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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
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Acta Neuropathologica Communications

ISSN: 2051-5960

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