87.5) 4/4 (100) 9/9 (100) 4/6 (66.7) 26/31 (83.9) 9/11 (81.8) 7/8 (87.5) 9/11 (81.8) 1/1 (100)
 Empathy 17/27 (63) 5/8 (62.5) 2/4 (50) 7/9 (77.8) 3/6 (50) 22/31 (71) 8/11 (72.7) 7/8 (87.5) 6/11 (54.5) 1/1 (100)
 Hyperoral behavior 18/27 (66.7) 4/8 (50) 3/4 (75) 9/9 (100) 2/6 (33.3) 18/31 (58.1) 6/11 (54.5) 5/8 (62.5) 7/11 (63.6) 0/1 (0)
 Ritualistic behavior 25/27 (92.6) 8/8 (100) 4/4 (100) 9/9 (100) 4/6 (66.7) 24/31 (77.4) 6/11 (54.5) 7/8 (87.5) 10/11 (90.9) 1/1 (100)
Language features, N (%)
 Word-finding diff 21/27 (77.8) 8/8 (100) 3/4 (75) 6/9 (66.7) 4/6 (66.7) 25/31 (80.6) 10/11 (90.9) 4/8 (50) 10/11 (90.9) 1/1 (100)
 Agrammatism 13/27 (48.1) 6/8 (75) 1/4 (25) 1/9 (11.1) 5/6 (83.3) 3/31 (9.7) 1/11 (9.1) 1/8 (12.5) 1/11 (9.1) 0/1 (0)
 Imp gramm compr 12/27 (44.4) 6/8 (75) 1/4 (25) 0/9 (0) 5/6 (83.3) 8/31 (25.8) 4/11 (36.4) 2/8 (25) 2/11 (18.2) 0/1 (0)
 Imp naming 21/27 (77.8) 7/8 (87.5) 1/4 (25) 8/9 (88.9) 5/6 (83.3) 21/31 (67.7) 7/11 (63.6) 4/8 (50) 9/11 (81.8) 1/1 (100)
 Nonfluent speech 16/27 (59.3) 7/8 (87.5) 1/4 (25) 3/9 (33.3) 5/6 (83.3) 6/31 (19.4) 4/11 (36.4) 2/8 (25) 0/11 (0) 0/1 (0)
 Imp repetition 14/27 (51.9) 6/8 (75) 0/4 (0) 3/9 (33.3) 5/6 (83.3) 6/31 (19.4) 4/11 (36.4) 1/8 (12.5) 1/11 (9.1) 0/1 (0)
 Imp single-word compr 7/27 (25.9) 3/8 (37.5) 0/4 (0) 4/9 (44.4) 0/6 (0) 11/31 (35.5) 2/11 (18.2) 2/8 (25) 7/11 (63.6) 0/1 (0)
Other features, N (%)
 MND signs 1/27 (3.7) 1/8 (12.5) 0/4 (0) 0/9 (0) 0/6 (0) 3/31 (9.7) 1/11 (9.1) 2/8 (25) 0/11 (0) 0/1 (0)
 Parkinsonism 23/27 (85.2) 6/8 (75) 3/4 (75) 8/9 (88.9) 6/6 (100) 15/31 (48.4) 8/11 (72.7) 3/8 (37.5) 3/11 (27.3) 1/1 (100)
  1. CBD = corticobasal degeneration; compr = comprehension; diff = difficulties; FTLD-Tau = frontotemporal lobar degeneration with inclusions of the protein tau; FTLD-TDP = frontotemporal lobar degeneration with inclusions of the TDP-43 protein; imp = impaired; MAPT = FTLD-tau with MAPT gene mutation; PiD = Pick’s disease; MND = motor neuron disease; PSP = progressive supranuclear palsy; Type A/Type B/Type C/Type E = subtypes of FTLD-TDP
  2. aMedian and interquartile range are displayed for these variables
  3. bBaseline features were defined as those occurring within the first three years from disease onset. 28 patients (FTLD-Tau = 12; FTLD-TDP = 16), with no visits available within the first three years, were excluded from baseline data
  4. cFollow-up features were defined as those occurring after three years from disease onset. 31 patients (FTLD-Tau = 9; FTLD-TDP = 22), with no visits available after the first three years of disease, were excluded from follow-up data